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ENT Publications by Dr. Desarda
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221297

TRANSTYMPANIC LOW DOSE GENTAMICIN IN  MENIER’S DISEASE.


K.K.DESARDA.  D. BHISEGAONKAR   SHEETAL SANT   KEM HOSPITAL PUNE.


ABSTRACT:

Current role of transtympanic gentamicin therapy in the management of unilateral meniere’s disease is discussed in detail with its efficacy in  the management.
Transtympanic low dose gentamicin infusion is an alternative to surgical labyrinthectomy and Vestibular nerve section for the treatment of refractory vertigo associated with Meniere’s Gentamicin, the current drug of choice provides excellent vertigo control and is a less Invasive method to destroy the vestibular labyrinth. The goal of the treatment is to eliminate the abnormal vestibular inputs from the vestibular inputs from the diseased ear without adversely affecting the hearing. It’s salutary effect results from the damage of both the sensory neuroepithelium and the dark cells of the labyrinth. Intratympanic low dose gentamicin may cause sensory neural hearing loss hearing loss in some patients {10–15%}. Despite this S.N.. loss, results are encouraging. 50 cases of unilateral Meniere’s disease were treated at KEM hospital, Pune, during 1996 to 2012 with the follow–up of 2 to 4 yrs. All cases were infused with low dose gentamicin transtympanically for 4 to 6 weeks. This prospective study of gentamicin infusion revealed high success rate of controlling vertigo in about 92% patients. This treatment modality offers a less invasive but effective option for treating refractory vertigo of Meniere’s disease.We strongly recommend this modality of treatment for treating Mniere’s disease.

Low dose -- our preference
The low dose method involves using 1-2 injections of gentamicin, waiting a month between injections. This variant stops vertigo 70-80% of the time, with no significant side effects at all. The low dose variant is relatively new, and there is not nearly as much data concerning outcome as the high-dose variant.
The 2nd injection is given only if there has been a vertigo spell in the 2 weeks prior. In other words, instead of titrating to the onset of damage to vestibular system (as is done for high-dose ITG/TTG), the criterion is a good effect on the disease. This simple idea seems to result in far better results. Occasionally a 3rd dose is given. Usually this results in complete vestibular loss



Key words: Gentamicin, Meniere’s, chemical labyrinthectomy, micro wick

Introduction
Meniere’s disease is a clinical disorder characterized by acute episodes of vertigo, fluctuating hearing loss, aural fullness & tinnitus2. 80% patients of Meniere’s disease are treated successfully by medical treatment. Remaining 20% who have failed medical treatment need either surgical or chemical ablation of vestibular function. Surgical procedures designed to prevent endolymphatic hydrops such as cochleostomy, endolymphatic sac shunt are falling out of favour due to high incidence of sensorineural hearing loss (20–30%). Vestibular nerve section, although very effective is a difficult surgery with significant morbidity and not uncommon complications

Schucknecht (1957) introduced transtympanic mode of delivery with streptomycin4 & Beck Schmidt (1978) first used gentamicin by the Transtympamnic route5 for treating Meniere’s disease and proved the efficacy of the gentamycin infusion. The success rate was 92–100%2,4,5,6. This prospective study of 50 patients of unilateral Meniere’s disease in KEM hospital, Pune, during 1996–2012 revealed that intratympanic gentamicin therapy has a success rate of controlling vertigo in 92% with S. N. loss in 10% of the cases. This being a safe, less invasive and readily accepted treatment modality was the choice of treatment in controlling refractory vertigo of Meniere’s disease. The intratympanic low dose gentamicin infusion (40mg/ml) buffered with 7.5% sodium bicarbonate solution was used slowly over a 10 minutes period. We have recorded our observations and post infusion results for over sixteen years and found that gentamicin therapy is an ideal option to surgical labyrinthectomy. The ease with which gentamicin can be obtained and apparently lower incidence of its cochleotoxic side–effect has currently made it the preferred aminoglycoside for chemical treatment of Meniere’s disease.

Materials & methods:
(TABLE I, II, III)
50cases of 32 males & 18 females within age group of 30–70 years of proved unilateral Meniere’s disease were treated with repeated transtympanic gentamicin infusion through the grommet for 4–6 weeks on weekly basis. During the study, we have excluded CSOM., acoustic neuroma, acoustic trauma, barotraumas, diabetes, hypertension, cervical spondylosis & anaemia with the relevant investigations. Only unilateral Meniere’s disease cases were included in the study group. Prior to infusion, all patients were subjected to routine investigation such as PT Audiometry, Tone decay, SISI, caloric tests, MRI. brain especially for internal auditory meatus and posterior cranial fossa and all relevant biochemical tests. All 50 cases were given medical treatment for at least 3 months prior to gentamicin infusion therapy. The treatment includes diuretics, vasodilators, labyrinthine sedatives, antiallergics and steroids.

All cases were also informed about the side effects of this treatment such as sensorineural hearing loss in 10%, ataxia lasting for 4–6 weeks and imbalance until central compensatory mechanisms take over and the need for the head and neck Catwhorne Cooksey’s exercises after the treatment end–point. The infusion of low dose gentamicin 40 mg/ml was used with dilution with 7.5% sodium bicarbonate solution. 10mg/ml gentamicin was slowly infused intratympanically by poster inferior quadrant myringotomy through grommet .

The study ear was elevated by 45 degrees and infusion continued slowly over 10 minutes. The patient maintained the supine position with the study ear above for about 45 minutes post–infusion in the recovery room. The infusions were administered on weekly basis for 4–6 wks. Depending on clinical response in controlling vertigo. The end point of the treatment was total relief from vertigo and associated symptoms. The morbidity of unsteadiness/dysequilibrium, S.N. loss and the appearance of spontaneous nystagmus were cardinal signs of the efficacy of the gentamicin infusion.

Table I: Age distribution:
Age group (years) No. of cases
30–40
41–50
51–60
61–70
Total 06
13
22
09
50


Table II – The sex ratio:
Sex No. of cases
Male
Female
Total 32
18
50

Table III – Transtympanic infusions required:
No. of Infusions Cases
1–2
3–4
5–6
Total 10
23
17
50

The results (As per guidelines of AAO–HNS, 1985) 7:
Table IV: Vertigo Relief (n=50)
Vertigo control No. of patients Percentage
Complete 36 72
Substantial 10 20
Limited 02 04
Insignificant 02 04
Worse 00 00

Table V: Hearing loss (n=50)
Hearing loss No. of patients Percentage
Worsened 05 10
Unchanged 37 74
Improved 08 16


Table VI : Tinnitus control (n=50)
Tinnitus control No. of patients Percentage
Absent 09 18
Improved 36 72
Unchanged 05 10

Table VII: Aural Fullness Control (n=50)
Aural Fullness Control No. of patients Percentage
Absent 31 62
Improved 19 38
Unchanged 00 00


Table VIII : Post Treatment  Caloric Response

Post Treatment Caloric Response          No. of patients Percentage
No. response                36         72
Poor response                10         20
No. Change                04         08

Table IX: Comparison of Results of various Authors.

          Authors        Vertigo Control       Hearing Loss
Beck & Schmidt (1978)5                95%              15%
Odkivist (1988) 8                95%              22%
Nedzelski (1993) 9               100%              37%
Lorne (1993) 10               100%              41%
Susanne & Pyykko (1995) 11                90%              32%
KEM Hospital Pune, Study (KKD)                92%              15%

It was observed that 4–6 wks period was taken to achieve excellent vertigo control. Post infusion audio vestibular tests were done in all cases to record the observations and results.


Results: (Table–IV, V, VI, VII, VIII)

During study, we have recorded complete control in 36 cases (72%), limited in 2 cases (4%) and insignificant in 2 cases (4%). The hearing loss worsened in 5 cases (10%), improved in 37 cases (74%) & not improved in 6 cases (18%). The tinnitus was absent in 9 cases (18%), improved in 36 cases (72%), and unchanged in 5 cases (10%). The aural fullness was absent in 31 cases (62%), improved in 19 cases (38%) and unchanged in 0 cases (0%).

Discussion: (Table IX)

One of the exciting new developments in inner ear research is the feasibility to place medications directly into the inner ear. Transtympanic low dose gentamicin infusion can be done by several methods such as transtympanic injection13, Micro Wick of Silverstien1, Microcatheter4, myringotomy & grommet, etc. the gentamicin is the current amino glycoside of choice because it is less cochleotoxic than streptomycin3 (John Shea, 1994) 13. Gentamicin has its ototoxic effect on the sensory neuroepithelium and it destroys the endolymph secreting cells (dark cells of utricle, base of ampullae & lateral wall of crus communes) 14.

We have chosen the myringotomy & grommet route for its simplicity and the repeated procedures required during the treatment. Since this is an office procedure, can be repeated on weekly basis, easily accepted by all the patients and is noninvasive and cost effective, this mode of drug delivery appeared to be the best to us.

Review of the literature revealed that results obtained in vertigo control and hearing loss are variable. Beck & Schmidt (1978) 5, had vertigo control was 95% and S. N. hearing loss was 15%. Odkivist (1988)8, had 95% vertigo control and 22% S. N. loss, Nedzelski (1992)9, had 100% vertigo control and 37% S.N. loss. Lorne (1993) 10 also had 100% vertigo control and 47% S. N.Loss. Susanne and Pyykko (1995)11 showed 90% vertigo control and 32% S.N. loss. Our study revealed 92% vertigo control and 15% S.N.loss.

From the study it appears that there are some disadvantages for gentamicin therapy such as 10% risk of hearing loss. Tinnitus and aural fullness may persist, and it is also difficult to regulate the actual degree of diffusion into perilymph bypassing the cohlea. It was also noted that there are various factors altering absorption of gentamicin in the inner ear like the thickness of round window membrane, scarring and adhesions in middle ear, head position and dependency or round window, potency of eustatian tube, rate of turnover of perilymph and endolymph and individual susceptibility to ototoxic gentamicin3.

The concentration of intratympanic gentamicin is most important in predicting the degree of ototoxicity while the duration of therapy appears to be less significant15. The optimal treatment regimen for Meniere’s disease will be such that vestibular hypo activity will be achieved but there will be no hearing loss.

It was also observed during the study that the no response to gentamicin infusion is probably be due to be central lesion e.g. migraine, micro vascular compression or it may be a bilateral Meniere’s disease or it could be due to the round window adhesions (which prevents proper passage of the drug delivery  to the inner ear) or other causes of vertigo. Due respect must be given to the accurate diagnosis of the Meniere’s disease and until one is very very sure about the diagnosis, one should not try this treatment. The other modality of treatment is nonchemical ablation of the vestibular endorgan by ultrasonic and cryosurgery which is not easily available at all the centeres2.

In our study all cases were administered medical treatment for 3 months before the transtympanic infusion. The follow–up was kept on regular basis at 3 months, 6 months, and yearly after the completion of the treatment. It was our observation that six patients (12%) developed irritative nystagmus following transtympanic gentamicin perfusion during the treatment, which recovered in 2 weeks time. This unique new finding may represent a recovery phenomenon resulting from a temporarily reversible ototoxic effect in the treatment ear. Despite small percentage of S. N. loss (10%) the results are encouraging with gentamicin infusion treatment

.


Author’s Conclusion:

Interest has been growing in the intratympanic application of medicine for the control of Meniere disease and other otologic maladies. Although the use of the aminoglycosides streptomycin and gentamicin has received the most attention, other medications, including dexamethasone and lidocaine, have also been given transtympanically. Despite the growing amount of research, many questions remain un ansered regarding the efficacy, safety, and dosing regimens of these treatments.
Optimal methods of inner ear drug delivery will depend on toxicity, therapeutic dose range, and characteristics of the agent to be delivered. Advanced therapy development will likely require direct intracochlear delivery with detailed understanding of associated pharmacokinetics.
Transtympanic  low dose gentamicin infusion has a consistent vertigo control (92%), is relatively inexpensive, easy to perform under local anesthesia as an office procedure and without significant morbidity. This chemical ablation provides a reasonably safe and effective method for controlling acute, recurrent vertigo in patients of Meniere’s disease who have failed medical therapy.
Intratympanic therapies offer an advantage over endolymphatic sac or destructive surgeries in that injections can be repeated with minimal costs and morbidity, and may be titrated to clinical response.

We strongly recommend this modality of treatment for severe, unilateral, refractory intractable vertigo of Meniere’s disease before destructive surgery is contemplated because long–term success with this procedure is significantly greater than with sac surgery or vestibular neurectomy.

References
Silverstein H. (1999) : Use of a new device, the Micro Wick (tm) to deliver medication to the inner ear. ENT Journal 79:8.
Scott Brown’s Otolaryngology, 6th Edition (1997): Butterworth & Heinemann Publication, Meniere’s Disease, 3:19:1–3:19:38.
Otolaryngologica Clinics of North America, Hirsh B. E., Kamerer D. B. (Dec. 1997): Role of chemical labyrinthectomy in the treatment of Meniere’s disease, Vol. 30, No. 6, 1039–1049.
Schuknecht H. F. (Dec.1997) : Ablation therapy in the management of Meniere’s disease. Acta Otolaryngology supplement (Stockh), 13:1–41.
Beck C., Schmidt C. L., (1978) : Ten years of experience with intratympanically applied streptomycin (Gentamicin) in the therapy of morbus Meniere, Archives Otolaryngology 221:149–152.
Surgery of the ear, Shambaugh, Glasscock, 4th Edition. W. B. Sounder’s Publication, Surgical treatment of periferal vestibular disorders, 467–500.
Pearson B. W., Brackmann D. E. (Chairman) (1985): Committee on hearing and equilibrium guidelines for repeating treatment results in Meniere’s disease. Otolaryngology Head and Neck Surgery, 13:579–581.
Odkvist L. M. (1988): Middle ear ototoxic treatment for Meniere’s disease. ACT Otolaryngology (Stockh) supplement 457:83–86.
Nedzelski J. M., Bryle G. E., Pfleiderer A. G.(1993): Treatment of Meniere’s disease: Update of an ongoing study. American Journal of Otolaryngology 14:278–282.
Lorne S. Parnes, Duncan Riddel, (1993): Irritative spontaneous nystagmus following intratympanic gentamicin for Meniere’s disease, Laryngoscope 103:745–759.
Susanna K., Pyykko I., Ishizaki H. & Aalto H., (1995): Effect of intratympanically administeree gentamicin on hearing & tinnitus in Meniere’s disease. Acta Otolaryngology (Stockh) supplement, 520:184–185.
Hirsch B. E., Kamerer D. B. (1997): Intratympanic Gentamicin in Meniere’s disease. American Journal of otolaryngology, 18:44–51.
John Shea Jr. & Xianxi G. E. (April 1994): Streptomycin perfusion of the labyrinth through the round window plus intravenous streptomycin, Otolaryngologc clinics of North America 78:542–561.
Kimura R. S. (1979): Distribution structure & function of dark cells in vestibular labyrinth American journal of Otolaryngology 78:542–561.
Mangnuson M., Paloan S. (1991): Delayed onset of ototoxic effects of gentamicin in the treatment of Meniere’s effects of gentamicin in the treatment of Meniere’s disease, Acta otolaryngology (Stockh) 111:671.
Address for correspondence
Dr. K. K. Desarda
Benali, Karve Road, Nal Stop,
Pune 411 004, Maharashtra, India.

Contributed by Dr. K. K. Desarda


.


          TRAGAL CARTILAGE IN MIDDLE EAR RECONSTRUCTION


Desarda K. K.a Dr. Nilima. Kharade,Dr.Sheetal (ENT Residents)
Professor and head department of ORL, KEM hospital, Pune.
Chief residents department of ORL, KEM hospital.
This paper was read at AOI conference, Cochin, January 2000.

Email: kdesarda@gmail.com
Address:
Dr.K.K.Desarda.MS.FACS.DLO.(Lond)
Prof.Emeritus & Head Otolaryngology,
KEM Hospital, Pune

Abstract

Cartilage has become an alternative to more traditional grafting materials for the tympanic membrane reconstruction.  Vein graft was very popular for many years, but has been replaced by temporalis fascia.  Perichondrium and dura matter have also being used.  Currently temporalis fascia and perichondrium are most commonly materials used. Cartilage has shown itself to be a novel material with high success rate in more challenging cases such as retraction pockets, recurrent perforation, atelectasis, cholesteatoma and ossicular chain reconstruction

To date, temporalis fascia and perichondrium remain the most commonly employed materials for closure of tympanic membrane perforations.  The success rate in TM reconstruction with these materials approaches 90%. In certain situations, such as the atelectatic ear, cholesteatoma, and revision tympanoplasty, the results with these materials have not been as gratifying.  Fascia and perichondrium have been shown to undergo atrophy and subsequent failure in the postoperative period.  This has led to the use of cartilage, which is a less compliant, more rigid material that resists resorption and retraction.  It has also been shown by different studies that it is well tolerated by the middle ear and hearing results have been comparable with those of fascia and perichondrium

The study presents six hundred ear operations of varied middle ear pathology using tragal cartilage and perichondrium as a choice graft. The technical advantages of tragal perichondrium graft in myringoplasty, ossiculoplasty, ossiousplasty, and mastoid cavity obliteration are discussed.





KEM Hospital Pune.

The study was conducted at K.E.M. Hospital, ENT department during 1980 to 2000. we have recorded our observations and results and concluded that tragal perichondrium and cartilage is an ideal graft material for reconstructive tympanoplasty. The objective of study was to assess the efficacy of tragal perichondrium and cartilage, the functional capacity in restoring hearing acuity, it’s mechanical survival, it’s extrusion rate and it’s functional integrity in tympanomastoid reconstruction.

Keywords: Cartilage, Perichondrium, Sialastic.

Introduction




INTRODUCTION:


The technique of ‘Reconstructive Tympanoplasty’ has been improved and refined ever since the introduction of operative microscope. The methods of radical and modified radical mastoid operations have not changed for decades except for minor variations. The innumerable graft materials being used to restore the dry and functioning ear. The autologous, homologous and allograft, synthetic materials lik plastics, ceramics, hydroxyapatite and golds were used but none of these have established their universal acceptability as a proved graft except the autologous grafts (cartilage, ossicles, fascia). The functioning and survival of each graft material varies as each one has certain advantages ad disadvantages and technical problems during and after surgery.
Cartilage has become an alternative to more traditional grafting materials for the tympanic membrane reconstruction.  Vein graft was very popular for many years, but has been replaced by temporalis fascia.  Perichondrium and dura matter have also being used.  Currently temporalis fascia and perichondrium are most commonly materials used. Cartilage has shown itself to be a novel material with high success rate in more challenging cases such as retraction pockets, recurrent perforation, atelectasis, cholesteatoma and ossicular chain reconstruction.

To date, temporalis fascia and perichondrium remain the most commonly employed materials for closure of tympanic membrane perforations.  The success rate in TM reconstruction with these materials approaches 90%. In certain situations, such as the atelectatic ear, cholesteatoma, and revision tympanoplasty, the results with these materials have not been as gratifying.  Fascia and perichondrium have been shown to undergo atrophy and subsequent failure in the postoperative period.  This has led to the use of cartilage, which is a less compliant, more rigid material that resists resorption and retraction.  It has also been shown by different studies that it is well tolerated by the middle ear and hearing results have been comparable with those of fascia and perichondrium

 We present our experince of twenty years (1980–2000) in using ‘Tragal Cartilage and Perichondrium’ in the reconstructive tympanoplasty. This study includes 600 cases of varied middle ear pathologies grouped in to four main divisions such as myringoplasty, ossiculoplasty, ossiousplasty (for defects in attic, posterosuperior quadrant, posterior canal wall and annular defects) and cavity obliterations. This study is not a comparative study to prove the superiority of any particular graft material over another.



Principles of Cartilage Tympanoplasty

This study includes 600 cases of varied middle ear pathologies of both safe and unsafe C.S.O.M. All cases were treated conservatively for prolonged time before being subjected for reconstruction. The special attention was paid to Eustachian tube function. The relevant investigations as routine otomicroscopy, mastoid X–rays, paranasal sinus X–rays, audiometries and blood biochemistry were done.


Study Design: 600 Cases


The study was designed in four groups. Group A – Myringoplasty (n=300), Group B–Ossiculoplasty (n=110), Group C – Ossiculoplasty (n=120), and Group D – Mastoid cavity obliteration (n=70) All cases were subjected for reconstruction after eradicating the middle ear pathology by various surgical approaches. The enomeatal (n=192), endaural (n=312), postaural (n=60) and transtympanic (n=36). The age group was 15 to 55 years and males were predominant. Most of these cases were done under local anaesthesia with sedation (n=480) and smaller group under general anaesthesia (n=120).

During the study it was observed that the middle ear showed different pathologies such as perforations (n=240), adhesive otitis media (n=24), tympanosclerosis (n=36) and cholesteatomas (n=120). Statistical Analysis was done in SPSS 10.0 using chi–square test.


.
CLASSIFIED GROUPS:


Group A - Myringoplasty (n=300):


Out of 300 cases onlay grafting was done in 172 cases and inlay grafting was done in 128 cases. The tragal perichondrium and catilage was the choice graft used with excellent post of results. The success rate was 96% and failures 4% in this group. The hearing gain with SRT was achieved within 15 dB AB gap closure. The failure of 4% were subjected to revision surgery. The dry and healed middle ear was seen within three months time. The failure cases were attributed to infection, unhygienic conditions,prosthesis displacement, graft rejection and  poor follow–ups. In this group the follow up was 2 to 6 yrs. Audiometric thresholds revealed 15–20 dB A–B gap closure. The follow up was achieved in 50% of cases for 2 to 4 years.





Group B– Ossiculoplasty (n=110) (Fig.1–6):


In this group all cases were subjected for tympanomastoidectomy with ossicular reconstruction by tragal cartilage and perichondrium struts of various types as L–shape, Bow–shape and Boomrang strut. Various combinations of Incudo–stapedial assembly, malleo–stapes strut, malleo–footplate assemblies were done. In all cases sialistic sheet was used so also the anterior canal skin as covering the graft assembly. In this group the success rate was 84% and failure rate was 16%. The failures were due to infection, prosthesis displacements and extrusion of the graft. Audiometric thresholds revealed 15–20 dB A–B gap closure. The follow up was achieved in 50% of cases for 2 to 4 years.

The technique used for cartilage reconstruction with ossiculoplasty depends on the presence or absence of the malleus manubrium. In the malleus present situation, the palisade technique is very effective and also provides good acoustic benefit. The malleus-absent situation represents one of the most challenging situations for cartilage tympanoplasty and ossicular reconstruction. The perichondrium/cartilage island flap is used in these cases to prevent the prosthesis touching the tympanic graft and preventing extrusion. In these cases, the anterior portion of the cartilage is held securely in place while the posterior half is folded out to expose the trailing edge of the anterior piece of cartilage, which acts, in effect, as a neo-malleus. The distance between the stapes footplate or suprastructure and this trailing edge is measured and the prosthesis is cut to the appropriate length. The posterior portion is unfolded.  The nice thing about folding the prosthesis in half is that you can visualize the prosthesis and have precise placement.




  Different ossicular defects and their correction by cartilage struts.























Group C– Osseusplasty (attic, PSQ, PCW, annular defects) (n=70):





 



Attic,marginal,post.superior quadrants defects(cholesteatoma)


Cholesteatoma represents one of the most controversial but important pathologic conditions in which cartilage is used. The primary purpose of cholesteatoma surgery is to eradicate disease and provide a safe, hearing ear. The magnitude of the controversy regarding optimal surgical care is beyond the scope of this presentation, but cartilage should arguably be involved in each technique. The palisade technique has been very useful in the cholesteatoma setting as it gives the opportunity, if needed, to perform an ossiculoplasty in a precise way. Also, some authors prefer to leave the anterior portion of the TM without cartilage for surveillance and possible tube placement, if necessary, in the postoperative period. However, cartilage placement in the posterior aspect of the TM can certainly delay a recurrence. but, in most series, cholesteatoma will recur in the anterior portion of the TM and it can be suspected in the setting of a recurrence in conductive hearing loss. After my review of literature, I found that the recurrence rate for cholesteatoma after cartilage tympanoplasty is less than 10%.  And if we compare this rate with cholesteatoma recurrence rate overall in children, we can appreciate that is much lower than rates previously reported in the literature which ranges from 10-46%

In this group the various defects of attic, posterosuperior quaderants, posterior canal wall and annular defects were closed by tragal perichondrium and cartilage grafts. The composite graft proved to be the best than nonbiological grafts in takeup and restoring dry ears. The cholesteatoma from the defect was removed and the defect was closed with the grafts. The posterior canal wall defect was reconstructed with the tragal cartilage graft and lined by perichondrum and anterior canal wall skin. This group achieved 75% success rate and 25% were failures which needed revision surgery.



Group D– Mastoid obliterations (n=120):




Mastoid obliterations

All mastoid cavities were preoperatively treated by suction clearance, dry mopping with antifungal and antibiotic drops for about 4–6 weeks. The cavities were fashioned by smooth drilling and removing all debris, pockets of cholesteatomas etc. the tragal cartilage was arranged in the palisade manner with the perichondrium coverage and the pedicled temporalis muscle was swinged to obliterate the mastoid cavities for good healing. Periodical follow up and aural toilet were done. The cavities re–epithelised well and achieved 70% success rate. 30% failures was because of infection and poor post op. follow ups. The modified radical mastoidectomy cavities were transformed into radical cavities to achieve good healing. The problems of mastoid cavities are still unresolved despite the treatment of various modified techniques being weak.

Table I – Age group in the study:
Age group (years) No. of cases Percent
15–25 168 28
25–35 264 44
35–45 120 20
45–55 48 08
Total 600 100

Table II: Sex distribution:
Age group (years) Male (no.) Female (no.) Total
15–25 108 60 168
25–35 120 144 264
35–45 72 42 120
45–55 24 24 48
Total 324 276 600
Percent 54 46 100


Table III – Surgical approaches:
Approaches No. of cases Percent
Endaural 312 52
Endomeatal 192 32
Postaural 60 10
Transtympanic 36 06
Total 600 100

Table IV – Anaesthesia:
Anaesthesia No. of cases Percent
General 120 20
Local + Sedation 480 80
Total 600 100


Table V – Pathological defects:
Type of Pathology No. of Cases %
Performation 240 40
Adhesive Otitis media 24 04
Tympanosclerosis 36 06
Retration pocket 180 30
Cholesteatoma 120 20
Total 600 100


Discussion:

Cartilage has become an alternative to more traditional grafting materials for the tympanic membrane reconstruction.  Vein graft was very popular for many years, but has been replaced by temporalis fascia.  Perichondrium and dura matter have also being used.  Currently temporalis fascia and perichondrium are most commonly materials used. Cartilage has shown itself to be a novel material with high success rate in more challenging cases such as retraction pockets, recurrent perforation, atelectasis, cholesteatoma and ossicular chain reconstruction




Pathological-Defects

For many years the so called conservative methods of radical mastoid operations (Barany, Bondy, Citelli, Heerman, Stacke) were done in the clearance of disease but none of these proved better. At later dates Farrior, House, Lempert, Morrison added some minor variations in the technique of reconstructive tympanoplasty but still could not achieve the good results because the recurrence of the disease was very high. To modify these Victor Goodhill, Heerman and Heerman demonstrated their new techniques which prevented the recurrence of the cholesteatoma and gained the high success rates.


Distinct Advantages of Tragal Cartilage Graft

In this study 600 ear operations were performed with tragal perichondrium and cartilage as a composite graft in various types of middle ear reconstructions such as myringoplasty, ossiculoplsty, osseous reconstructions and mastoid obliterations. This study was done at K.E.M. Hospital E.N.T. department, Pune during 1980–2000. We have presented our observations of this reconstructive study of 20 years and found that the tragal cartilage is an ideal graft for the reconstructive middle ear surgery.

In the simple myringoplasty group the tragal perichondrium and cartilage achieved 96% success rate, the small, large and subtotal central perforations healed well in six weeks time. The inlay and onlay methods were used in the neotympanic reconstruction. In the total perforations and missing annulus the perichondrium angle was appropriate fit in forming the new annulus the perichondrium angle was appropriate fit in forming the new annulus. By this technique the blunting and lateralisation of the graft was prevented from the various cartilage assemblies in ossicular reconstruction achieved excellent stability and contact to bridge the gap in transformer mechanism. The incudostapedial gap was restored by cartilage sturt and maintained assembly. The malleostapedial, malleofootplate assembly proved good in restoring hearing.
 I

Ideal Graft

In TORP. & PORP ossicular graft the interposed tragal cartilage and drum have increased the ossicular stability and improved hearing to 75% (Victor Goodhill). Chronic endotympanic depression is a pathological entity which leads to atelectasis, retraction pockets and cholestestoma formation. The tragal cartilage and perichondrium composite graft intervention has prevented the recurrence of the cholesteatoma pocket adhesions and tympanosclerosis. The postop results were dryhealed middle ears with good hearing.



Results of Cartilage Tympanoplasty

During the study it was observed that middle ear patology of 40% perforaytions of safe and unsafe types, 4% adhesive otitis media, 6% tympaosclerosis, 30% retraction pockets and 20% cholesteatoma sacs. All these pathologies were corrected by radical removal and tragal cartilage reconstruction.

In mastoid obliteration the palisade cartilageplasty proved in gaining dry cavities in 70% of the cases. The Eustachian tubal obstruction was relieved by tunnelplasty and improved the good middle ear aeration. The cartilage bridge over promontory and hypotympanum assures the proper contact with stapes and in the combined approach tymparoplasty procedure the recurrence of cholesteatoma in the sinus tympani and facial recess could be prevented by incorporating the composite tragal cartilage and perichondrium. In open cavities the tympanocartige stapedopexy improved the hearing. It was our observation that biological material like tragal cartilage, perichondrium, facia or ossicles etc. are much better than nonbiological materials in reconstructive surgery.



Poor Results in Cartilageplasty


The survival rate of tgragal graft material is much better than nonbiological materials.

The extrusion rate of cartilage is very minimal as compared to the other graft materials. The review of literature revealed the different extrusion rates of different materials, such as autologous, 1.19%, isografts 3.06% the synthetics 5.04%, human dentine 7.14%, gold prosthesis 8.7%. Overall the tragal cartilage and perichondrium proved to be the best graft materials in reconstructive tympanoplasty which is universally accepted.




Table VIII :Extrusion rates of commonly used graft materials:
Graft material Extrusion rate (%)
Autograft 1.19
Isograft 3.06
Synthetic 5.04
Human Dentine 7.14
Gold prosthesis 8.70

Table IX :Results of cartilage tympanoplasty: P=0.0001 by chi square:
Group Success (%) Failure (%)
Myringoplasty 96 04
Ossiculoplasty 84 16
Osseusplasty 75 25
Mastoid obliteration 70 30

Table X – Poor results in cartilageplasty:
Causes No. of cases
Displacement 12
Fibrosis 10
Absorption 06
Infection 08
Total 36

TABLE V – Pathological Defects:
Type of Pathology No. of Cases Percet
Performation 240 40
Adhesive Otitis media 24 04
Tympanosclerosis 36 06
Retration pocket 180 30
Cholesteatoma 120 20
Total 600 100


Table VI – Ossiculoplasty:
Lesion No. of cases Percent
Attic 55 50
Posterosuperior quadrant 33 30
Posterior canal wall 11 10
Eustachian tube 11 10
Total 110 100

Table VII – Ossicular Status (300 cases): –P=0.0001 by Chi square:
Structure Normal Eroded Destroyed
Maleus 120 72 108
Incus 000 96 204
Stapes 108 00 192










 Conclusion

 The use of cartilage is experiencing a renaissance in ear surgery because it appears to offer an extremely reliable method for reconstruction of the tympanic membrane in cases of advanced middle ear pathology and Eustachian tube dysfunction.
Cartilage is particularly useful for the atelectatic ear, cholesteatoma, high risk perforation and for reinforcement of the tympanic membrane in conjunction with ossiculoplasty.
Hearing improvement can be experienced with the use of cartilage regarding the underlying pathology. Excellent clinical and experimental evidence exists to justify the use of cartilage as a grafting material in pediatric tympanoplasty. Cartilage tympanoplasty provides a tympanic membrane repair with greater structural stability and strength than traditional graft materials in many patients with challenging middle ear environments
In view of the above study we strongly recommend the tragal perichondrium and cartilage composite graft in various tympanoplasty reconstructions. The main reason being the cartilage is easily available at the site of operation, nontoxic, less, extrusion, minimum shrinkage, and lateralisation above all it is very cost effective to our patients. The hearing improvement within 15db of bone conduction has become almost a standard criterion for the analysis of surgical success.







Extrusion Rate of Commonly Used Graft Material


References:

Aeaham Evitor and Bronx NY: Tragal perichondrium and cartilage in reconstructive ear surgery, Laryngoscopy, 88 (Suppl.): 1–23,1978.
Heerman and Heerman tympanoplasty and mastoidoplasty, Laryngorhinootology, 46:370–382, May 1968.
Plester D.: Myringoplasty methods, Archieves otolaryngology, 78:310–316, Sept.1963.
House H. P.: Surgical repair of the perforated drum, Annales otorhinolaryngology, 62 : 1072–1093, 1956.
Goodhill Victor, Harris I., and Brockman S. J.: Tympanoplsty with perichondrium graft, Archieves otolaryngology, 79, 131, 1963.
Claus Jansen : Cartilage tympanoplasty, Laryngoscope, 73: 1288, 1963.
Heerman and Heerman: Fascia and cartilage palisade tympanoplasty, Archieves otology, 91 : 228–241, 1970.
Victor Goodhill: Tragal perichondrium and cartilage in tympanoplasty, Archieves otology, 85:480–491,1963.
Jansen C.: Use of perichondrium for tympanoplasty, Archives ohren, 182:610–614, 1963.
Shea J. J.: Vein graft in tympanic reconstruction, Journal of laryngootology, 74:358–362, 1960.
Contributed by Dr. K. K. Desarda



                             PRIMARY  RHINOSPORIDIOSIS  OF NASOPHARYNX.

                                  DR.K.K.DESARDA. DR. SHEETAL.DR.NEELIMA.
                                                       KEM HOSPITAL PUNE.


                  
Abstract.

A rare case of primary Nasopharyngeal rhinosporidiosis with extension to nasal cavity,maxillary sinus anteriorly and posteriorly extending to oropharynx is reported in a young male patient. The pathophysiology, clinical feature, diagnosis and management of this condition are reviewed.
Key words: Rhinosporidiosis, Rhinosporidium Seeberi

Indroduction:

Rhinospordiosis is a chronic granulomatous disease characterized by production of polyps or other manifestations of hyperplasia on mucous membrane surfaces, the etiologic agent being Rhinosporidium seeberi. The disease was First described  by seeber (1900) in Argentina. This noval pathogen commonly affects mucosa of nose, eye and upper aero-digestive tract of men and animals. Isolated deep seated Rhinosporidiosis is rare. Diagnosis is mainly based on clinical suspicion and histopathological confirmation. At present, no existing medical treatment cures the disease and endoscopic excision of the mass with cauterization of the base is considered as treatment of choice.

Case Report

A 45 year old male patient came to us with history of left nasal obstruction,epistaxis, distorted speech with nasal twang and intermittent dysphagia and headache for over 6 yrs He was treated at the local district hospital but without great relief.
The patient did not give any history of TB,DM and any majot illness in past apart from nasal obstruction , intermittent nose bleeds,and nasal speech with poor intake of food.. patient was chchectic with mild pallor, with a pulse rate of 84/minute, regular, BP-100/80 mmHg, CVS-NAD, Chest:-NAD.


Investigations:

Hb%-8.0gm% ,TLC 8000/mm ,DC-N80%,L-18%,E-2%,ESR-5mm.
Serum urea 30mg%,serum creatinine-1.42%,Urine –NAD,
AbsAg –negative,Anti HCV-negative,Elisa for HIV-negative,serum Bilirubin0.6mg%
SGPT and SGOT within normal limits

DC:-N-82%, L-17%, E-01%
FBS:-106 mg%
ESR:- 5mm in 1st hr,
Sr.
Radiological investigations: x-ray chest NAD,CT coronal PNS revealed Hughe mass occupying left maxilla,OMU blocked with mass. The mass was occupied in the nasal cavity, nasopharynx with extension to oropharynx.FNAC done for HP. Examination.which revealed rhinospordiosis.
Nasal endoscopy revealed pinkish mass occupying left nasal cavity,nasopharynx and left maxilla. and mass was,protruding in to oropharynx ,pushing the palate anteriorly.The septum was pushed to right   causing  nasal obstruction.. Clinical diagnosis of Rhinospordiosis.was made.
Patient was advised surgery and subsequently undergone endoscopic excision.
Nasal endoscopy revealed pinkish mass occupying left  nasal cavity,nasopharynx and protruding in to oropharynx,pushing the palate anteriorly. The mass bled during the clearance. Complete endoscopic clearance of the naso-maxillary-and nasopharynx was done. The complete haemostasis secured.by cauterization.The anterior and post nasal packing with BIPP was done .The  enblock specimen was sent  for histopathological examination. Post op recovery was uneventful.
Patient was given broad spectrum antibiotic,anti inflammatory agents and Dapsone theray . He was advised to continue Dapsone theray 100mg OD for six months and attend follow up clinic every 3 months for any recurrence.
    Rhinosporidosis  post surgery specimen.
   
  Enblock  resection of  Rhinosporidium of Naso-oro pharynx.
    Histopathlogy:
                                             
 H&E stain of nasal polyp showing two mature sporangia and
several immature ones with a single centrally placed spore.



.
DISCUSSION:

Rhinosporidiosis is a chronic granulomatous disease characterized by production of polyps or other manifestations of hyperplasia on mucous membrane surfaces. The etiologic agent is Rhinosporidium seeberi.Most of the early studies of rhinosporidiosis were made in India and Ceylon where the disease occurs frequently. Sporadic case have been detected and studied in many parts of the world. The systematic position of R. seeberi is still uncertain. Most investigatrs consider it has not been isolated in culture.
 Friable, highly vascular, sessile or pedunculated polyps may appear on almost any mucosal surface, and rarely secondary lesions are found on skin, probably as aresult of autoinoculation by scratching. Lesions of the mucosae often spread by extension beyond the mucocutaneous border.
Primary lesions appear most often on the nasal mucosa and are accompanied by painless itching and a profuse mucoid discharge. The lesion is at first flat and sessile. Later hyperplastic growth greatly exceeds lateral extension of the lesion so that a polypoid mass much larger than the peduncle develops. The polyp may extend from the neres into the pharynx or externally over the lip and may reach weight of 20grams. It is friable and bleeds freely after trauma. Its surface is mucoid and papillate or so lobulate that its surface suggests that of a cauliflower. The color varies from pink to purplish red, and close examination of the surface mayh reveal minute white sports which are the mature sporangia of the fungus.
Lesions of the eye may cause symptoms similar to those produced by a foreign body, lacrimation or photophobia. Growth of the polyp may cause eversion of the lid. Lesions on th eskin being as papillomas and become warty with inclusions of myxomatous material. They are relatively painless except when on the sole of the foot and when they become so large as to be uncomfortably heavy. Dissemination to visceral organs is rare.

Differential diagnosis:

Typical lesions of rhinospordiosis can be recognized usually by the pink to purple colour, friable consistency and the presence of barely visible white sporangia within the polyp. Atypical lesions or those in unusual anatomical sites must be differential from warts, condylomata and hemorrhoids.

Immunology:

Little is known about the immunology of rhinosporidiosis.
Pathology:
H&E stain of nasal polyp showing two mature sporangia and
several immature ones with a single centrally placed spore

On the examination of the gross tissue, unless rhinosporidiosis has been suggested by the clinician, or by the history of the patient's geographic residence, the pathologist may consider the specimen an ordinary nasal polyp. The correct diagnosis can usually be made without difficulty on examination of routine H and E stained slide. Under the scanning lens of the microscope, although the polypoid structure may be evident, the histopathologic pattern differs greatly from tht of the common nasal polyp. The most striking feature is the presence in the stroma or epidermis of numerous sharply defined globular cysts which usually vary from 10 to 200.. Some of the cysts may be partly collapsed, assuming a semilunar shape. In contrast to the loose, edematous, myxomatous stroma of the ordinary nasal polyp, the stroma in rhinospordiosis is rather dense. There is a chronic inflammatory reaction in which neutrophils, plasma cells and lymphocytes are prominent. In contrast to the usual nasal polyp. Eosinophils are inconspicuous. Occasionally purulent microabscesses occur.
The cysts of all size have a sharply defined chitinous appearing wall. In a large maturing cyst the wall alone may be 5  thick. Histologically, rhinosporidiosis should be differentiated, specially in immuno-suppressed persons with other fungal infection like Coccidioides immitis.

Epidemiology:

Although rhinosporidiosis is seen most often in children and young adults, it occurs at any age. No racial difference in susceptibility are recognized. The disease is seen much more frequently in men than in women, but the extent to which this difference is related to greater frequency or severity of exposure is difficult to evaluate. Infections are seen most often in labourers and in those with frequent exposure to water of streams and pools.

Geographic distribution:
Rhinosporidiosis is found must often in India and Ceylon, but it is reported also from the East Indies, the Malay, States, the Philippines, Iran, South Africa, Italy, England, Scotland, Southern United States, Mexico, Cuba, Argentina, Brazil and Paraguay.
Source of infection:
The disease is not contagious, and sources of infection are exogenous. The frequent history of prior extended to water of pools and rivers and the occurance of multiple cases among those members of a group of workmen most intimately and repeatedly exposed to water source suggest the R. seeberi has a natural habitat in water. Rhinosporidiosis was observed in workmen who dived under water to bring up san din buckets, but not in their associates who carried the sand from the water's edge. It has been suggested that water insects or fish may be hosts of the fungus.

Laboratory diagnosis:

Direct examination of the surface of the polyp may reveal the subsurface position of sporangia which are white and so large (up to 350  in diameter) that thery can be seen with naked eye. Dissection of sporangia or excision and microscopic examination of tissue confirms the diagnosis. Culture is not successful, and the inability of R. seeberi to grow on artificial media, as well as some peculiarties about its reproductive cycle in tissues, have raised the question whether it is actually a fungus.
It is resembles in general appearance and in manner of sporulation some species of synchytrium, which are obligate parasites of plants, and which produce characteristic galls on the host plant. Animal inocultion is not helpful in diagnosis. Although R.seeberi is found in natural infections of horses, mules and cows, experimental infections usually do not succeed. Recently molecular methods like polymerase chain reaction are being developed for diagnosis.

Conclusion:
 Rhinosporidiosis, a fungal infection due to Rhinosporidium seeberi, frequently produces polypoidal lesions in the nose. Sites like the conjunctiva, larynx, trachea, nasopharynx, skin and genitourinary tract are less frequently involved. Generalized rhinosporidiosis with skin and visceral involvement is extremely rare... Smears revealed numerous sporangia and spores of R seeberi. There were no mucocutaneous lesions. Histologic examination confirmed the  diagnosis of Rhinospordiosis.  The FNAC diagnosis of rhinosporidiosis is specific. Preoperative diagnosis is possible even in cases with unusual clinical presentations.
Rhinosporidiosis should be suspected or considered in all cases of swellings of nose,
 nasopharynx and skin. Although disseminated Rhinosporidiosis is very rare, still
remains a possibility and requires a different mode of treatment. Presently the medical
treatment of Rhinosporidiosis is not satisfactory and requires further study and
research.Meanwhile patients should receive Dapsone therapy for over six months.


References:
1. Caldwell, G.T. and Roberts, J.D.: Rhinosporidiosis in the United States J.A.M.A. 1938; 110,1964.
2. Karunaratne W.A.E.: Rhinosporidiosis in Man, London, Athlone Press, 1964.
3. Weller, C.V. and Riker, A.D.: Rhinosporidiosis in Man, London, Athlone Press, 1964.
4. Weller, C.V. and Riker, A.D.: Rhinosporidiosis seeberi Am.J.Path, 1930,6,721-732.
5. Baron, E.J., Peterson, L.R. Finegold, S.M. New, Controversial difficult-to-cultivate or non-cultivate etiological agents of disease in Bailey and Scott's Diagnostic Microbiology, 9th Edition, Mosby, st.Louis, Baltimore, Boston, 1994; p-585.




DIODE LASER TREATMENT IN ORAL SUBMUCOUS FIBROSIS.
(KEM-PUNE STUDY)
Dr.K.K.DESARDA.
Abstract:
Oral submucous fibrosis (OSMF) is a high-risk pre-cancerous condition characterised by slowly progressive chronic fibrotic disease of the oral cavity and oropharynx, in which the oral mucosa loses its elasticity and develops fibrous bands, which ultimately leads to difficulty in opening the mouth. The malignant transformation rate of oral submucous fibrosis is as high as 7.6%. A wide range of treatments such as medical management, surgical therapy and physiotherapy have been attempted in the past with varying degrees of benefit, but none of them have been proved to be a conclusive method of treatment. . There are very few reports to correlate the clinical stage to histopathological grading in OSMF.The aim of our study was to relive trismus caused by submucous fibrosis,to evaluate the efficacy of diode laser therapy without any grafting procedures, and to maintain mouth opening with props physiotherapy.
 A hospital-based study was conducted on 65 OSMF cases who visited ENT dept. KEM Hospital Pune from 1990 -1996. A detailed history of each patient was recorded along with a clinical examination. Biopsy was performed for histopathological correlation.We have  tried all modalities like diathermy excision,skin grafts.tongue flaps,palatal flaps but without much  benefits in improving trismus for our patients  and  lastly  we adopted  Diode surgical therapy  with spring props for physiotherapy  which proved excellent  treatment in improving trismus.
Key words: submucous fibrosis, Diode laser,props,skin grafts,tongue flaps.
Email:kdesarda@gmail.com
Adress: Dr.K.K. desarda.
Prof.Emeritus & Head Otolaryngology,
KEM Hospital’Pune, India.
Inroduction:
On the Indian subcontinent, the use of smokeless tobacco in various forms is very popular. This habit, which usually involves the chewing of a betel quid (combined areca nut, betel leaf, tobacco and slack lime), has led to the development, in a large proportion of users, of a unique generalized fibrosis of the oral soft tissues, called oral submucous fibrosis.The condition is found in 10/1,000 adults in rural India and as many as 5 million young Indians are suffering from this precancerous condition as a result of the increased popularity of the habit of chewing pan masala.  Pan masala is a mixture of spices including, betel nuts, catechu, menthol, cardamom, lime and others.  It has a mild stimulating effect and is often eaten at the end of the meal to help digest food and feel comfortable.
Oral submucous fibrosis is a chronic debilitating disease of the oral cavity characterized by inflammation and progressive fibrosis of the submucosal tissues (lamina propria and deeper connective tissues).  Oral submucous fibrosis results in marked rigidity and an eventual inability to open the mouth.  The buccal mucosa is the most commonly involved site, but any part of the oral cavity such as the soft palate, pterygomandibular raphe, the anterior pillars of fauces and even the pharynx can be involved.
 The treatment of patients with oral submucous fibrosis depends on the degree of clinical involvement. If the disease is detected at a very early stage, cessation of the habit is  sufficient. Most patients with oral submucous fibrosis present with moderate-to-severe disease which is irreversible. Medical treatment is symptomatic and predominantly aimed at improving mouth opening.. We have  treated stage II  with medical treatment and injection kenacort for six weeks and  Stage III  & iv with severe fibrosis + trismus + dysphagia with Dioded laser therapy and achieved excellent results with inter-incisor opening ranging between 32 to 36 mm .
Because of developments in Diode Laser technologies, it has found great applications in surgery due to improved power and precise controllability. It has found great applications in oral surgery practice as well as in other areas. By changing the wavelengths we can control the energy levels and other desired properties that determine incision quality and coagulation parameters.
PATHOGENESIS OF ORAL SUBMUCOUS FIBROSIS:
    ;

Diode lasers built with semiconductor materials are portable and very compact in size and can be used in different modes such as pulsed or continuous mode. Diode laser surgery can be successfully used in surgical treatment of Submucous fibrosis. On the Indian subcontinent, the use of smokeless tobacco in various forms is very popular. This habit, which usually involves the chewing of a betel quid (combined areca nut, betel leaf, tobacco and slack lime), has led to the development, in a large proportion of users, of a unique generalized fibrosis of the oral soft tissues, called oral submucous fibrosis.The condition is found in 10/1,000 adults in rural India and as many as 5 million young Indians are suffering from this precancerous condition as a result of the increased popularity of the habit of chewing pan masala.  Pan masala is a mixture of spices including, betel nuts, catechu, menthol, cardamom, lime and others.  It has a mild stimulating effect and is often eaten at the end of the meal to help digest food and as a breath mint.1
Oral submucous fibrosis is a chronic debilitating disease of the oral cavity characterized by inflammation and progressive fibrosis of the submucosal tissues (lamina propria and deeper connective tissues).  Oral submucous fibrosis results in marked rigidity and an eventual inability to open the mouth.  The buccal mucosa is the most commonly involved site, but any part of the oral cavity such as the soft palate, pterygomandibular raphe, the anterior pillars of fauces and even the pharynx can be involved.The condition is well associated with areca nut chewing; a habit practiced predominately in Southeast Asia and India. Worldwide, estimates of oral submucous fibrosis indicate that 2.5 million people are affected, with most cases concentrated on the Indian subcontinent, especially southern India.  The rate varies from 0.2-2.3% in males and 1.2-4.5% in females in Indian communities.  The migration of endemic betel quid chewers has also made oral submucous fibrosis a public health issue in many parts of the world, including the United Kingdom, South Africa, and many Southeast Asian countries.
A variety of aetiological factors including capsaicin, betal nut alkaloids, hypersensitivity, autoimmune genetic predisposition  and malnutrition have been suggested by various authors.The importance of this disease lies in its inability to open the mouth and dysplasia giving rise to malignancy.  The incidence of malignant change to squamous cell carcinoma in patients with OSMF ranges from 2 to 10%. Submucous fibrosis typically affects the buccal mucosa, lips, retromolar areas and the soft palate. Occasional involvement of the pharynx and esophagus is seen. Early lesions present as a blanching of the mucosa, imparting a mottled, marble-like appearance. Later lesions demonstrate palpable fibrous bands running vertically in the buccal mucosa and in a circular fashion around the mouth opening or lips.  As the disease progresses the mucosa becomes stiff, causing difficulty in eating and considerably restricting the patient's ability to open the mouth (trismus). If the tongue is involved, it becomes stiff and  atrophic.
Mucosal petechiae are seen in more than 10% of cases and most patients complain of a burning sensation, often aggravated by spicy foods.  Salivary flow is diminished and blotchy melanotic mucosal pigmentation is often seen. More than a fourth of affected persons develop precancerous leukoplakia of one or more oral surfaces. Once present, oral submucous fibrosis does not regress, either spontaneously or with cessation of betel quid chewing.Histologic findings in oral submucous fibrosis are generally characterized by diffuse hyalinization of the subepithelial stroma, atrophic epithelium and intercellular oedema, with or without keratosis, epithelial dysplasia, chronic inflammation and fibrosis in the minor salivary glands in the area of quid placement; and atrophy of the underlying muscle.
MATERIALS AND METHODS
  A total of 65 histologically proven cases of advanced oral submucous fibrosis having a mouth opening of less than 10 mm were treated by Diode laser excision. The procedure involved (1) bilateral release of fibrotic bands  .(2)extraction of bilateral upper and lower molars .3) Temporary acrylic prosthesis insertion between upper and lower molars bilaterally. 4) after six weeks patients were given spring prosthesis for extensive physiotherapy. 5)  Pre and post operative inter-incisors distances were measured  by caliper and recorded.6) supportive IV fluids, vitamins. and antioxidants therapy was continued for further three months. 7) Regular followup was done and results were assessed by comparing the  different modalities  responses in preoperative & postoperative maximum mouth opening.8) No grafting  procedures were  done in our series for the scarred tissue. 9) Post op healing was excellent.
        TABLE-1, Age group & sex Ratio:
       
     
  TABLE-2. Clinical staging and grouping:
Group I Earliest stage witht mouth opening limitations with an interincisal distance of greater than 35 mm.
Group II Patients with an interincisal distance of 26-35 mm.
Group III Moderately advanced cases with an interincisal distance of 15-26 mm.
Group IVA   Trismus is severe, with an interincisal distance of less than 10 mm
Group IVB Disease is most advanced, with  dysphagia  & premalignant and malignant changes in the mucosa
 
      TABLE-3 Grouping of Different modalities treatment result wise:
Grouping Procedure Cases Result
Group 1 Simple diatheramy excision                 25 cases             Triusmus opening short term with scarring
Group 2 Skin grafting                                       15 cases             High failure because of flap necrosis
Group 3       Tongue flaps                                       10 cases             Initial results good but very cumbersome for pts. Tongue flap necrosis, speech problems
Group 4 Diode laser therapy                           15 cases             Excellent long term results with improved                                    

Pre-Operative pictures of OSMF
                                       
Early branching in OSMF                Depapilliation of Tongue             severe blanching of tongue                   Bud-like uvula in OSMF        
                                                                   
                                     
        Buccal Lesion                                  Lower lip lesion                                Tongue lesion                              Retromolar lesion.

      Diode Laser Procedure:.

Under GA with tonsillectomy position the mouth gag(Boyle Davis) is inserted inthe oral cavity and the last upper and lower molars on either sides extractedand haemostasis secured. Next with Diode Laser the fibrotic bands from retromolar region to soft palate ,anterior and posterior tonsillar pillars and uvula were excised.Laser beam with ideally 5 watt power is directed. to the fibrotic bands .The excision of fibrous bands was followed by forcible separation of mucosa.using  Boyl davis gag  and oral cavity  opening stretched .at its maximum..After complete haemostasis the oral cavity is cleaned and temporary acrylic prosthesis is inserted  bilaterally between the last  upper and lower molars., and maintained for full 10 days.Post operative Ryles tube feedig continued for ten days . During the post op period  oral hyegine was mainted. After the oral mucosa has healed well ,the patients were given  spring props for for extensive physiotherapy.for further six weeks.All Patients were instructed to stop chewing betel nuts or other addictive habits. Patients were taught mouth opening exercises with this spring props six to eight times day along with chewing gums exercises.The inter- inscisor measurement were recorded. All patients were given i.v.antibiotics,anti inflammatory drugs for controlling the oral sepsis and pain . Patients were followed at an interval of 2 months, 6 months and 1 year where by interincisor distance was measured and documented..
Results
In our series  65 patients were studied from age 16 years to 60 years (Mean 32years) who were habitual betel nut chewers. Males had preponderance of 68%and females of 32%. The Mean maximum mouth opening of the patients preoperatively was 10 mm. The Mean intra operative interincisor distance after fibrotic band excision was 32mm. Two months postoperatively the average mouth opening was 34 mm.and at 6 months 36 mm, and at 1 year 34mm. Patients were very happy and satisfied after this treatment.Since there is no effective treatment for oral submucous fibrosis and the conditionis  is irreversible  we have decided to adopt to Diode laser therapy which gave excellent results. All patients were kept for periodical observation and  surface leukoplakias are handled by close follow-up and  repeat biopsies for malignment change.

Submucous Fibrosis pictures showing post-operative
                                                     
  Diode laser instrument            Pre-surgical Markings             Intra of Temporary prosthesis                   Post of spring prosthesis       Post operative final opening    
     Discussion
Oral submucous fibrosis is a chronic debilitating disease associated with restricted mouth opening and poor oral hygiene.   The treatment aims at good release of fibrosis and provides long term results in terms of mouth opening.  The various conservative treatments with intralesional injections of steroids,  (kenacort) ,hyaluronidase, placental extract and physiotherapy are not beneficial to provide a long-term effect in advanced cases of oral submucous fibrosis   Surgical intervention is required in these cases.  The surgical treatment commonly followed is the release of bilateral fibrotic bands with  surgery and various grafts, tongue flaps, etc were tried but not much of benefit.
A mucosal graft is the best treatment for oral submucous fibrosis, as it is ideal graft to cover the oral mucosa, but is limited by the quantity of oral mucosal available for grafting.  Thick mucosa taken from the cheek may result in scar formation, whereas a uniform thin graft removed with a microtome from the cheek is costly and complicated.  Split-skin grafting has been tried but it has a high failure rate as fibrotic areas have less vascular supply besides retaining the coloration of skin.  Also seen is the growth of hair and sweat glands.  Skin is not suitable for grafting in elderly people due to atrophy and inelasticity.
A nasolabial flap has also been used by some surgeons and has a good survival rate, but sometimes it may be too small to cover the whole defect.  It also causes a visible scar on the face and requires a second surgery for division.   Tongue flaps are bulky and when used bilaterally causes disarticulation, dysphagia and increases the chance of aspiration.   In addition, the tongue is involved with the disease process in 38% cases   The use of bilateral, small, bipaddical radial forearm flaps for reconstruction of bilateral buccal defects requires two flaps with two microsurgeries.  The procedure is more time consuming and technically demanding, and it involves two forearm donor sites with sacrifice of the radial arteries in both the right and left hands.  Island palatal flaps again have limitation to reach posteriorly.
Of the 65 cases of OSMF studied, males were more than females. A literature survey shows a wide variation in age and sex distribution of OSMF. Some of the epidemiological surveys in India have shown a female predominance in the occurrence of this entity. A male predominance in OSMF cases was shown by Sinor et al.in India. We also observed a male predominance and the male to female ratio was 6:1. Half of the study population was in the age group of 20-29 years. This observation is different from that of Pindborg et al. who reported the maximum number of OSMF cases in the age group of 40-49 years in their study. Increase in the chewing habit of the areca nut without any tobacco and the use of various commercial products containing areca nut may explain the decrease in the age of OSMF cases due to various chewing habits. The mean age of occurrence was lower in males than in females and the difference was statistically significant (P<0.009).

Recent epidemiological studies in India and evidence from Indians living in South Africa point to the habit of chewing areca nut as the major aetiological factor of OSMF. In recent years, commercial preparations like paanmasala have become available in India and abroad. The main ingredient of these products is areca nut along with lime and catechu wrapped in a betel leaf with or without tobacco. Many patients with OSMF give a history of chewing paanmasala for very long time.
Seventy-five per cent of the patients in stage II had a habit of chewing commercially available areca nut products-"Paanmasala" and 50% of the total study population were in the age group of 20-29 years. It has been documented that paanmasala chewing was preferred by people in younger age groups (11-30 years). In addition, onset of OSMF changes occurred earlier with paanmasala chewing compared with areca nut / quid chewing. Absence of betel leaf, which has anti-oxidant properties and a consequently higher dry weight proportion of areca nut were responsible for early development of OSMF. These findings are of great concern because younger individuals are at greater risk as it has been well established that OSMF is a premalignant and crippling condition of the oral mucosa.
 The treatment aims at good release of fibrosis and provides long term results in terms of mouth opening.  The various conservative treatments with intralesional injections of steroids, hyaluronidase, placental extract and physiotherapy are not beneficial to provide a long-term effect in advanced cases of oral submucous fibrosis   Surgical intervention is required in these cases.  The surgical treatment commonly followed  in our series is the release of bilateral fibrotic bands with Diode Laser.with extraction of both upper lower molars  followed by post op.props for  extensive physiotherapy. We have achieved excellent results in all cases.
Conclusion
In this study, the occurrence of OSMF was higher in the younger age group of 20-29 years. The prevalence of OSMF was more in males than in females with a ratio of 6:1. The number of patients with a paanmasala chewing habit (68.0%) was higher than the number of patients with betel nut (17.4%) or betel quid chewing habits (14.6%). The chewing of paanmasala was associated with earlier presentation of OSMF as compared to betel nut chewing. Significant and direct correlation to the manifestation of OSMF was seen with frequency rather than duration of chewing.

The maximum number of patients (74.3%) as well as most of the paanmasala chewers were in clinical stage II. Although various degrees of epithelial dysplasia were observed, malignant transformation was not seen. There was no correlation between clinical staging to histopathological grading. This observation could be explained by the fact that patients with higher histopathological grading could have had more collagenous bands in the posterior region, which restricted the mouth opening. Chronic inflammatory cell infiltrate was observed in a large number of cases in histopathological grade I but less so in higher histopathological grades, possibly due to a stabilisation of the lesion and a decrease in the levels of proinflammatory mediators.   The follow up examinations after the surgery showed significant improvement in  mouth opening . The key point was extensive physiotherapy to sustain the mouth opening.  There is no doubt that diode laser surgery is very effective and less invasive technique to treat Submucous fibrosis and offers great relief to the terrible state the patients suffer because of this disease .This technique has less morbidity and is suitable for Asian population as it requires less hospital stay and less followup as compared to other surgical methods.
 
References:
1. Schwartz J. Atrophia Idiopathica Mucosae Oris. London: Demonstrated at the 11th Int Dent Congress; 1952.
2. Joshi SG. Submucous Fibrosis of The Palate And Pillars. Ind J Otolaryng 1953; 4:1-4.
3. Canniff JP, Harvey W, Harris M. Oral submucous fibrosis: its pathogenesis and management. Br Dent J. 1986 Jun 21;160(12):429-34.
          4. Cox SC, Walker DM. Oral submucous fibrosis. A review. Aust Dent J. 1996 Oct;41(5):294-9.   5.
          5. Aziz SR. Oral submucous fibrosis: an unusual disease. J N J Dent Assoc. 1997 Spring;68(2):17-9.  6.
          6. Paissat DK. Oral submucous fibrosis. Int J Oral Surg. 1981 Oct;10(5):307-12.
7. Paul RR, Mukherjee A, Dutta PK, Banerjee S, Pal M, Chatterjee J, Chaudhuri K, Mukkerjee K. A novel wavelet neural network based pathological stage detection technique for an oral precancerous condition.  J Clin Pathol. 2005 Sep;58(9):932-8.
8. Pindborg JJ. Lesions of the oral mucosa to be considered premalignant and their epidemiology. In: Mackenzie IC, Dabelstein E, Squire CA, eds.: Oral premalignancy. Iowa City, IA: University of Iowa Press, 1980: 2-12.
9. Sinor PN, Gupta PC, Murti PR, Bhonsle RB, Daftary DK, Mehta FS, Pindborg JJ. A case-control study of oral submucous fibrosis with special reference to the etiologic role of areca nut.  J Oral Pathol Med. 1990 Feb;19(2):94-8
10. Pindborg JJ. Oral submucous fibrosis: a review. Ann Acad Med Singapore. 1989 Sep;18(5):603-7.
           11. Khanna JN, Andrade NN. Oral submucous fibrosis: a new concept in surgical management. Report of 100 cases. Int J Oral Maxillofac Surg. 1995 Dec;24(6):433
12. Rao Venkat & Raju P.N: A preliminary report of the treatment of submucous fibrosis of oral cavity with cortisone. Ind. Jour. Otolarng. 1954. 59-330.  
13. Kakar PK, Puri RK, Venkatachalam VP. Oral submucous fibrosis--treatment with hyalase. J Laryngol Otol. 1985 Jan;99(1):57-9.
14. Pruitt BA Jr, Levine NS. Characteristics and uses of biologic dressings and skin substitutes. Arch Surg. 1984 Mar;119(3):312-22
15. Kavarana NM, Bhathena HM. Surgery for severe trismus in submucous fibrosis. Br J Plast Surg. 1987 Jul;40(4):407-9.
16. Pindborg JJ, Bhonsle RB, Murti PR, Gupta PC, Daftary DK, Mehta FS. Incidence and early forms of oral submucous fibrosis. Oral Surg Oral Med Oral Pathol. 1980 Jul;50(1):40-4.  
17. Lee JT, Cheng LF, Chen PR, Wang CH, Hsu H, Chien SH, Wei FC. Bipaddled radial forearm flap for the reconstruction of bilateral buccal defects in oral submucous fibrosis. Int J Oral Maxillofac Surg. 2007 Jul;36(7):615-9. Epub 2007 May 11.  
18. Gupta RL, et al: Role of collagen sheet cover in burns—A clinical study. Indian J Surg.  40:646, 1978
19. Gupta RL, et al: Fate of collagen sheet for artificial created wounds. Indian J Surg.   40:641, 1978
20. AAlexander JM, Rabinowitz JL. Microfibrillar collagen (Avitene) as a hemostatic agent in experimental oral wounds. J Oral Surg. 1978 Mar;36(3):202-5
21. GGüngörmüş M, Kaya O. Evaluation of the effect of heterologous type I collagen on healing of bone defects.
J Oral Maxillofac Surg. 2002 May;60(5):541



Nasopharyngeal rhabdomyosarcoma

Abstract
Nasopharyngeal Rhabdomyosarcoma, a common soft tissue sarcoma in children, is a rare identity, so far only four–cases have been reported as per the review of literature. Ours is the fifth case presented exclusively in the nasopharnx, which is, hereby reported. The prognosis of this clinical entity is always gloomy and the modality of the treatment is always surgery, chemotherapy and radiotherapy.

Case report
A 5 year old boy was referred to ENT department of K.E.M. hospital with the complaints of nasal obstruction, noisy breathing and mouth breathing for over four months. There was no history of bleeding from the nose or any other significant ENT problems. Clinical examination revealed both nostrils clear with no evidence of any nasal mass occluding the nasal passage. Airways were blocked. Posterior rhinoscopy revealed huge polypoidal and grape like mass arising from the nasopharynx and hanging in the oropharynx pushing the soft palate downward and forward. Palatal movements were normal and there was no neurological involvement. Rest of the ENT examination was insignificant. Rest of the ENT examination was insignificant. Routine hematological tests and urine exam was normal. X–ray nasopharynx and compromising the nasal airway. Provisional diagnosis of nasopharyngeal malignancy was made. CT scan could not be done because of poor economical status of the patient. He was advised surgery and was subjected to excision under G. A. with routine tonsillectomy position. With Boyle–Davis gag, mouth was opened and with adenoid curette the whole mass was curetted. Bleeding was not alarming and controlled with post nasal packing. Postoperative recovery was uneventful. The mass sent for histopathology revealed rhabdomyosarcoma. Patient was advised post–op radiation and chemotherapy. Post operative recovery was uneventful and his symptoms of mouth breathing noisy breathing and nasal obstruction was cleared.

Dissussion
Rhabdomyosarcoma is a highly malignant tumor of childhood arising from the rhabdomyosarcoma of mesenchymal connective tissue. It frequently involves the head and neck region as orbit, nasopharynx, cheek, external ear, maxilla, tonsillar area and soft palate.

The presentation in the nasopharynx is very rare. Only four cases so far have been reported in the literature. Ours is another rare case which exclusively presented in the nasopharynx.

The first mention of this pathological entity was made by Weber (1855), Mason and Soule (1956). The histological classification was made by Wills R. A. (1967). Histologically, the tumor is characterized by round or spindle cells with distinct granular vacuolated cyto plasm.

The Rhabdomyosarcomas can be divided into four subtypes
  • Embryonal and embroyonal botryoid.
  • Alveolar.
  • Pleomorphic.
  • Mixed.
The embryonal is most frequently encountered rhabdomyosarcoma of childhood (80%). The disease usually runs a rapid course unless diagnosed and treated promptly. Clinically, it may be mistaken as antrochoanal polyp or angiofibroma or hypertrophic adenoid. The tendency of lymph node metastasis is more in embryonal rhabdomyosarcoma. Hemotogeneous spread to lung and bones are also not uncommon. Prior and Stoner (1957), Vieta et al (1962). Mason and Soule (1963).

The prognosis of rhabdomyosarcoma is very gloomy with less than 10% long time survival. The treatment modality adopted is a multi modality therapy. This entails surgery, chemotherapy and radiotherapy.

References

  • Horn R. C. Jr. Enterline HT (1958). Rhabdomyosarcoma a clincopathological study and classification Cancer11, 181.
  • Masson J. K. and Soule EH (1965). Embryonal rhabdomyosar coma of Head and Neck. American Journal of Surgery 110,585.
  • Stout A. D. (1946). Rhabdomyosarcomas of the skeletal muscles. Annals of Surgery 123,447.
  • Vieta L. R. Guraieb SR and Obregon MA (1962) Archives of Otolaryngology 75,248.
  • Wilis R. A. (1967) Pathology of Tumors 4th edition. Editorial Butterworth, London.


Management of Myasthenia Gravis in ENT Practice

Dr.K.K.Desarda. Leena Parulekar .Hemant Todmal

Abstract:
Myasthenia gravis is a neuromuscular disorder characterized by variable weakness of voluntary muscles, which often improves with rest and worsens with activity. The condition is caused by an abnormal immune response.
In myasthenia gravis, weakness occurs when the nerve impulse to initiate or sustain movement does not adequately reach the muscle cells. This is caused when immune cells target and attack the body's own cells (an autoimmune response). This immune response produces antibodies that attach to affected areas, preventing muscle cells from receiving chemical messages (neurotransmitters) from the nerve cell.
The cause of autoimmune disorders such as myasthenia gravis is unknown. In some cases, myasthenia gravis may be associated with tumors of the thymus (an organ of the immune system). Patients with myasthenia gravis have a higher risk of having other autoimmune disorders like thyrotoxicosis, rheumatoid arthritis, and systemic lupus erythematosus.
Myasthenia gravis affects about 3 of every 10,000 people and can affect people at any age. It is most common in young women and older men.


Introduction

Myasthenia gravis is a neuro–muscular disorder characterized by marked weakness and fatigue of muscles affecting the motor apparatus at the Myoneural junction. The management of this disease in ENT practice has been outlined briefly below. This disorder involves all age groups and any muscles in the body, but the disease shows special affinity for muscles innervated by bulbar nuclei (face, lips, eyes, tongue and neck). It is because of this affection that patients of this disorder first go to see the Otolaryngologists.

The cause of the myasthenia gravis is not known, but it is said to be a metabolic disorder. Thymus gland abnormalities have been described in some patients. It has also been said that myasthenia gravis is an auto immune disease, since multiple auto antibodies have been found in the sera of patients of this disorder. The women are more often affected than men and the disease appears between 20 and 40 years of age.


Twenty cases of proved myasthenia gravis were being treated at KEM hospital in Pune since 1978. Out of this, three patients required thymectomy, two patients went into respiratory failure and had to be subjected for tracheotomy and were subsequently put on the respirator. They died ultimately. Fourteen cases were managed on medical therapy e.g. neostigmine, alternate day steroids and supportive therapy.



Three interesting cases which were presented to our department are given below. The patients were diagnosed for oropharyngeal complaints like dysphagia, dyspnoea, dysphonia, hoarseness, diplopia and ptosis, mimicking the presentation of bulbar palsy, cricopharyngeal and nasopharyngeal malignancies, with neurological involvements.

Case Reports:

Case 1
A 40 year old man complaining of difficulty in swallowing, heaviness of speech and inability to open his eye, was referred by his practitioner to ENT OPD as the symptoms were rapidly progressing. The detailed examination of his nose, pharynx, nasopharynx and larynx did not reveal any abnormality except weak movements of palate (soft), pharyngeal wall and vocal cords. There was no evidence of any malignancy either.

Routine blood, urine, X–ray chest, ECG and lipid profiles were within normal limits. The only positive findings were ptosis, diplopia and marked fatigue. The nasal regurgitation ,dysarthria and the patient had difficulty in swallowing even liquids. A provisional diagnosis of Myasthenia gravis was made and confirmed by a positive neostigmine test.

Case 2
A 40 year old female was admitted for difficulty in breathing, hoarseness of voice and difficulty in swallowing. A routine ENT examination was done. Her nose, nasopharynx and larynx looked normal except for weakness in palatal movements, pharyngeal musculature and sluggish mobility of the cords. She had ptosis though diplopia and fundi were normal. Routine blood, urine and serum electrolyte studies did not reveal any abnormality. An X–ray of the chest revealed huge shadows in the mediastinum suggestive of thymoma. Provisional diagnosis of myasthenia gravis was confirmed by positive neostigmine test.

Case 3
A 35 year old female was attending an ENT clinic for slurring of speech, nasal regurgitation and change of voice for six weeks. She would feel fine in the mornings, but as the day advanced, the symptoms would progress and at the end of the day, she would become dyspneic and dysphonic with marked fatigue in articulation.

A routine ENT examination did not reveal any marked abnormality except weakness in palatal movements, nasal twang, and weak movements of vocal cords. Even routine hemogram, urine and chest X–ray did not reveal any abnormality. A provisional diagnosis of myasthenia gravis was made and confirmed by a positive neostigmine test.


Clinical Presentation

Most of these patients come to an otolaryngologist for their nasal or pharyngeal symptoms.
Clinical presentation is as follows:
Occular Symptoms: Ptosis, diplopia or both.
Oropharyngeal, symptoms: Dysphagia, dysarthria, dyspnoea.
Weakness of muscles of mastication and facial muscles.
Nasal twang (nasal speech), nasal regurgitation and marked fatigue in articulation.
Pharyngeal musculature weakness may cause dysphagia for liquids and solids.
Laryngeal muscle weakness may cause dyspnea, dysphonia and hoarseness of voice and weak cough reflex.
Involvement of respiratory muscle may cause respiratory striders.
Diagnosis
It is based on a detailed history of the clinical course of the disease, clinical examination and laboratory investigation. The positive finding like ptosis, diplopia, facial weakness and progressive fatigue in chewing, swallowing and speaking. The diagnosis is confirmed by a positive neostigmine test.

The hysteria, disseminated sclerosis, bulbar lesions, and polymyositis do mimic the picture of myasthenia gravis and should be excluded by appropriate tests and laboratory investigations. It is also stressed that malignancy of oropharyngeal, nasopharyngeal spaces should be kept in mind in evaluating the diagnosis. The importance of positive neostigmine test is the main stay in the diagnosis of the myasthenia gravis.


Treatment
There is no known cure for myasthenia gravis. However, treatment may result in prolonged periods of remission.
Lifestyle adjustments may enable continuation of many activities. Activity should be planned to allow scheduled rest periods. An eye patch may be recommended if double vision is bothersome. Stress and excessive heat exposure should be avoided because they can worsen symptoms.
Some medications, such as neostigmine or pyridostigmine, improve the communication between the nerve and the muscle. Prednisone and other medications that suppress the immune response (such as azathioprine, cyclosporine, or mycophenolate mofetil) may be used if symptoms are severe and there is inadequate response to other medications.
Plasmapheresis, a technique in which blood plasma containing antibodies against the body is removed from the body and replaced with fluids (donated antibody-free plasma or other intravenous fluids), may reduce symptoms for up to 4 - 6 weeks and is often used to optimize conditions before surgery.
When other treatments do not improve systems, patients may receive intravenous immunoglobulin.
Surgical removal of the thymus (thymectomy) may result in permanent remission or less need for medicines.
Patients with eye problems may try lens prisms to improve vision. Surgery may also be performed on the eye muscles.
Several medications may make symptoms worse and should be avoided. Therefore, it is always important to check with your doctor about the safety of a medication before taking it.


Medical treatment
Most of these patients are well maintained on a regime of medical drugs.
Neostigmine bromide 15 mg orally four times/day and may be increased upto 180 mg/day.
Edrophonium chloride (Tensilon) 10 mg 1/v may relieve myasthenic weakness in 20 to 30 seconds. 25–30 mg  im daily gives relief for several hours.
Ephenrine sulphate: 12 mg/day. The side effects of these drugs like abdominal cramps, nausea, vomiting may be prevented by adding an atropine like drug.
Corticosteroids and corticotropine: Encouraging results have been reported. Taking alternative day steroids orally is the recommended therapy.

Surgical treatment



Myasthenia gravis with Thymus tumor can be subjected for Thymectomy in females under 40 years, where medical treatment has failed. Complete remission may occur in 1/3rd of patients. The surgical result in men are uncertain.




Deep X–ray therapy

Those patients whose medical treatment has failed and those who have refused surgery, may be subjected for deep X–ray therapy (3000 R) to thymus in 10 to 12 divided doses. Partial remission may occur in half the patients.

Emergency Treatment

The Myasthenic crisis is an emergency and should be attended to immediately. Sudden inability to breath or swallow may occur any time. These patients should carry two ampules of 0.5 mg. of Neostigmine methysulphate to be given immediately S/c or 1/m if severe symptoms develop.

Progressive and potentially dangerous respiratory stridor may take place and will require immediate emergency tracheostomy. Therefore both, a tracheostomy oxygen and suction unit should be kept near the patient. If these facilities are not available, then the patient should be transferred to an intensive care unit for management where respirator facilities will be available. During this emergency treatment body fluids and electrolytes should be maintained.

Prognosis:

As regards the prognosis of this condition, it is always guarded. The spontaneous remission occurs frequently but a relapse is the rule. Myasthenic crisis and sudden death may also occur. The most critical period following the onset is two years.There is no cure, but long-term remission is possible. There may be minimal restriction on activity in many cases. Patients that only have eye symptoms (ocular myasthenia gravis), may progress to have generalized myasthenia over time.
Pregnancy is possible for a woman with myasthenia gravis but should be closely supervised. The baby may be temporarily weak and require medications for a few weeks after birth but usually does not develop the disorder.
            Possible Complications
  Restrictions on lifestyle (possible)
  Side effects of medications (see the specific medication)
  Complications of surgery
  Myasthenic crisis (breathing difficulty), may be life threatening
.


           References
Brumner N. G. (1972). ‘Corticosteroids in Myasthenia Gravis’. Neurology 22, COC.
Cape C. A., Utterback R. A. (1973). ‘Maintenance of ARTH in Myasthenia Gravis’. New England Journal Med. 27,288.
Kreal et al. (1967). ‘Role of Thymectomy in the management of Myasthenia Gravis’. Ann Surgery 165,111.
Warmolts J. R., Engel W. K. (1972). ‘Benefit from alternate prednisone in Myasthenia Gravis’. New England J Med. 17,286.

           Contributed by

           Dr. K. K. Desarda
         Prof.& Head otolaryngology,
         KEM Hospital Pune.



             MALIGNANT FIBROUS HISTEOCYTOMA
.
                         DR.K.K.DESARDA.  DR.S. PUNTAMBEKAR  KEM HOSPITAL PUNE.




INTRODUCTION

Malignant fibrous histiocytoma (MFH) is a tumor derived from mesenquimal tissue composed by 5 cell types  The term MFH was suggested to describe a tumor histiocytic-like with fibrous tissue . Its origin is believed to be from totipotent cells for presenting 2 different cell types. In histological terms, MFH is divided into 5 subtypes.

MFH is usually malignant when occurs on profound soft tissue. Around 3% of such tumors occur in the area of head and neck, and it is rare in larynx. New immunohistochemical and by electronic microscopy techniques have increased accuracy of histopathological diagnosis.
We described a rare case of MFH in the larynx and discussed its diagnosis and treatment.


CASE REPORT

54-year-old-male patient, smoker for 30 years was  seen in ENT OPD at KEM Hospital, Pune. in 2005 with the complaints of change of voice,odynophagia, and Foreign body   sensations in the throat for 18 months duration. There was no signs of respiratory distress or he neither had symptoms of dyspnoea, dysphagia nor important medical medical problems. Videolaryngoscopy, revealed smooth polypoidal mass on the anterior two third of left vocal cord with no paresis . There was no palpable cervical lymphadenopathy. In June, 2005, a microlaryngoscopy with polyp excision was performed and the biopsy taken from the mass was subjected for histopathological study, which was reported as  malignant fibrous histcytoma.He was then advised for surgical excision.and frontolateral left  laryngectomy was performed. The post operative recovery was excellent.He was reviewed at monthly intervals for almost two yrs, and there was recurrence seen.
 


  Videolaryngoscopy showing polypoidal mass over the left vocal cord


             Light microscopy showing large proliferation of fibroblasts mixed in several histiocytic, multinucleated cells.



DISCUSSION

The term MFH was suggested by Kauffman and Stout in 1961 in order to describe a histiocytic-like tumor with predominance of fibroblasts

MFH on head and neck area is more common in men, in a proportion of 3:1 and it affects this area only in 3% of the cases  on the skin and soft tissues. Nose, paranasal sinus and mandible are the most common areas  with malignant feature when in profound areas  There are 30 cases reported  and most of them are subglotti , preferably in men on glottic area and in women in subglottic  Patient with glottic MFH are usually older and smoker, opposing from subglottic MFH.

It is histologically divided into 5 types: pelomorphic, myxoid, inflammatory, giant cells and angiomatous  It is derived from mesenquimal tissue composed by 5 cell types: fibroplast, histiocytic, indistinguished, giant multinucleated and xantomatous cells  Its origin is believed to be from totipotent cells for presenting two different cell types. Structural analysis through electronic microscopy, immunohistochemical techniques and culture of tissue help in order to differ this type of tumor . The immunohistochemical anti-vimentin V9 was positive in this case.

This tumor often presents only clinical symptoms, such as dyspnoea, dysphagia, hemoptysis, stridor and tiredness when achieves larger sizes . Diagnosis for it is usually established after the removal of the tissue mass.

Differential diagnosis should be performed with pleomorphic rhabdomyosarcoma, fibrosarcoma, spinocellular carcinoma, angiosarcoma, hemangiopericytoma, pleomorphic liposarcoma and lymphoma

Surgery is the therapy with block resection of tumor. The hemilaryngectomy technique was chosen, though tumor was limited to left vocal fold. Cervical emptying was not performed because physical exam. did not presente lymphonode involvement. Radiotherapy is reserved to high risky patients, recurrence of non-operated patients and cases of metastasis to distance . Some authors suggest chemotherapy for other types of sarcomas .  Adjuvant radiotherapy and chemotherapy with surgery requires more studying.

MFH of the larynx is a rare type of tumor, but a high recurrence, fast growth, unexpected clinical behaviour and it tends to local and distance metastasis, especially to brain and lungs There is a lack of prognosis when there is sign of metastasis and glottic MFH, what differs from subglottic MFH


CONCLUSION

MFH is a rare type of tumor and there are few reported cases in the literature,thus makes its prognosis and therapy  difficult. Surgical therapy is the one with ample resection of tumor. It has high recurrence rate and hence periodical review is mandatory.


BIBLIOGRAPHY

1. Barnes L, Kanbour A. Malignant fibrous histiocytoma of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:1149-56.

2. Bernaldez R, Nistal M, Kaiser C, Gavilán J. Malignant histiocytoma of the larynx. J Laryngol Otol 1991;105:130-3.

3. Blitzer A, Lawson W, Biller HF. Malignant fibrous histiocytoma of the head and neck. Laryngoscope 1977, 87:1479-99.

4. Canalis R, Green M, Donard H, Hirose F, Cooper S. Malignant fibrous xanthoma of the larynx. Arch Otolaryngol 1975, 101:135-7.

5. Enzinger FM, Weiss SW. Soft Tissue Tumours. St. Louis: C.V. Mosby Company; 1983. pp. 125-35, 170-98.

6. Ferlito A, Nicolai P, Recher G, Name S. Primary laryngeal malignant fibrous histiocytoma: review of the literature. Laryngoscope 1983;93:1351-8.

7. Godoy J, Jacobs JR, Crissman J. Malignant fibrous histiocytoma of the larynx. J Surg Oncol 1986;31:62-5.

8. Kauffman SL, Stout AP. Histiocytic tumors (fibromas, xanthoma and histiocytoma) in children. Cancer 1961;14:469-82.

9. Keenan J, Snyder G, Toomey J. Malignant fibrous histiocytoma of the larynx. Otolaryngol Head Neck Surg 1979;87:599-603



 INVERTED PAPILLOMA OF NOSE &     PARANASAL   SINUSES:

                                   


 Dr.K.K.Desarda.  Prof. & Head otolaryngology KEM Hospital Pune.

                                                           
Abstract:



 Inverted papilloma (Schneiderian papilloma) is a primarily benign lesion that occurs in the nasal cavity and paranasal sinuses. Clinical problems include a tendency towards local destruction,recurrence and malignant transformation into squamous cell carcinoma. Hence, complete surgical removal is the therapy of choice and a meticulous follow-up is mandatory. The different histological types of nasal papilloma, their pathogenesis and the clinical and histopathological diagnosis., staging systems, therapeutic approaches, and surgical concepts are discussed.

The medical management is limited. Historically, radiotherapy was used in the management of inverted papillomas. Inverted papillomas have not shown to be radiosensitive. With radiotherapy in recent times, it has been used in patients with synchronous squamous cell carcinoma. Currently, medical management is used as an adjunct to specific complications, such as sinusitis. Surgical management is the mainstay of treatment of inverted papilloma. Selection of the surgical procedure is based on its extent, location, and the presence of concurrent malignancy.



Introduction:


Inverted Papilloma is a benign neoplasm originating from the Schneiderian membrane of the nose and paranasal sinus cavities. It has varied clinical and histological features involving the lateral nasal wall, septum, ethmoids, maxillary and sphenoid sinuses and at times involves the skull base. Three  cses interesting cases are reported below because of varied presentation of inverted papilloma.
Since Inverted Papilloma is associated with chronic sinusitis, patients always had nasal and sinus surgery. Most authorities will consider this a true neoplasm because of its transformation into carcinoma as transitional cell, papilloma or squamous papillary epithelioma. Early clinical diagnosis and thorough evaluation by biochemical tests, high resolution coronal CT scanning MRI studies and radical surgery is advocated. For its recurrence and malignant transformation few authorities advocate deep X–ray therapy following radical surgery. Fifty six  cases of proved inverted papilloma were treated  by different surgical procedures during 1980 to 2000 at KEM Hospital  Pune .
The advantages and disadvantages of various techniques  are discussed. No single technique gives better result so combinations were tried for better results.
The etiology of inverted papilloma is still unknown, but a number of nonspecific causes, as seen in the slide to the right, have been implicated. It is most often considered a true epithelial neoplasm as its intense proliferation of epithelium is its dominant histologic feature. Finally, a viral etiology has also been suggested. The support for viral etiology includes its multifocal origin and its high rate of recurrence, as well as the fact that it has been found to be responsible for other papillomas in other areas of the body. Human papilloma virus is most often associated with inverted papilloma. HPV DNA has been identified in 32 percent of inverted papillomas by in situ heparinization and PCR. Among inverted papillomas associated with carcinomas, HPV is present in 58 percent. Type 16 is the most prevalent in inverted papillomas,
 Because of its varied presentation inverting papillomas can be difficult to distinguish from other nasal tumors and they tend to recur after limited operation and also tend to transform into carcinoma. Hence, it is impossible to predict which inverting papillomas will become malignant.

.Sinonasal papillomas are characterized by being in general unilateral, although bilateral papillomas occur infrequently. Second, they have a destructive capacity with an ability to extend on into adjacent areas by spreading along a mucosa. Third, they have a tendency to recur, and they will recur even if completely excised. Finally, they have the potential for malignant degeneration.The clinical appearance of the nasal mass resembles an allergic polyp looking like a gray and red nasal mass.

The incidence of associated malignancy has been estimated to be approximately 10 % to 15%  Lawson and Allen in 2003 reported that 7 percent of patients have associated malignancy with synchronous carcinoma and 4 with metachronous carcinoma. These develop three to eight years after initial diagnosis. In their literature review of 26 series published between 1970 and 2001, consisting of over 1400 patients, 8.9% were found to have associated malignancy, 67% with synchronous, and 32% with metachronous











Grading of Inverted Papilloma



Grade I  Lesions involving nasal cavity only.
Grade II  Lesions involving nasal cavity + Paranasal sinuses.
Grade III
 Lesions involving  nasal cavity + Paranasal sinuses +Intracranial  extension
.




Management:

 Key to diagnosis is a detailed history, of course. Patients may have a history of unilateral symptoms or history of multiple surgical procedures for nasal polyps. Second, a thorough physical exam where a unilateral mass may be seen or endoscopic exam may reveal multiple polypoid masses with multiple digitations located laterally to the middle turbinate. Three, biopsy is key. Given the similar appearance and possible presence of concurrent polyps, histologic examination is critical. Multiple biopsies may be necessary, as seen in our case, due to inadequate sampling or sampling of concurrent polyp or inflammatory tissue or error in diagnosis.
Finally, radiographic evaluation is critical. CT scan is considered to be the study of choice. The most common CT profile is a unilateral mass with a lobulant surface occupying the middle meatus and extending into one or more of the adjacent sinuses. Opacification, mucosal thickening, of the paranasal sinuses may be seen, as well as bony thinning remodeling or erosion caused by inverted papilloma growth. Disadvantages of the CT scan are primarily due to its difficulty to differentiate inspissated mucus, polyps, or mucoperiostial thickening from inverted papilloma. MRI may also be used in the diagnosis of inverted papilloma. It is superior to CT scan for distinguishing papillomas from underlying inflammation and provides better delineation of lesions in contrast to surrounding soft tissue.
The medical manageent is limited. Historically, radiotherapy was used in the management of inverted papillomas. Inverted papillomas have not shown to be radiosensitive. With radiotherapy in recent times, it has been used in patients with synchronous squamous cell carcinoma. Currently, medical management is used as an adjunct to specific complications, such as sinusitis. Surgical management is the mainstay of treatment of inverted papilloma. Selection of the surgical procedure is based on its extent, location, and the presence of concurrent malignancy.
Three procedures that have been used to treat inverted papillomas are lateral rhinotomy and medial maxillectomy. Currently, the gold standard for the treatment of inverted papilloma is midfacial degloving and endoscopic sinus surgery. Lateral rhinotomy medial maxillectomy is particularly useful for inverted papillomas that are perilacrimal, nasofrontal, supraorbital, ethmoidal, or in the orbit. A curvilinear incision beginning just below the medial aspect of the eyebrow is made inferiorly half-way between the medial canthus and the nasion. The incision is extended inferiorly along the lateral aspect of the nose around the ala. The incision includes a full thickness skin down to periosteum. The periosteum is then elevated as far lateral as the lateral aspect of the maxillary antrum, as far superior as the orbital rim exposing and preserving the infraorbital nerve, and along the nasal bone in the ascending process of the maxilla. The periorbit is then undermined off the lamina papyracea, dislocating the lacrimal sac out of the lacrimal fossa and transacting the lacrimal duct as far distal as possible. The periorbit is then further undermined off the medial floor of the orbit. The anterior and posterior ethmoid artery is identified. These are the most constant landmarks for the frontoethmoid suture line. Staying below the suture line is critical to avoid entrance into the anterior cranial fossa. An opening is made through the anterior wall of the maxillary antrum, and the entire front wall of the maxillary antrum is removed up to the orbital rim. Then osteotomies are made, first along the floor of the nose through the bone between the antrum and the nasal cavity; second, through the frontoethmoid suture line below the level of the anterior ethmoid artery; and finally, along the medial floor of the orbit to the posterior wall of the antrum. The lateral nasal wall is then removed by cutting through the middle inferior turbinate attachments and then all mucosa is removed from the maxillary antrum. Then the sphenoid sinus is opened and its mucosa also removed. Dacryocystorhinostomy is then performed to avoid epiphora, a common postoperative complication of this procedure. It can be accomplished in two ways—either by catheterization of the lacrimal duct using an indwelling silicone or incising the lacrimal sac along it long axis and then suturing the edges in place to adjacent tissues. The main advantages of this approach include a radical excision, access to all lateral sinuses, the skull base, nasopharynx, and orbit. Disadvantages include, obviously, a surgical scar, possible CSF leak, epiphora, injury to orbit, and mucocele formation.
The contraindications to a purely endoscopic resection of inverted papillomas include the concomitant presence of squamous cell carcinoma, massive skull base erosion, intradural or intraorbital extension, and extensive involvement of the frontal sinus.
Midfacial degloving is particularly useful for inverted papillomas that are bilateral nasal in origin. Four types of incisions are required in this approach. The first is bilateral intercartilaginous incisions, bilateral septocolumellar incisions, a complete transfixion incision, and then a gingivobuccal incision. This is made from one maxillary tubercule to another. The soft tissue is then incised around the piriform aperture and nasal floor is undermined as high as the orbital rims. These incisions facilitate the exposure of a piriform aperture in the lateral nasal wall. A medial maxillectomy is then performed . and this can be combined with the frontal sinus osteoplastic flap for access to the frontal sinus. Its main advantages are that there is no external scar, invisibility, and bilateral access. Its disadvantages include insufficient access to more distant areas, such as vestibular, orbital, ethmoid cells; and complications include vestibular stenosis, oral antral fistula, epistaxis, and nasal congestion.
Endoscopic sinus surgery is particularly useful for inverted papillomas in the lateral nasal and nasal cavity, middle meatus, maxillary sinus, and the anterior and posterior ethmoid cells. The procedure largely consists of tumor debulking through a microdebridder until the origin is identified, and then lesions are usually excised en bloc from the identified tumor attachment sites with a wide cup of normal mucosa. Frozen sections are obtained, negative margins confirmed, and bone may be removed from underlying sites of attachment Although traditionally endoscopic surgery is used more for small lesions, bulky lesions have made endoscopic surgery difficult.  However, a new technique has been described for treatment of massive tumors with attachments within the maxillary sinus. It is called SSES (Sequential Segmental Endoscopic sinus Surgery). Basically, this involves sequential excision of larger tumors into massive segments, four segments usually. First, the nasal cavity; second, the middle meatus, including portions of the ostiomeatal complex; third, the maxillary sinus ostium and antrum along with the maxillary sinus medial wall if an endoscopic medial maxillectomy is performed; and fourth the frontal or sphenoid sinus .Advancements in diagnosis and treatment of sinus disease have led to successful use of endoscopic techniques in the treatment of inverted papilloma .

Case presentations:

Case No. 1
Mr. D. K. aged 68 had complaints of left nasal obstruction, headache, epiphora and bleeding from nostrils (epistaxis). He had nasal polypectomy and intranasal antrostomy done seven years ago. An ENT examination revealed a large greyish mass occupying the left nasal cavity pushing the septum to right side. A probe could not be passed around the nasal mass since it was coming from the lateral nasal wall. The nasal vault was tender and there was a evidence of nasolacrimal duct obstruction. Left maxilla was tender and mass did not bleed on touch. A provisional diagnosis of recurrence of nasal polyposis was made with the view of malignancy in mind.

The routine bio–chemical tests were within normal limits, X–ray paranasal sinuses revealed opacity and left maxillary sinuses with medial wall destruction. A CT scan also revealed a lesion of maxillary sinus with erosion of medial wall and a soft tissue mass in the left nasal cavity extending to the nasopharynx.
In view of the recurrence and erosion of the medial wall, the patient was subjected to lateral rhinotomy with medial maxillectomy procedure. The mass was removed with maxillary clearance. Histopathology confirmed the diagnosis of inverted papilloma with no evidence of malignancy. Post operative recovery was uneventful. The patient was reviewed in a follow up clinic and had no recurrence for the last one and a half year.

Case No. 2
Mrs. M. L. aged 48 had a bilateral nasal obstruction, mouth breathing and headache. She was non–diabetic, non–hypertensive and had nasal polypectomy two years ago. A routine clinical examination revealed bilateral polypoidal masses occupying both the nostrils. The color of the mass was grayish and firm in consistency and did not bleed on touch. Both maxillary sinuses were tender. Postnasal space did not show any soft tissue mass..
Clinical diagnosis of bilateral nasal polyposis was made (Recurrence) with associated maxillary sinusitis and no extension to nasopharynx. CT PNS revealed extensive opacity of maxillary and ,ethmoid sinuses without any erosion of orbital cavity. Endoscopic  sinus surgery  was planned under GA .  Post operative recovery was uneventful. Histopathology of the biopsy revealed inverted papilloma. A review after 18 months was satisfactory without recurrence.
Case No. 3
Mr. A. K. aged 28 came to the ENT Clinic with the chief complaints of nasal obstruction, bleeding from left nostril, headache, and proptosis of left eye for over six months. A clinical diagnosis of the left nasal polyposis was made after a thorough clinical examination. A routine hemogram was performed and this including biochemical tests were within normal limits. A CT scan revealed a lesion involving the entire left ethmoid, nasal cavity, maxillary sinus, nasopharynx and anterior wall of sphenoid sinus.A planned endoscopic ethmoidectomy and transantral maxillary clearance was done. The post operative period was uneventful. and  the patient’s left proptosis decreased . Histopathology report revealed inverted papilloma. Post–operative review after six months was quite satisfactory and without recurrence.
Discussion:
The search for an ideal surgical approach to removing inverted papillomas has been fairly controversial, with proponents of radical surgery vying with those who support endoscopic endonasal procedures. The external approaches include medial maxillectomy, frontoethmoidectomy, mid facial degloving and Caldwell-Luc surgeries. Recurrence rates following these procedures have been reported to range from 4 to 35%.  The short-term complications of these approaches include epiphora, dacryocystitis, diplopia, transient blepharitis, lid edema, and cerebrospinal fluid leak. Late complications include persistent crusting, pain, nasocutaneous fistula, frontal sinus mucocele, vestibular stenosis, unacceptable scarring, and nasal collapse

Inverted papillomas are relatively uncommon tumors of the nasal cavity comprising approximately 0.5% to 4% of all primary nasal tumors. Its incidence ranges from 0.75 to 1.5 cases per 100,000 per year. They occur approximately 15 th as often as inflammatory polyps. Age ranges widely from 6 to 89 years, and most are usually diagnosed in the 5 th to 7 th decade. Average age of diagnosis is 53 years. There is a male predominance 3:1, and it affects primarily Caucasians.
Sinonasal papillomas have been categorized in to three distinct subtypes based on histologic appearance. Inverted papillomas (70%),cylindrical cell papilloma (19%), and fungiform  papillomas  (11%),although all these are histologically benign in nature but inverted and cylindrical  papillomas  may be associated with malignancy which ranges from 4% to 17 % for inverted papillomas and 9% to 13% for cylindrical papillomas..
Symptoms are nonspecific often mimicking sinusitis. The most common is unilateral nasal obstruction seen in over 60% of patients. Nasal discharge, headache, facial pressure and pain, epistaxis, and anosmia may also be seen. Signs may include a polypoidal mass filling the nasal cavity extending from the vestibule to the nasopharynx. The nasal septum is often bowed to the contralateral side due to slow expansile growth. Proptosis and facial swelling  is also seen in these patient  at late stages.
Sinonasal papillomas are characterized by being in general unilateral, although bilateral papillomas occur infrequently. Second, they have a destructive capacity with an ability to extend on into adjacent areas by spreading along a mucosa. Third, they have a tendency to recur, and they will recur even if completely excised. Finally, they have the potential for malignant degeneration Although most of the lesions arise from the lateral wall, middle meatus and ethmoid complex, they may sometimes arise from the septum and underlying perichondrium, the cartilage, lateral nasopharyngeal wall, maxillary sinus, sphenoid sinus and may involve the base skull. The distribution is: lateral wall 68%, ethmoid complex 57%, septum 28% intracranium 4%. The highest recurrence rate  is 70%.
 Advantages and Disadvantages of endoscopic surgery.
It is a less invasive procedure and you have a multiangle visualization and absence of facial scarring. In terms of disadvantages - orbit injury, CSF leak, and periorbital ecchymosis. It is especially difficult for the larger tumors that fill the entire nasal cavity. This is increasingly circumvented by the ability to first debulk the tumor and then perform excision in segments. The main controversy in the treatment of inverted papilloma lies in the final advantage and disadvantage: seemingly contradictions, comparable versus greater recurrence rate. This inherent contradiction is reflected in the debate that still exists regarding whether less extensive procedures result in incomplete excision of the inverted papilloma and, thus, more recurrence



Acknowledgement

I am thankful to  Dr. B.J. Coyaji Chief Medical officer,KEM Hospital Pune for permitting me to publish this paper.I extend sincere thanks to  Nursing staff and staff ENT Dept for their kind co-operation in  the preparation of this manuscript.


Bibillography.
Frank, C. A., Oliver D. J. and Jack, G (1985): Usual anatomic presentation of inverted papilloma – Head and neck Surgery, 243:45.
Feinmessar, R., Goy. I., Weessel, J. M. and Ben–Bessat H. (1985): Malignant Transformation of inverted Papilloma. Ann Otol 94:39–43.
William, M. Menden, H., Rodney, R., Million, N. J. and Cassissi Kenal, P. K. (1985): Biological aggressive Papilloma of Nasal Cavity – Role of radiation therapy. Laryngoscope, 95:344–347.
Thomas, C. and Calceterra, W. T. (1980): Varied presentation of inverted Papilloma, 90:53–60.

 Alba JR, Diaz MAA, Perez A, Rausell N, Basterra J. Inverted papilloma of the sphenoid sinus. Acta Oto-Rhino-Laryngologic Belg 2002:56:399-402.
Baruah P, Deka RC. Endoscopic management of inverted papillomas of the nose and paranasal sinuses. ENT-Ear Nose Throat J 2003;82:317-320.
Benninger MS, Roberts JK, Sebek BA, Levine HL, Tucker HM, Lavertu P. Inverted papillomas and associated squamous cell carcinomas. Otolaryngol Head Neck Surg 1990;103:457-461.
Buchwald C, Lindeberg H, Pedersen BL, Franzmann M-B. Human papilloma virus and p53 expression in carcinomas associated with sinonasal papillomas: A Dutch epidemiological study 1980-1998. Laryngoscope 2001;111:1104-1110.
Bull TR. Color Atlas of ENT Diagnosis. New York: Thieme.
Calcaterra TC, Thompson JW, Paglia DE. Inverting papillomas of the nose and paranasal sinuses. Laryngoscope 1980;90:53-60
Cummings BW, Goodman ML. Inverted papillomas of the nose and paranasal sinus. Arch Otolarygol 1970;92:445-449.
Eisen MD, Buchmann L, Litman RS, Kennedy DW. Inverted papilloma of the sphenoid sinus presenting with auditory symptoms: A report of two cases. Laryngoscope 2002;112:1197-1200.
Gulya AJ, Wilson WR. An Atlas of Ear, Nose and Throat Disorders. New York: Parthenon Publishing Company.

Contributed by Dr. K. K. Desarda,





Cryosurgery Treatment for Hemangioma of Tongue:
A Case Report

Dr.K.K. Desarda .Dr. Nilima,Dr.Sheetal.


ABSTRACT

Hemangiomas are frequently seen benign tumors which based on vascular tissues. These lesions are mainly identified in to two groups which are namedas capillary and cavernous hemangiomas due to vascularization of the lesions.Capillary hemangiomas consist of small capillary vessels which show lobulesformation. Cavernous hemangiomas consist of large dilated vessels and theycan reach to large sizes. Several treatment modalities including sclerotherapy,embolisation, laser surgery and cryosurgery have been described forhemangiomas. In this significant case, the cryosurgery treatment of a 32 yearsold female patient who was suffering from the huge hemangioma in the rightside of her tongue is presented with complete regression of haemangioma without any tongue deformity or atrophy.or post op morbidity.


KEYWORDS


Hemangioma, Cryosurgery

 Contact Author

Dr. K.K.Desarda-Prof.Emeritus-& Head ORL KEM Hospital, Pune.

E-mail : kdesarda@gmail.com


INTRODUCTION


The author using cryoapplicator of his own modification presents cryosurgery as a successful surgical technique in cases of haemangiomas involving , tongue and oral cavity. From 1980 to 2012 in the ENT-Clinic ,KEM Hospital,Pune. Over 60 patients were operated upon using this method. Most of the cases were treated under local anaesthesia. The results were very encouraging. Total regression of haemangiomas and regeneration of normal mucosa with no noticeable scar resulted. A method of cryosurgery especially suitable for very young patients or for those who are poor risks because of associated disease and advanced age is suggested.

Hemangiomas are benign tumors ofvascular tissue which are most likely to be seen at the head and neck region. They are the most common tumors of the childhood. They
show higher prevalence in women. Most of these lesions are described hamartomas instead of tumors. Vast majority of hemangiomas are known to be regressive(1).Hemangiomas are classified as capillary and cavernouson the basis of the vascularization system. (1). Capillary hemangiomas consist of small capillary bodies that organize lobularly. Cavernous hemangiomas consis of wide and dilated vessels and can reach to large size..Hemangiomas can be encountered intraorally; on,tongue, anterior gingival and buccal mucosa. Especially the lesions on the cheeks and tongue can be traumatized by chewing and bleeding can occur.  (1-4).It is known that deep hemangiomas could be seen as blue-purple lesions during intraoral examination. For the diagnosis of cavernous hemangiomas, bidigital palpation of the region and detection of disappearance of the blood due to finger pressure and after removal of finger pressure observation of revascularization are important signs. Furthermore, if the lesion has an arterial origin, pulse can be obtained by finger pressure(1,2).

Although most hemangiomas of the tongue are asymptomatic, they could sometimes cause significant bleeding, pain or difficulty in chewing, speaking, and  even swallowing, if they are large enough. Small lesions can be excised with impunity.  Large lesions, if excised by surgery could result in significant functional  disability.  This is why several modalities of less invasive treatment have recently been advocated (Argon laser, Nd:YAG laser,radiofrequency,scelotherapy, cryosurgery and superselective embolization to avoid functional disability caused by tissue loss).

Age and general condition of the patient has great importance as well as the size and characteristics ofthe lesion in the treatment of hemangiomas. Small lesions can be surgically excised while larger ones require specific surgical interventions. These treatment methods are sclerotherapy, embolisation, laser surgery, radiofrequency and cryosurgery (1-5)
.
Amongest all modalities Cryosurgery is a very effective method for thetreatment of intraoral cavernous hemangiomas. It can be applied under basic local anesthesia. Freezing should be repeated twice for each area for three to five minutes to ensure adequate effect. The ice ball should extend a little beyond the limits of the lesion so that about three-quarter of the diameter of the observed circumference of the frozen tissue will be at a cell lethal temperature of – 15° C of normal tissue. Sloughing of some of the central tissue usually takes places at about one week post operatively and healing may be expected with 4-6 weeks.


CASE REPORT


A 32  year old female patient presented to ENT dept KEM Hospital Pune who was suffering from progressive huge swelling  on her right lateral border of tongue,pain while swallowing,distorted speech and at time occasional bleeding for over six months.She was treated conservatively by the medical practioner,She was also seen by general surgeon who advised surgery (hemiglossectomy) which patient refused.



Clinical examination :

Revealed wide, bluish purple lesion was detected on the right lateral side of the patients tongue (Figure 1). The signs of revascularization after finger pressure was determined and no pulsations were obtained from the lesion and the lesion was diagnosed with hemangioma. Rest of the ENT examination was WNL
.A clinical diagnosis of tongue haemangioma was confirmed  and patient advised for crysurgery treatment,which she has accepted since it was less invasive procedure.Routine lab study and general fitness was taken and patient was posted for cryosurgical intervention .
...
Under local anaesthesia 10cc (xylocaine + Adr.1:200000) infiltration all around the haemangiomatous mass was done with 3mm normal tongue margin. Liquid nitrogen via large contact cryo tip was applied to the entire lesion for 3 to 5 minutes .including 3mm peripheral normal tissue margin (Fig 1 & 2) Following this process, the area was washed with NaCl 0.9% and the patient was prescribed with antibiotics, analgesic, anti-inflammatory agents and tantum mouth wash.. Necrotic and sloughing area were observed in the postoperative first week (Figure 3 & 4 )and within four to six weeks  the lesion was successfully  and completely healed (Figure 5 & 6)



Pre operative & post operative results of cryosurgery shown in these pictures.







 
Fig.1.Pre op Tongue Haemangioma     Fig.2. cryo-freezing.of Haemangioma.
                                                           
                                                                   




 

 Fig.3.   Intra op cryosurgery lesion           Fig.4.    Intra op cryosurgery lesion
 
Fig.5   Post cryosurgery result 4 wks     Fig.   Post cryosurgery result 6 wks


.
DISCUSSION

The diagnosis and the classification of the vascularmalformations have a great importance on the treatment plan of the lesions. Mulliken and Glowackiproposed a terminology for classifying these lesionsthat is based on clinical and microscopic features(6) .This system broadly classifies vascular lesions into hemangiomas and vascular malformations. The hemangioma is the true vascular tumor that results from a neoplastic overgrowth of normal vascular tissue.The hemangioma grows by endothelial proliferation.In distinction to hemangiomas, vascular malformation results from abnormal vascular or lymphatic vesselmorphogenesis, not as the result of abnormal endothelial growth. Hemangiomas are usually present at birth and can be diagnosed by 1 year, where asvascular malformations are present at birth but often not diagnosed until second decade of life.Hemangiomas show rapid growth until 6-8 monthsand involute by 5-9 year
 Vascular malformations show slow growth throughout life with increase in response to infection, trauma, or hormonal fluctuation and they do not involute. Osseous involvement of the hemangiomas is rare but 35% of the vascular malformations show osseous involvement .A wide variety of methods are utilized for the treatmentof intraoral hemangiomas.
 Embolisation technique which is one of them has been utilized since early nineties. However, embolisation technique has two major disadvantages. One of these disadvantages is the risk of embolisation material reaching cranial cavity via external and internal carotid arteries and the need for an experienced radiologist in order to perform this procedure. The other disadvantage is the temporary blockage of flow . It would be proper to utilize this method prior to the surgical excision of large hemangiomas in order to reduce to risk of bleeding

Another method for the treatment of hemangiomas is sclerotherapy. In this method, a sclerotic agent is injected into or peripheral to the vein that the hemangioma originates from. This method is successfully utilized in the treatment of extra oral lesions. However, pressured bandage can not be applied to the region after the injection of sclerotic agent inintraoral lesions. Thus, sclerotherapy is recommended to be applied together with other treatment methods Subzero temperatures can cause so-called ‘frosbite’whilst temperatures above the coagulation point of proteins results in ‘burns’. Thermal surgery employs these effects in carefully controlled manner: the use of temperatures below freezing point constituting cryosurgery while heating effects are obtained by theuse of lasers normally functioning in the infrared range of wavelengths.
The results of the cryosurgery can be explained as acold-induced coagulative necrosis.

 Cryodestruction of a normal or benign neoplastic tissue normally requiresthe attainment of a temperature of at least -15°C (thetemperature at which intracellular ice forms) while total ablation of malignant tumor tissue calls for somedegree of overkill at level of -50°C .For the management of oral benign, premalignant and malignant lesions liquid nitrogen is used as a freezing agent and delivered by either probes or sprays  Inthis case, probes are preferred in order to have limited effect on the lesion.In cryosurgery tissue regeneration is remarkably betterthan the other surgical techniques. When the body spontaneously separates the coagulated slough there is a powerful stimulus to cellular division, hyperplasia and apparent hypertrophy; this may be associated with concomitant cytokine release .Laser surgery is another effective method used for the treatment of intraoral hemangiomas  But, it has several disadvantages when compared with cryosurgery. Laser surgery is a much more complex process compared with cryosurgery


Laser surgery is a much more complex process compared to cryosurgery and requires general anesthesia.(5) Nerve damage is less in cryosurgery and regeneration is quicker.Postoperative scar formation is less in cryosurgery.Laser surgery application can be hazardous around salivary gland ducts, which should be taken into consideration. Laser surgery costs much more expensive compared to cryosurgery. However in laser surgery, postoperative edema is less and the procedure does not require to be repeated. Additionally, laser surgery is a faster and more dramatic technique



CONCLUSION

A variety of methods of treatment are thus available for intraoral hemangiomas. The majority of theselesions can be regarded as capillary-cavernous hemangiomas. In this case, cryosurgery was preferred for the treatment of the cavernous hemangioma since it has multiple advantages like being an easy, cheap,successful method which can be done under local anesthesia and can be repeated if required for residual lesion.


REFERENCES

1. Langdon JD, Patel MF: OperativeMaxillofacial Surgery, pp393-396. London
Chapman&Hall Medical, 1998.
2. Tal H. Cryosurgical treatment of hemangiomas of the lip. Oral Surg Oral
Med Oral Pathol 1992;73:650-54.
3. Hartmann PK, Verne D, Davis RG.Cryosurgical removal of a large oral
hemangioma. Oral Surg 1984;58:280-82.
4. Reischle S, Schuller-PetrovicS.Treatment of capillary hemangiomas of
early childhood with a new method ofDept. of Oral and Maxillofacial Surgery
Faculty of Dentistry, Ankara UniversityAnkara, Turkey.
5.Mulliken JB, Glowacki J. Hemangiomasand vascular malformations in infants andchildren: A classification based on
endothelial characteri





BERA STUDY IN 150 CHILDREN UNDER 5 YRS AGE

Dr. K. K. Desarda
Professor Emeritus & Head, Otolaryngology.KEM Hospital Pune.
&
Dr. A. N. Sangekar
Audiologist and Speech Pathologist,
KEM Hospital, Pune


BERA (BSER OR BAEP)

Abstract

OBJECTIVES: The brainstem evoked response audiometry (BERA) is an objective neurophysiological method for the evaluation of the hearing threshold and diagnosing retrocochlear lesions. The aim of the study was to investigate the hearing level in children with suspected hearing loss or pathological speech development. PATIENTS AND METHODS: The BERA diagnostic procedure was applied in 150 children ranging from 1 to 5years of age at KEM Hospital Pune.. RESULTS: We found profound hearing loss (deafness) in 15 children, severe hearing loss in 10 children, moderate hearing loss in 35 children, mild hearing loss in 30 children, and normal hearing level in 60 children. Out of the children suspected for hearing loss, 42% actually had some level (mild-moderate) of hearing loss. Out of the children with delayed speech, 63% had some level (mild-profound) of hearing loss which actually caused the delay in speech development; 37% had normal hearing, but inadequate verbal communication affected their language acquisition and speech development. CONCLUSIONS: These results illustrate the necessity to test children hearing even with the slightest suspicion by the parent or doctor of hearing loss.
INTRODUCTION
BERA has  proved to be a useful tool in diagnosing hearing impairments in children which could be conductive or sensorineural in nature. Thanks to early detection, rehabilitative procedures could be started early which will help speech and language development.

We have studied 150 cases below five years of age, and our observations are recorded with case history profiles like high risks, referral for adaptions, congenital malformations and delayed speech. We feel BERA is the only tool which can present an accurate picture of hearing sensitivity.

BERA (Brainstem evoked response audiometry), ABR (Auditory brain stem response), BAER (Brainstem auditory evoked response audiometry).

BERA is an electro-physiological test procedure which studies the electrical potential generated at the various levels of the auditory system starting from cochlea to cortex. This investigation was first described by Jewett and Williston in 1971.
Procedure: The stimulus either in the form of click or tone pips is transmitted to the ear via a transducer placed in the insert ear phone or head phone. The waves of impulses generated at the level of brain stem are recorded by the placement of electrodes over the scalp.

Electrode placement: Since the electrodes should be placed over the head, the hair must be oil free. The patient should be instructed to have shampoo bath before coming for investigation. The standard electrode configuration for BERA involves placing a non inverting electrode over the vertex of the head, and inverting electrodes placed over the ear lobe or mastoid prominence. One more earthing electrode is placed over the forehead. This earthing electrode is important for proper functioning of preamplifier.
BERA is resistant to the effects of sleep, sedation, sleep and anesthesia. Its threshold has been found to be within 10dB as elicited by conventional audiometry.

The waves detected in BERA tests
1. Auditory nerve
2. Cochlear nucleus
3. Superior olivary complex
4. Lateral lemniscus
5. Inferior colliculus
6 and 7. Medical geniculate body

.
Hearing problems are common among the children which could be conductive or sensorineural in nature. Early diagnosis of hearing impairment is important as the rehabilitative procedure can be started early which will help speech and language development.

Various audiological test procedures are used to assess the hearing sensitivity of children. Some of the common ones are: Behavioral Observation.
Free Field Audiometry.
Peep Show.
Pediatric Tester.
All the above techniques are useful in estimating hearing sensitivity but have their own limitations. The major difficulties involved in the use of these techniques are:
Co–operation of children.
Consistency of responses.
Subjectivity on the part of the tester.
Assessment of children with multiple handicaps.
BERA, (BSER or BAEP) has proved to be a useful objective tool in diagnosing hearing impairments in children. The use of Auditory Brainstem Response (ABR) (1) audiometry as an audiological tool focused on two principal areas:
The assessment of hearing sensitivity and slope of loss, (if any) in patients, who are unable or unwilling to participate in standard psychoacoustic test procedures.
To study the neurological integrity of acoustic nerve and brainstem pathway.
To achieve this objective, the paradigm employed is to collect appropriate ABR data for a case, analyze it and compare the data with relevant norms and draw inferences.

Material and Methods

In this study, an attempt is made to study the findings of BERA in children below the age of five years. These children reported to us or were referred to us for the following reasons:
Delay in speech and language development.
Inconsistent responses to sound or inability to respond to sound.
History of high risk factors – Deafness in the family, consanguineous marriage, difficult/obstructed labor, pre–term/premature labor, administration of antibiotic drugs during pregnancy, diseases contracted by the mother such as Rubella, Meningitis or Hyperbilirubinemia.
To rule out hearing impairment before adoption.
To rule out the extent of malformation anomalies especially in Atresia.

Test Procedure

All patients were administered the test procedures with prior appointment. An ENT check up was done to rule out the possibility of wax, ear infection, middle ear problems etc. The parents were instructed to wash the scalp of the child thoroughly as a requirement of the test. Prior to the test, each child was examined by the pediatrician and the dosage for sedation was prescribed. Drugs used for sedation were Trichloryl and Phenergan in combination.

Test was carried out in pre–cooled, quiet (not treated) room. The instrument used was Nicolet EP Four Compact which is a fully computerized machine with the facility of artifact rejection. The skin was cleaned with spirit and OMEN abrasive skin preparatory paste. The silver electrode were placed as follows: Cz–vertex, A–1 LF mastoid, A–2 values was not more than 1ohms. Electrode electrolyte gel was used and electrodes were fixed. Acoustically shielded THD 32 ear phones were placed on the ear and head bands were adjusted. The clicks of 11.4/sec duration were used as stimulus. The filter settings used were a 50Hz–300Hz. The polarity used was alternate and the analysis time was 10m/sec. About 4,000 responses were averaged. First, stimulus was given at 105 dBnHL level (i.e. maximum intensity level available). If peak V was detected at a particular level, intensity was increased by 5dB. The existence of peak V was considered as sound stimulus heard and perceived by the auditory mechanism. The threshold for each ear was confirmed. The guidelines used for the confirmation of peak V were as follows:
1.Peak V occurs around latency of 5.7 m/sec with S.D. of 0.25 (as per our norms).
     2.With decrease, an intensity level latency of peak V increases and its amplitude decreases.
     3.Reproduction of peak in re–run.
     4.Peculiar in shape.
     5.Use of a neutral run.
Since the measurement of hearing sensitivity in children under five years of age was the only aim of this study, the latency values and interpeak intervals even though measured, are not considered. Each child's hearing sensitivity was assessed, and they were sub–grouped in the following categories.
Normal hearing sensitivity. Hg. thresholds up to 25dB level and below.
Mild hearing impairment. Hg. thresholds between 30dB to 45dB.
Moderate hearing impairment Hg. thresholds between 50dB to 65dB.
Severe hearing impairment. Thresholds between 70dB to 85dB.
Profound hearing impairment. Thresholds above 90dB.

Observation
We have studies BERA findings of 150 children (below the age of five yrs) for this study.

Given below is the age–wise distribution of these children:

AGE No.
0–1 52
1–2 52
2–4 24
4–5 22

The case history profile of these cases is as follows:

High risk clinics. 82 cases
Referred for adoption. 31 cases
Patients with ear anamolies (congenital). 3 cases
Patients brought by atresia. 34 cases
Patients with complaint of delayed speech or inability to respond to sound.

The BERA findings of 15 cases studied:
Normal hearing sensitivity. 53
Mild hearing impairment. 12
Moderate hearing impairment. 21
Severe hearing impairment. 30
Profound hearing impairment. 34
Total 150

Out of 53 patients with normal hearing sensitivity, 33 were sent for adaption, 26 patients were found to be mentally subnormal and 5 patients had multiple anomalies. Out of 12 patients with mild hearing impairment nine patients had history of ear discharge and out of 21 patients with moderate hearing loss 10 had history of ear discharge either in one ear or in both ears and three patients had congenital anomalies of the ear and four had a history of high risk factors. Out of 30 patients with severe hearing impairment 22 had high risk factors contributing to their history and of 34 patients of profound hearing loss, 23 had high risk factors contributing to their history.

Out of 82 high risk cases, 22 had severe hearing loss, 23 had profound hearing loss, four had moderate hearing loss, 33 had normal hearing sensitivity. All the above cases were sent for further rehabilitative procedures as per their requirement.

Conclusion

BERA is a very useful in early detection of hearing loss and planning rehabilitative procedures. In case of multiple handicaps, BERA is the only test which can give accurate picture of hearing sensitivity. In cases of high risk babies, BERA should be carried out as a routine procedure to detect hearing loss. BERA test helps us to conclude regarding the cause of delay in speech and language development. BERA is the only tool which can confirm the normal sensitivity of hearing whenever required.

References
Chaturvedi V. N., Chaturvedi P. (1980): Assessment of hearing in small children. Indian Journal of Paediatrics. 27: 827–831.
Jerger J and Hall (1980): Effects of age and sex on Auditory Brainstem response. Archives of otolaryngology.
Jerger and Mauldin. (1978): Prediction of S N Hearing loss from BERA. Archives of otolaryngology.
awson S., Mc Cromic B., Wood S. (1995): BERA in children and normative study.
Kilney (1982): Auditory brainstem responses as indicators of hearing aid performance. Annals of otology, Rhinology and Laryngology pp 91.
Alberti P. W., Hyde M. L., Riko K., Corbin H., Abramovich S. (1984): Laryngoscope BERA in high risk neonates.
Contributed by Dr. K. K. Desarda(This paper was read in AOI conference.)



TRANSTYMPANIC LOW DOSE GENTAMICIN IN  MENIER’S DISEASE.


K.K.DESARDA.  D. BHISEGAONKAR   SHEETAL SANT   KEM HOSPITAL PUNE.


ABSTRACT:

Current role of transtympanic gentamicin therapy in the management of unilateral meniere’s disease is discussed in detail with its efficacy in  the management.
Transtympanic low dose gentamicin infusion is an alternative to surgical labyrinthectomy and Vestibular nerve section for the treatment of refractory vertigo associated with Meniere’s Gentamicin, the current drug of choice provides excellent vertigo control and is a less Invasive method to destroy the vestibular labyrinth. The goal of the treatment is to eliminate the abnormal vestibular inputs from the vestibular inputs from the diseased ear without adversely affecting the hearing. It’s salutary effect results from the damage of both the sensory neuroepithelium and the dark cells of the labyrinth. Intratympanic low dose gentamicin may cause sensory neural hearing loss hearing loss in some patients {10–15%}. Despite this S.N.. loss, results are encouraging. 50 cases of unilateral Meniere’s disease were treated at KEM hospital, Pune, during 1996 to 2012 with the follow–up of 2 to 4 yrs. All cases were infused with low dose gentamicin transtympanically for 4 to 6 weeks. This prospective study of gentamicin infusion revealed high success rate of controlling vertigo in about 92% patients. This treatment modality offers a less invasive but effective option for treating refractory vertigo of Meniere’s disease.We strongly recommend this modality of treatment for treating Mniere’s disease.

Low dose -- our preference
The low dose method involves using 1-2 injections of gentamicin, waiting a month between injections. This variant stops vertigo 70-80% of the time, with no significant side effects at all. The low dose variant is relatively new, and there is not nearly as much data concerning outcome as the high-dose variant.
The 2nd injection is given only if there has been a vertigo spell in the 2 weeks prior. In other words, instead of titrating to the onset of damage to vestibular system (as is done for high-dose ITG/TTG), the criterion is a good effect on the disease. This simple idea seems to result in far better results. Occasionally a 3rd dose is given. Usually this results in complete vestibular loss



Key words: Gentamicin, Meniere’s, chemical labyrinthectomy, micro wick

Introduction
Meniere’s disease is a clinical disorder characterized by acute episodes of vertigo, fluctuating hearing loss, aural fullness & tinnitus2. 80% patients of Meniere’s disease are treated successfully by medical treatment. Remaining 20% who have failed medical treatment need either surgical or chemical ablation of vestibular function. Surgical procedures designed to prevent endolymphatic hydrops such as cochleostomy, endolymphatic sac shunt are falling out of favour due to high incidence of sensorineural hearing loss (20–30%). Vestibular nerve section, although very effective is a difficult surgery with significant morbidity and not uncommon complications

Schucknecht (1957) introduced transtympanic mode of delivery with streptomycin4 & Beck Schmidt (1978) first used gentamicin by the Transtympamnic route5 for treating Meniere’s disease and proved the efficacy of the gentamycin infusion. The success rate was 92–100%2,4,5,6. This prospective study of 50 patients of unilateral Meniere’s disease in KEM hospital, Pune, during 1996–2012 revealed that intratympanic gentamicin therapy has a success rate of controlling vertigo in 92% with S. N. loss in 10% of the cases. This being a safe, less invasive and readily accepted treatment modality was the choice of treatment in controlling refractory vertigo of Meniere’s disease. The intratympanic low dose gentamicin infusion (40mg/ml) buffered with 7.5% sodium bicarbonate solution was used slowly over a 10 minutes period. We have recorded our observations and post infusion results for over sixteen years and found that gentamicin therapy is an ideal option to surgical labyrinthectomy. The ease with which gentamicin can be obtained and apparently lower incidence of its cochleotoxic side–effect has currently made it the preferred aminoglycoside for chemical treatment of Meniere’s disease.

Materials & methods:
(TABLE I, II, III)
50cases of 32 males & 18 females within age group of 30–70 years of proved unilateral Meniere’s disease were treated with repeated transtympanic gentamicin infusion through the grommet for 4–6 weeks on weekly basis. During the study, we have excluded CSOM., acoustic neuroma, acoustic trauma, barotraumas, diabetes, hypertension, cervical spondylosis & anaemia with the relevant investigations. Only unilateral Meniere’s disease cases were included in the study group. Prior to infusion, all patients were subjected to routine investigation such as PT Audiometry, Tone decay, SISI, caloric tests, MRI. brain especially for internal auditory meatus and posterior cranial fossa and all relevant biochemical tests. All 50 cases were given medical treatment for at least 3 months prior to gentamicin infusion therapy. The treatment includes diuretics, vasodilators, labyrinthine sedatives, antiallergics and steroids.

All cases were also informed about the side effects of this treatment such as sensorineural hearing loss in 10%, ataxia lasting for 4–6 weeks and imbalance until central compensatory mechanisms take over and the need for the head and neck Catwhorne Cooksey’s exercises after the treatment end–point. The infusion of low dose gentamicin 40 mg/ml was used with dilution with 7.5% sodium bicarbonate solution. 10mg/ml gentamicin was slowly infused intratympanically by poster inferior quadrant myringotomy through grommet .

The study ear was elevated by 45 degrees and infusion continued slowly over 10 minutes. The patient maintained the supine position with the study ear above for about 45 minutes post–infusion in the recovery room. The infusions were administered on weekly basis for 4–6 wks. Depending on clinical response in controlling vertigo. The end point of the treatment was total relief from vertigo and associated symptoms. The morbidity of unsteadiness/dysequilibrium, S.N. loss and the appearance of spontaneous nystagmus were cardinal signs of the efficacy of the gentamicin infusion.

Table I: Age distribution:
Age group (years) No. of cases
30–40
41–50
51–60
61–70
Total 06
13
22
09
50


Table II – The sex ratio:
Sex No. of cases
Male
Female
Total 32
18
50

Table III – Transtympanic infusions required:
No. of Infusions Cases
1–2
3–4
5–6
Total 10
23
17
50

The results (As per guidelines of AAO–HNS, 1985) 7:
Table IV: Vertigo Relief (n=50)
Vertigo control No. of patients Percentage
Complete 36 72
Substantial 10 20
Limited 02 04
Insignificant 02 04
Worse 00 00

Table V: Hearing loss (n=50)
Hearing loss No. of patients Percentage
Worsened 05 10
Unchanged 37 74
Improved 08 16


Table VI : Tinnitus control (n=50)
Tinnitus control No. of patients Percentage
Absent 09 18
Improved 36 72
Unchanged 05 10

Table VII: Aural Fullness Control (n=50)
Aural Fullness Control No. of patients Percentage
Absent 31 62
Improved 19 38
Unchanged 00 00


Table VIII : Post Treatment  Caloric Response

Post Treatment Caloric Response          No. of patients Percentage
No. response                36         72
Poor response                10         20
No. Change                04         08

Table IX: Comparison of Results of various Authors.

          Authors        Vertigo Control       Hearing Loss
Beck & Schmidt (1978)5                95%              15%
Odkivist (1988) 8                95%              22%
Nedzelski (1993) 9               100%              37%
Lorne (1993) 10               100%              41%
Susanne & Pyykko (1995) 11                90%              32%
KEM Hospital Pune, Study (KKD)                92%              15%

It was observed that 4–6 wks period was taken to achieve excellent vertigo control. Post infusion audio vestibular tests were done in all cases to record the observations and results.


Results: (Table–IV, V, VI, VII, VIII)

During study, we have recorded complete control in 36 cases (72%), limited in 2 cases (4%) and insignificant in 2 cases (4%). The hearing loss worsened in 5 cases (10%), improved in 37 cases (74%) & not improved in 6 cases (18%). The tinnitus was absent in 9 cases (18%), improved in 36 cases (72%), and unchanged in 5 cases (10%). The aural fullness was absent in 31 cases (62%), improved in 19 cases (38%) and unchanged in 0 cases (0%).

Discussion: (Table IX)

One of the exciting new developments in inner ear research is the feasibility to place medications directly into the inner ear. Transtympanic low dose gentamicin infusion can be done by several methods such as transtympanic injection13, Micro Wick of Silverstien1, Microcatheter4, myringotomy & grommet, etc. the gentamicin is the current amino glycoside of choice because it is less cochleotoxic than streptomycin3 (John Shea, 1994) 13. Gentamicin has its ototoxic effect on the sensory neuroepithelium and it destroys the endolymph secreting cells (dark cells of utricle, base of ampullae & lateral wall of crus communes) 14.

We have chosen the myringotomy & grommet route for its simplicity and the repeated procedures required during the treatment. Since this is an office procedure, can be repeated on weekly basis, easily accepted by all the patients and is noninvasive and cost effective, this mode of drug delivery appeared to be the best to us.

Review of the literature revealed that results obtained in vertigo control and hearing loss are variable. Beck & Schmidt (1978) 5, had vertigo control was 95% and S. N. hearing loss was 15%. Odkivist (1988)8, had 95% vertigo control and 22% S. N. loss, Nedzelski (1992)9, had 100% vertigo control and 37% S.N. loss. Lorne (1993) 10 also had 100% vertigo control and 47% S. N.Loss. Susanne and Pyykko (1995)11 showed 90% vertigo control and 32% S.N. loss. Our study revealed 92% vertigo control and 15% S.N.loss.

From the study it appears that there are some disadvantages for gentamicin therapy such as 10% risk of hearing loss. Tinnitus and aural fullness may persist, and it is also difficult to regulate the actual degree of diffusion into perilymph bypassing the cohlea. It was also noted that there are various factors altering absorption of gentamicin in the inner ear like the thickness of round window membrane, scarring and adhesions in middle ear, head position and dependency or round window, potency of eustatian tube, rate of turnover of perilymph and endolymph and individual susceptibility to ototoxic gentamicin3.

The concentration of intratympanic gentamicin is most important in predicting the degree of ototoxicity while the duration of therapy appears to be less significant15. The optimal treatment regimen for Meniere’s disease will be such that vestibular hypo activity will be achieved but there will be no hearing loss.

It was also observed during the study that the no response to gentamicin infusion is probably be due to be central lesion e.g. migraine, micro vascular compression or it may be a bilateral Meniere’s disease or it could be due to the round window adhesions (which prevents proper passage of the drug delivery  to the inner ear) or other causes of vertigo. Due respect must be given to the accurate diagnosis of the Meniere’s disease and until one is very very sure about the diagnosis, one should not try this treatment. The other modality of treatment is nonchemical ablation of the vestibular endorgan by ultrasonic and cryosurgery which is not easily available at all the centeres2.

In our study all cases were administered medical treatment for 3 months before the transtympanic infusion. The follow–up was kept on regular basis at 3 months, 6 months, and yearly after the completion of the treatment. It was our observation that six patients (12%) developed irritative nystagmus following transtympanic gentamicin perfusion during the treatment, which recovered in 2 weeks time. This unique new finding may represent a recovery phenomenon resulting from a temporarily reversible ototoxic effect in the treatment ear. Despite small percentage of S. N. loss (10%) the results are encouraging with gentamicin infusion treatment

.


Author’s Conclusion:

Interest has been growing in the intratympanic application of medicine for the control of Meniere disease and other otologic maladies. Although the use of the aminoglycosides streptomycin and gentamicin has received the most attention, other medications, including dexamethasone and lidocaine, have also been given transtympanically. Despite the growing amount of research, many questions remain un ansered regarding the efficacy, safety, and dosing regimens of these treatments.
Optimal methods of inner ear drug delivery will depend on toxicity, therapeutic dose range, and characteristics of the agent to be delivered. Advanced therapy development will likely require direct intracochlear delivery with detailed understanding of associated pharmacokinetics.
Transtympanic  low dose gentamicin infusion has a consistent vertigo control (92%), is relatively inexpensive, easy to perform under local anesthesia as an office procedure and without significant morbidity. This chemical ablation provides a reasonably safe and effective method for controlling acute, recurrent vertigo in patients of Meniere’s disease who have failed medical therapy.
Intratympanic therapies offer an advantage over endolymphatic sac or destructive surgeries in that injections can be repeated with minimal costs and morbidity, and may be titrated to clinical response.

We strongly recommend this modality of treatment for severe, unilateral, refractory intractable vertigo of Meniere’s disease before destructive surgery is contemplated because long–term success with this procedure is significantly greater than with sac surgery or vestibular neurectomy.

References
Silverstein H. (1999) : Use of a new device, the Micro Wick (tm) to deliver medication to the inner ear. ENT Journal 79:8.
Scott Brown’s Otolaryngology, 6th Edition (1997): Butterworth & Heinemann Publication, Meniere’s Disease, 3:19:1–3:19:38.
Otolaryngologica Clinics of North America, Hirsh B. E., Kamerer D. B. (Dec. 1997): Role of chemical labyrinthectomy in the treatment of Meniere’s disease, Vol. 30, No. 6, 1039–1049.
Schuknecht H. F. (Dec.1997) : Ablation therapy in the management of Meniere’s disease. Acta Otolaryngology supplement (Stockh), 13:1–41.
Beck C., Schmidt C. L., (1978) : Ten years of experience with intratympanically applied streptomycin (Gentamicin) in the therapy of morbus Meniere, Archives Otolaryngology 221:149–152.
Surgery of the ear, Shambaugh, Glasscock, 4th Edition. W. B. Sounder’s Publication, Surgical treatment of periferal vestibular disorders, 467–500.
Pearson B. W., Brackmann D. E. (Chairman) (1985): Committee on hearing and equilibrium guidelines for repeating treatment results in Meniere’s disease. Otolaryngology Head and Neck Surgery, 13:579–581.
Odkvist L. M. (1988): Middle ear ototoxic treatment for Meniere’s disease. ACT Otolaryngology (Stockh) supplement 457:83–86.
Nedzelski J. M., Bryle G. E., Pfleiderer A. G.(1993): Treatment of Meniere’s disease: Update of an ongoing study. American Journal of Otolaryngology 14:278–282.
Lorne S. Parnes, Duncan Riddel, (1993): Irritative spontaneous nystagmus following intratympanic gentamicin for Meniere’s disease, Laryngoscope 103:745–759.
Susanna K., Pyykko I., Ishizaki H. & Aalto H., (1995): Effect of intratympanically administeree gentamicin on hearing & tinnitus in Meniere’s disease. Acta Otolaryngology (Stockh) supplement, 520:184–185.
Hirsch B. E., Kamerer D. B. (1997): Intratympanic Gentamicin in Meniere’s disease. American Journal of otolaryngology, 18:44–51.
John Shea Jr. & Xianxi G. E. (April 1994): Streptomycin perfusion of the labyrinth through the round window plus intravenous streptomycin, Otolaryngologc clinics of North America 78:542–561.
Kimura R. S. (1979): Distribution structure & function of dark cells in vestibular labyrinth American journal of Otolaryngology 78:542–561.
Mangnuson M., Paloan S. (1991): Delayed onset of ototoxic effects of gentamicin in the treatment of Meniere’s effects of gentamicin in the treatment of Meniere’s disease, Acta otolaryngology (Stockh) 111:671.
Address for correspondence
Dr. K. K. Desarda
Benali, Karve Road, Nal Stop,
Pune 411 004, Maharashtra, India.

Contributed by Dr. K. K. Desarda


.


          TRAGAL CARTILAGE IN MIDDLE EAR RECONSTRUCTION


Desarda K. K.a Dr. Nilima. Kharade,Dr.Sheetal (ENT Residents)
Professor and head department of ORL, KEM hospital, Pune.
Chief residents department of ORL, KEM hospital.
This paper was read at AOI conference, Cochin, January 2000.

Email: kdesarda@gmail.com
Address:
Dr.K.K.Desarda.MS.FACS.DLO.(Lond)
Prof.Emeritus & Head Otolaryngology,
KEM Hospital, Pune

Abstract

Cartilage has become an alternative to more traditional grafting materials for the tympanic membrane reconstruction.  Vein graft was very popular for many years, but has been replaced by temporalis fascia.  Perichondrium and dura matter have also being used.  Currently temporalis fascia and perichondrium are most commonly materials used. Cartilage has shown itself to be a novel material with high success rate in more challenging cases such as retraction pockets, recurrent perforation, atelectasis, cholesteatoma and ossicular chain reconstruction

To date, temporalis fascia and perichondrium remain the most commonly employed materials for closure of tympanic membrane perforations.  The success rate in TM reconstruction with these materials approaches 90%. In certain situations, such as the atelectatic ear, cholesteatoma, and revision tympanoplasty, the results with these materials have not been as gratifying.  Fascia and perichondrium have been shown to undergo atrophy and subsequent failure in the postoperative period.  This has led to the use of cartilage, which is a less compliant, more rigid material that resists resorption and retraction.  It has also been shown by different studies that it is well tolerated by the middle ear and hearing results have been comparable with those of fascia and perichondrium

The study presents six hundred ear operations of varied middle ear pathology using tragal cartilage and perichondrium as a choice graft. The technical advantages of tragal perichondrium graft in myringoplasty, ossiculoplasty, ossiousplasty, and mastoid cavity obliteration are discussed.





KEM Hospital Pune.

The study was conducted at K.E.M. Hospital, ENT department during 1980 to 2000. we have recorded our observations and results and concluded that tragal perichondrium and cartilage is an ideal graft material for reconstructive tympanoplasty. The objective of study was to assess the efficacy of tragal perichondrium and cartilage, the functional capacity in restoring hearing acuity, it’s mechanical survival, it’s extrusion rate and it’s functional integrity in tympanomastoid reconstruction.

Keywords: Cartilage, Perichondrium, Sialastic.

Introduction




INTRODUCTION:


The technique of ‘Reconstructive Tympanoplasty’ has been improved and refined ever since the introduction of operative microscope. The methods of radical and modified radical mastoid operations have not changed for decades except for minor variations. The innumerable graft materials being used to restore the dry and functioning ear. The autologous, homologous and allograft, synthetic materials lik plastics, ceramics, hydroxyapatite and golds were used but none of these have established their universal acceptability as a proved graft except the autologous grafts (cartilage, ossicles, fascia). The functioning and survival of each graft material varies as each one has certain advantages ad disadvantages and technical problems during and after surgery.
Cartilage has become an alternative to more traditional grafting materials for the tympanic membrane reconstruction.  Vein graft was very popular for many years, but has been replaced by temporalis fascia.  Perichondrium and dura matter have also being used.  Currently temporalis fascia and perichondrium are most commonly materials used. Cartilage has shown itself to be a novel material with high success rate in more challenging cases such as retraction pockets, recurrent perforation, atelectasis, cholesteatoma and ossicular chain reconstruction.

To date, temporalis fascia and perichondrium remain the most commonly employed materials for closure of tympanic membrane perforations.  The success rate in TM reconstruction with these materials approaches 90%. In certain situations, such as the atelectatic ear, cholesteatoma, and revision tympanoplasty, the results with these materials have not been as gratifying.  Fascia and perichondrium have been shown to undergo atrophy and subsequent failure in the postoperative period.  This has led to the use of cartilage, which is a less compliant, more rigid material that resists resorption and retraction.  It has also been shown by different studies that it is well tolerated by the middle ear and hearing results have been comparable with those of fascia and perichondrium

 We present our experince of twenty years (1980–2000) in using ‘Tragal Cartilage and Perichondrium’ in the reconstructive tympanoplasty. This study includes 600 cases of varied middle ear pathologies grouped in to four main divisions such as myringoplasty, ossiculoplasty, ossiousplasty (for defects in attic, posterosuperior quadrant, posterior canal wall and annular defects) and cavity obliterations. This study is not a comparative study to prove the superiority of any particular graft material over another.



Principles of Cartilage Tympanoplasty

This study includes 600 cases of varied middle ear pathologies of both safe and unsafe C.S.O.M. All cases were treated conservatively for prolonged time before being subjected for reconstruction. The special attention was paid to Eustachian tube function. The relevant investigations as routine otomicroscopy, mastoid X–rays, paranasal sinus X–rays, audiometries and blood biochemistry were done.


Study Design: 600 Cases


The study was designed in four groups. Group A – Myringoplasty (n=300), Group B–Ossiculoplasty (n=110), Group C – Ossiculoplasty (n=120), and Group D – Mastoid cavity obliteration (n=70) All cases were subjected for reconstruction after eradicating the middle ear pathology by various surgical approaches. The enomeatal (n=192), endaural (n=312), postaural (n=60) and transtympanic (n=36). The age group was 15 to 55 years and males were predominant. Most of these cases were done under local anaesthesia with sedation (n=480) and smaller group under general anaesthesia (n=120).

During the study it was observed that the middle ear showed different pathologies such as perforations (n=240), adhesive otitis media (n=24), tympanosclerosis (n=36) and cholesteatomas (n=120). Statistical Analysis was done in SPSS 10.0 using chi–square test.


.
CLASSIFIED GROUPS:


Group A - Myringoplasty (n=300):


Out of 300 cases onlay grafting was done in 172 cases and inlay grafting was done in 128 cases. The tragal perichondrium and catilage was the choice graft used with excellent post of results. The success rate was 96% and failures 4% in this group. The hearing gain with SRT was achieved within 15 dB AB gap closure. The failure of 4% were subjected to revision surgery. The dry and healed middle ear was seen within three months time. The failure cases were attributed to infection, unhygienic conditions,prosthesis displacement, graft rejection and  poor follow–ups. In this group the follow up was 2 to 6 yrs. Audiometric thresholds revealed 15–20 dB A–B gap closure. The follow up was achieved in 50% of cases for 2 to 4 years.





Group B– Ossiculoplasty (n=110) (Fig.1–6):


In this group all cases were subjected for tympanomastoidectomy with ossicular reconstruction by tragal cartilage and perichondrium struts of various types as L–shape, Bow–shape and Boomrang strut. Various combinations of Incudo–stapedial assembly, malleo–stapes strut, malleo–footplate assemblies were done. In all cases sialistic sheet was used so also the anterior canal skin as covering the graft assembly. In this group the success rate was 84% and failure rate was 16%. The failures were due to infection, prosthesis displacements and extrusion of the graft. Audiometric thresholds revealed 15–20 dB A–B gap closure. The follow up was achieved in 50% of cases for 2 to 4 years.

The technique used for cartilage reconstruction with ossiculoplasty depends on the presence or absence of the malleus manubrium. In the malleus present situation, the palisade technique is very effective and also provides good acoustic benefit. The malleus-absent situation represents one of the most challenging situations for cartilage tympanoplasty and ossicular reconstruction. The perichondrium/cartilage island flap is used in these cases to prevent the prosthesis touching the tympanic graft and preventing extrusion. In these cases, the anterior portion of the cartilage is held securely in place while the posterior half is folded out to expose the trailing edge of the anterior piece of cartilage, which acts, in effect, as a neo-malleus. The distance between the stapes footplate or suprastructure and this trailing edge is measured and the prosthesis is cut to the appropriate length. The posterior portion is unfolded.  The nice thing about folding the prosthesis in half is that you can visualize the prosthesis and have precise placement.




  Different ossicular defects and their correction by cartilage struts.























Group C– Osseusplasty (attic, PSQ, PCW, annular defects) (n=70):





 



Attic,marginal,post.superior quadrants defects(cholesteatoma)


Cholesteatoma represents one of the most controversial but important pathologic conditions in which cartilage is used. The primary purpose of cholesteatoma surgery is to eradicate disease and provide a safe, hearing ear. The magnitude of the controversy regarding optimal surgical care is beyond the scope of this presentation, but cartilage should arguably be involved in each technique. The palisade technique has been very useful in the cholesteatoma setting as it gives the opportunity, if needed, to perform an ossiculoplasty in a precise way. Also, some authors prefer to leave the anterior portion of the TM without cartilage for surveillance and possible tube placement, if necessary, in the postoperative period. However, cartilage placement in the posterior aspect of the TM can certainly delay a recurrence. but, in most series, cholesteatoma will recur in the anterior portion of the TM and it can be suspected in the setting of a recurrence in conductive hearing loss. After my review of literature, I found that the recurrence rate for cholesteatoma after cartilage tympanoplasty is less than 10%.  And if we compare this rate with cholesteatoma recurrence rate overall in children, we can appreciate that is much lower than rates previously reported in the literature which ranges from 10-46%

In this group the various defects of attic, posterosuperior quaderants, posterior canal wall and annular defects were closed by tragal perichondrium and cartilage grafts. The composite graft proved to be the best than nonbiological grafts in takeup and restoring dry ears. The cholesteatoma from the defect was removed and the defect was closed with the grafts. The posterior canal wall defect was reconstructed with the tragal cartilage graft and lined by perichondrum and anterior canal wall skin. This group achieved 75% success rate and 25% were failures which needed revision surgery.



Group D– Mastoid obliterations (n=120):




Mastoid obliterations

All mastoid cavities were preoperatively treated by suction clearance, dry mopping with antifungal and antibiotic drops for about 4–6 weeks. The cavities were fashioned by smooth drilling and removing all debris, pockets of cholesteatomas etc. the tragal cartilage was arranged in the palisade manner with the perichondrium coverage and the pedicled temporalis muscle was swinged to obliterate the mastoid cavities for good healing. Periodical follow up and aural toilet were done. The cavities re–epithelised well and achieved 70% success rate. 30% failures was because of infection and poor post op. follow ups. The modified radical mastoidectomy cavities were transformed into radical cavities to achieve good healing. The problems of mastoid cavities are still unresolved despite the treatment of various modified techniques being weak.

Table I – Age group in the study:
Age group (years) No. of cases Percent
15–25 168 28
25–35 264 44
35–45 120 20
45–55 48 08
Total 600 100

Table II: Sex distribution:
Age group (years) Male (no.) Female (no.) Total
15–25 108 60 168
25–35 120 144 264
35–45 72 42 120
45–55 24 24 48
Total 324 276 600
Percent 54 46 100


Table III – Surgical approaches:
Approaches No. of cases Percent
Endaural 312 52
Endomeatal 192 32
Postaural 60 10
Transtympanic 36 06
Total 600 100

Table IV – Anaesthesia:
Anaesthesia No. of cases Percent
General 120 20
Local + Sedation 480 80
Total 600 100


Table V – Pathological defects:
Type of Pathology No. of Cases %
Performation 240 40
Adhesive Otitis media 24 04
Tympanosclerosis 36 06
Retration pocket 180 30
Cholesteatoma 120 20
Total 600 100


Discussion:

Cartilage has become an alternative to more traditional grafting materials for the tympanic membrane reconstruction.  Vein graft was very popular for many years, but has been replaced by temporalis fascia.  Perichondrium and dura matter have also being used.  Currently temporalis fascia and perichondrium are most commonly materials used. Cartilage has shown itself to be a novel material with high success rate in more challenging cases such as retraction pockets, recurrent perforation, atelectasis, cholesteatoma and ossicular chain reconstruction




Pathological-Defects

For many years the so called conservative methods of radical mastoid operations (Barany, Bondy, Citelli, Heerman, Stacke) were done in the clearance of disease but none of these proved better. At later dates Farrior, House, Lempert, Morrison added some minor variations in the technique of reconstructive tympanoplasty but still could not achieve the good results because the recurrence of the disease was very high. To modify these Victor Goodhill, Heerman and Heerman demonstrated their new techniques which prevented the recurrence of the cholesteatoma and gained the high success rates.


Distinct Advantages of Tragal Cartilage Graft

In this study 600 ear operations were performed with tragal perichondrium and cartilage as a composite graft in various types of middle ear reconstructions such as myringoplasty, ossiculoplsty, osseous reconstructions and mastoid obliterations. This study was done at K.E.M. Hospital E.N.T. department, Pune during 1980–2000. We have presented our observations of this reconstructive study of 20 years and found that the tragal cartilage is an ideal graft for the reconstructive middle ear surgery.

In the simple myringoplasty group the tragal perichondrium and cartilage achieved 96% success rate, the small, large and subtotal central perforations healed well in six weeks time. The inlay and onlay methods were used in the neotympanic reconstruction. In the total perforations and missing annulus the perichondrium angle was appropriate fit in forming the new annulus the perichondrium angle was appropriate fit in forming the new annulus. By this technique the blunting and lateralisation of the graft was prevented from the various cartilage assemblies in ossicular reconstruction achieved excellent stability and contact to bridge the gap in transformer mechanism. The incudostapedial gap was restored by cartilage sturt and maintained assembly. The malleostapedial, malleofootplate assembly proved good in restoring hearing.
 I

Ideal Graft

In TORP. & PORP ossicular graft the interposed tragal cartilage and drum have increased the ossicular stability and improved hearing to 75% (Victor Goodhill). Chronic endotympanic depression is a pathological entity which leads to atelectasis, retraction pockets and cholestestoma formation. The tragal cartilage and perichondrium composite graft intervention has prevented the recurrence of the cholesteatoma pocket adhesions and tympanosclerosis. The postop results were dryhealed middle ears with good hearing.



Results of Cartilage Tympanoplasty

During the study it was observed that middle ear patology of 40% perforaytions of safe and unsafe types, 4% adhesive otitis media, 6% tympaosclerosis, 30% retraction pockets and 20% cholesteatoma sacs. All these pathologies were corrected by radical removal and tragal cartilage reconstruction.

In mastoid obliteration the palisade cartilageplasty proved in gaining dry cavities in 70% of the cases. The Eustachian tubal obstruction was relieved by tunnelplasty and improved the good middle ear aeration. The cartilage bridge over promontory and hypotympanum assures the proper contact with stapes and in the combined approach tymparoplasty procedure the recurrence of cholesteatoma in the sinus tympani and facial recess could be prevented by incorporating the composite tragal cartilage and perichondrium. In open cavities the tympanocartige stapedopexy improved the hearing. It was our observation that biological material like tragal cartilage, perichondrium, facia or ossicles etc. are much better than nonbiological materials in reconstructive surgery.



Poor Results in Cartilageplasty


The survival rate of tgragal graft material is much better than nonbiological materials.

The extrusion rate of cartilage is very minimal as compared to the other graft materials. The review of literature revealed the different extrusion rates of different materials, such as autologous, 1.19%, isografts 3.06% the synthetics 5.04%, human dentine 7.14%, gold prosthesis 8.7%. Overall the tragal cartilage and perichondrium proved to be the best graft materials in reconstructive tympanoplasty which is universally accepted.




Table VIII :Extrusion rates of commonly used graft materials:
Graft material Extrusion rate (%)
Autograft 1.19
Isograft 3.06
Synthetic 5.04
Human Dentine 7.14
Gold prosthesis 8.70

Table IX :Results of cartilage tympanoplasty: P=0.0001 by chi square:
Group Success (%) Failure (%)
Myringoplasty 96 04
Ossiculoplasty 84 16
Osseusplasty 75 25
Mastoid obliteration 70 30

Table X – Poor results in cartilageplasty:
Causes No. of cases
Displacement 12
Fibrosis 10
Absorption 06
Infection 08
Total 36

TABLE V – Pathological Defects:
Type of Pathology No. of Cases Percet
Performation 240 40
Adhesive Otitis media 24 04
Tympanosclerosis 36 06
Retration pocket 180 30
Cholesteatoma 120 20
Total 600 100


Table VI – Ossiculoplasty:
Lesion No. of cases Percent
Attic 55 50
Posterosuperior quadrant 33 30
Posterior canal wall 11 10
Eustachian tube 11 10
Total 110 100

Table VII – Ossicular Status (300 cases): –P=0.0001 by Chi square:
Structure Normal Eroded Destroyed
Maleus 120 72 108
Incus 000 96 204
Stapes 108 00 192










 Conclusion

 The use of cartilage is experiencing a renaissance in ear surgery because it appears to offer an extremely reliable method for reconstruction of the tympanic membrane in cases of advanced middle ear pathology and Eustachian tube dysfunction.
Cartilage is particularly useful for the atelectatic ear, cholesteatoma, high risk perforation and for reinforcement of the tympanic membrane in conjunction with ossiculoplasty.
Hearing improvement can be experienced with the use of cartilage regarding the underlying pathology. Excellent clinical and experimental evidence exists to justify the use of cartilage as a grafting material in pediatric tympanoplasty. Cartilage tympanoplasty provides a tympanic membrane repair with greater structural stability and strength than traditional graft materials in many patients with challenging middle ear environments
In view of the above study we strongly recommend the tragal perichondrium and cartilage composite graft in various tympanoplasty reconstructions. The main reason being the cartilage is easily available at the site of operation, nontoxic, less, extrusion, minimum shrinkage, and lateralisation above all it is very cost effective to our patients. The hearing improvement within 15db of bone conduction has become almost a standard criterion for the analysis of surgical success.







Extrusion Rate of Commonly Used Graft Material


References:

Aeaham Evitor and Bronx NY: Tragal perichondrium and cartilage in reconstructive ear surgery, Laryngoscopy, 88 (Suppl.): 1–23,1978.
Heerman and Heerman tympanoplasty and mastoidoplasty, Laryngorhinootology, 46:370–382, May 1968.
Plester D.: Myringoplasty methods, Archieves otolaryngology, 78:310–316, Sept.1963.
House H. P.: Surgical repair of the perforated drum, Annales otorhinolaryngology, 62 : 1072–1093, 1956.
Goodhill Victor, Harris I., and Brockman S. J.: Tympanoplsty with perichondrium graft, Archieves otolaryngology, 79, 131, 1963.
Claus Jansen : Cartilage tympanoplasty, Laryngoscope, 73: 1288, 1963.
Heerman and Heerman: Fascia and cartilage palisade tympanoplasty, Archieves otology, 91 : 228–241, 1970.
Victor Goodhill: Tragal perichondrium and cartilage in tympanoplasty, Archieves otology, 85:480–491,1963.
Jansen C.: Use of perichondrium for tympanoplasty, Archives ohren, 182:610–614, 1963.
Shea J. J.: Vein graft in tympanic reconstruction, Journal of laryngootology, 74:358–362, 1960.
Contributed by Dr. K. K. Desarda



                             PRIMARY  RHINOSPORIDIOSIS  OF NASOPHARYNX.

                                  DR.K.K.DESARDA. DR. SHEETAL.DR.NEELIMA.
                                                       KEM HOSPITAL PUNE.


                  
Abstract.

A rare case of primary Nasopharyngeal rhinosporidiosis with extension to nasal cavity,maxillary sinus anteriorly and posteriorly extending to oropharynx is reported in a young male patient. The pathophysiology, clinical feature, diagnosis and management of this condition are reviewed.
Key words: Rhinosporidiosis, Rhinosporidium Seeberi

Indroduction:

Rhinospordiosis is a chronic granulomatous disease characterized by production of polyps or other manifestations of hyperplasia on mucous membrane surfaces, the etiologic agent being Rhinosporidium seeberi. The disease was First described  by seeber (1900) in Argentina. This noval pathogen commonly affects mucosa of nose, eye and upper aero-digestive tract of men and animals. Isolated deep seated Rhinosporidiosis is rare. Diagnosis is mainly based on clinical suspicion and histopathological confirmation. At present, no existing medical treatment cures the disease and endoscopic excision of the mass with cauterization of the base is considered as treatment of choice.

Case Report

A 45 year old male patient came to us with history of left nasal obstruction,epistaxis, distorted speech with nasal twang and intermittent dysphagia and headache for over 6 yrs He was treated at the local district hospital but without great relief.
The patient did not give any history of TB,DM and any majot illness in past apart from nasal obstruction , intermittent nose bleeds,and nasal speech with poor intake of food.. patient was chchectic with mild pallor, with a pulse rate of 84/minute, regular, BP-100/80 mmHg, CVS-NAD, Chest:-NAD.


Investigations:

Hb%-8.0gm% ,TLC 8000/mm ,DC-N80%,L-18%,E-2%,ESR-5mm.
Serum urea 30mg%,serum creatinine-1.42%,Urine –NAD,
AbsAg –negative,Anti HCV-negative,Elisa for HIV-negative,serum Bilirubin0.6mg%
SGPT and SGOT within normal limits

DC:-N-82%, L-17%, E-01%
FBS:-106 mg%
ESR:- 5mm in 1st hr,
Sr.
Radiological investigations: x-ray chest NAD,CT coronal PNS revealed Hughe mass occupying left maxilla,OMU blocked with mass. The mass was occupied in the nasal cavity, nasopharynx with extension to oropharynx.FNAC done for HP. Examination.which revealed rhinospordiosis.
Nasal endoscopy revealed pinkish mass occupying left nasal cavity,nasopharynx and left maxilla. and mass was,protruding in to oropharynx ,pushing the palate anteriorly.The septum was pushed to right   causing  nasal obstruction.. Clinical diagnosis of Rhinospordiosis.was made.
Patient was advised surgery and subsequently undergone endoscopic excision.
Nasal endoscopy revealed pinkish mass occupying left  nasal cavity,nasopharynx and protruding in to oropharynx,pushing the palate anteriorly. The mass bled during the clearance. Complete endoscopic clearance of the naso-maxillary-and nasopharynx was done. The complete haemostasis secured.by cauterization.The anterior and post nasal packing with BIPP was done .The  enblock specimen was sent  for histopathological examination. Post op recovery was uneventful.
Patient was given broad spectrum antibiotic,anti inflammatory agents and Dapsone theray . He was advised to continue Dapsone theray 100mg OD for six months and attend follow up clinic every 3 months for any recurrence.
    Rhinosporidosis  post surgery specimen.
   
  Enblock  resection of  Rhinosporidium of Naso-oro pharynx.
    Histopathlogy:
                                             
 H&E stain of nasal polyp showing two mature sporangia and
several immature ones with a single centrally placed spore.



.
DISCUSSION:

Rhinosporidiosis is a chronic granulomatous disease characterized by production of polyps or other manifestations of hyperplasia on mucous membrane surfaces. The etiologic agent is Rhinosporidium seeberi.Most of the early studies of rhinosporidiosis were made in India and Ceylon where the disease occurs frequently. Sporadic case have been detected and studied in many parts of the world. The systematic position of R. seeberi is still uncertain. Most investigatrs consider it has not been isolated in culture.
 Friable, highly vascular, sessile or pedunculated polyps may appear on almost any mucosal surface, and rarely secondary lesions are found on skin, probably as aresult of autoinoculation by scratching. Lesions of the mucosae often spread by extension beyond the mucocutaneous border.
Primary lesions appear most often on the nasal mucosa and are accompanied by painless itching and a profuse mucoid discharge. The lesion is at first flat and sessile. Later hyperplastic growth greatly exceeds lateral extension of the lesion so that a polypoid mass much larger than the peduncle develops. The polyp may extend from the neres into the pharynx or externally over the lip and may reach weight of 20grams. It is friable and bleeds freely after trauma. Its surface is mucoid and papillate or so lobulate that its surface suggests that of a cauliflower. The color varies from pink to purplish red, and close examination of the surface mayh reveal minute white sports which are the mature sporangia of the fungus.
Lesions of the eye may cause symptoms similar to those produced by a foreign body, lacrimation or photophobia. Growth of the polyp may cause eversion of the lid. Lesions on th eskin being as papillomas and become warty with inclusions of myxomatous material. They are relatively painless except when on the sole of the foot and when they become so large as to be uncomfortably heavy. Dissemination to visceral organs is rare.

Differential diagnosis:

Typical lesions of rhinospordiosis can be recognized usually by the pink to purple colour, friable consistency and the presence of barely visible white sporangia within the polyp. Atypical lesions or those in unusual anatomical sites must be differential from warts, condylomata and hemorrhoids.

Immunology:

Little is known about the immunology of rhinosporidiosis.
Pathology:
H&E stain of nasal polyp showing two mature sporangia and
several immature ones with a single centrally placed spore

On the examination of the gross tissue, unless rhinosporidiosis has been suggested by the clinician, or by the history of the patient's geographic residence, the pathologist may consider the specimen an ordinary nasal polyp. The correct diagnosis can usually be made without difficulty on examination of routine H and E stained slide. Under the scanning lens of the microscope, although the polypoid structure may be evident, the histopathologic pattern differs greatly from tht of the common nasal polyp. The most striking feature is the presence in the stroma or epidermis of numerous sharply defined globular cysts which usually vary from 10 to 200.. Some of the cysts may be partly collapsed, assuming a semilunar shape. In contrast to the loose, edematous, myxomatous stroma of the ordinary nasal polyp, the stroma in rhinospordiosis is rather dense. There is a chronic inflammatory reaction in which neutrophils, plasma cells and lymphocytes are prominent. In contrast to the usual nasal polyp. Eosinophils are inconspicuous. Occasionally purulent microabscesses occur.
The cysts of all size have a sharply defined chitinous appearing wall. In a large maturing cyst the wall alone may be 5 &#61549; thick. Histologically, rhinosporidiosis should be differentiated, specially in immuno-suppressed persons with other fungal infection like Coccidioides immitis.

Epidemiology:

Although rhinosporidiosis is seen most often in children and young adults, it occurs at any age. No racial difference in susceptibility are recognized. The disease is seen much more frequently in men than in women, but the extent to which this difference is related to greater frequency or severity of exposure is difficult to evaluate. Infections are seen most often in labourers and in those with frequent exposure to water of streams and pools.

Geographic distribution:
Rhinosporidiosis is found must often in India and Ceylon, but it is reported also from the East Indies, the Malay, States, the Philippines, Iran, South Africa, Italy, England, Scotland, Southern United States, Mexico, Cuba, Argentina, Brazil and Paraguay.
Source of infection:
The disease is not contagious, and sources of infection are exogenous. The frequent history of prior extended to water of pools and rivers and the occurance of multiple cases among those members of a group of workmen most intimately and repeatedly exposed to water source suggest the R. seeberi has a natural habitat in water. Rhinosporidiosis was observed in workmen who dived under water to bring up san din buckets, but not in their associates who carried the sand from the water's edge. It has been suggested that water insects or fish may be hosts of the fungus.

Laboratory diagnosis:

Direct examination of the surface of the polyp may reveal the subsurface position of sporangia which are white and so large (up to 350 &#61549; in diameter) that thery can be seen with naked eye. Dissection of sporangia or excision and microscopic examination of tissue confirms the diagnosis. Culture is not successful, and the inability of R. seeberi to grow on artificial media, as well as some peculiarties about its reproductive cycle in tissues, have raised the question whether it is actually a fungus.
It is resembles in general appearance and in manner of sporulation some species of synchytrium, which are obligate parasites of plants, and which produce characteristic galls on the host plant. Animal inocultion is not helpful in diagnosis. Although R.seeberi is found in natural infections of horses, mules and cows, experimental infections usually do not succeed. Recently molecular methods like polymerase chain reaction are being developed for diagnosis.

Conclusion:
 Rhinosporidiosis, a fungal infection due to Rhinosporidium seeberi, frequently produces polypoidal lesions in the nose. Sites like the conjunctiva, larynx, trachea, nasopharynx, skin and genitourinary tract are less frequently involved. Generalized rhinosporidiosis with skin and visceral involvement is extremely rare... Smears revealed numerous sporangia and spores of R seeberi. There were no mucocutaneous lesions. Histologic examination confirmed the  diagnosis of Rhinospordiosis.  The FNAC diagnosis of rhinosporidiosis is specific. Preoperative diagnosis is possible even in cases with unusual clinical presentations.
Rhinosporidiosis should be suspected or considered in all cases of swellings of nose,
 nasopharynx and skin. Although disseminated Rhinosporidiosis is very rare, still
remains a possibility and requires a different mode of treatment. Presently the medical
treatment of Rhinosporidiosis is not satisfactory and requires further study and
research.Meanwhile patients should receive Dapsone therapy for over six months.


References:
1. Caldwell, G.T. and Roberts, J.D.: Rhinosporidiosis in the United States J.A.M.A. 1938; 110,1964.
2. Karunaratne W.A.E.: Rhinosporidiosis in Man, London, Athlone Press, 1964.
3. Weller, C.V. and Riker, A.D.: Rhinosporidiosis in Man, London, Athlone Press, 1964.
4. Weller, C.V. and Riker, A.D.: Rhinosporidiosis seeberi Am.J.Path, 1930,6,721-732.
5. Baron, E.J., Peterson, L.R. Finegold, S.M. New, Controversial difficult-to-cultivate or non-cultivate etiological agents of disease in Bailey and Scott's Diagnostic Microbiology, 9th Edition, Mosby, st.Louis, Baltimore, Boston, 1994; p-585.




DIODE LASER TREATMENT IN ORAL SUBMUCOUS FIBROSIS.
(KEM-PUNE STUDY)
Dr.K.K.DESARDA.
Abstract:
Oral submucous fibrosis (OSMF) is a high-risk pre-cancerous condition characterised by slowly progressive chronic fibrotic disease of the oral cavity and oropharynx, in which the oral mucosa loses its elasticity and develops fibrous bands, which ultimately leads to difficulty in opening the mouth. The malignant transformation rate of oral submucous fibrosis is as high as 7.6%. A wide range of treatments such as medical management, surgical therapy and physiotherapy have been attempted in the past with varying degrees of benefit, but none of them have been proved to be a conclusive method of treatment. . There are very few reports to correlate the clinical stage to histopathological grading in OSMF.The aim of our study was to relive trismus caused by submucous fibrosis,to evaluate the efficacy of diode laser therapy without any grafting procedures, and to maintain mouth opening with props physiotherapy.
 A hospital-based study was conducted on 65 OSMF cases who visited ENT dept. KEM Hospital Pune from 1990 -1996. A detailed history of each patient was recorded along with a clinical examination. Biopsy was performed for histopathological correlation.We have  tried all modalities like diathermy excision,skin grafts.tongue flaps,palatal flaps but without much  benefits in improving trismus for our patients  and  lastly  we adopted  Diode surgical therapy  with spring props for physiotherapy  which proved excellent  treatment in improving trismus.
Key words: submucous fibrosis, Diode laser,props,skin grafts,tongue flaps.
Email:kdesarda@gmail.com
Adress: Dr.K.K. desarda.
Prof.Emeritus & Head Otolaryngology,
KEM Hospital’Pune, India.
Inroduction:
On the Indian subcontinent, the use of smokeless tobacco in various forms is very popular. This habit, which usually involves the chewing of a betel quid (combined areca nut, betel leaf, tobacco and slack lime), has led to the development, in a large proportion of users, of a unique generalized fibrosis of the oral soft tissues, called oral submucous fibrosis.The condition is found in 10/1,000 adults in rural India and as many as 5 million young Indians are suffering from this precancerous condition as a result of the increased popularity of the habit of chewing pan masala.  Pan masala is a mixture of spices including, betel nuts, catechu, menthol, cardamom, lime and others.  It has a mild stimulating effect and is often eaten at the end of the meal to help digest food and feel comfortable.
Oral submucous fibrosis is a chronic debilitating disease of the oral cavity characterized by inflammation and progressive fibrosis of the submucosal tissues (lamina propria and deeper connective tissues).  Oral submucous fibrosis results in marked rigidity and an eventual inability to open the mouth.  The buccal mucosa is the most commonly involved site, but any part of the oral cavity such as the soft palate, pterygomandibular raphe, the anterior pillars of fauces and even the pharynx can be involved.
 The treatment of patients with oral submucous fibrosis depends on the degree of clinical involvement. If the disease is detected at a very early stage, cessation of the habit is  sufficient. Most patients with oral submucous fibrosis present with moderate-to-severe disease which is irreversible. Medical treatment is symptomatic and predominantly aimed at improving mouth opening.. We have  treated stage II  with medical treatment and injection kenacort for six weeks and  Stage III  & iv with severe fibrosis + trismus + dysphagia with Dioded laser therapy and achieved excellent results with inter-incisor opening ranging between 32 to 36 mm .
Because of developments in Diode Laser technologies, it has found great applications in surgery due to improved power and precise controllability. It has found great applications in oral surgery practice as well as in other areas. By changing the wavelengths we can control the energy levels and other desired properties that determine incision quality and coagulation parameters.
PATHOGENESIS OF ORAL SUBMUCOUS FIBROSIS:
    ;

Diode lasers built with semiconductor materials are portable and very compact in size and can be used in different modes such as pulsed or continuous mode. Diode laser surgery can be successfully used in surgical treatment of Submucous fibrosis. On the Indian subcontinent, the use of smokeless tobacco in various forms is very popular. This habit, which usually involves the chewing of a betel quid (combined areca nut, betel leaf, tobacco and slack lime), has led to the development, in a large proportion of users, of a unique generalized fibrosis of the oral soft tissues, called oral submucous fibrosis.The condition is found in 10/1,000 adults in rural India and as many as 5 million young Indians are suffering from this precancerous condition as a result of the increased popularity of the habit of chewing pan masala.  Pan masala is a mixture of spices including, betel nuts, catechu, menthol, cardamom, lime and others.  It has a mild stimulating effect and is often eaten at the end of the meal to help digest food and as a breath mint.1
Oral submucous fibrosis is a chronic debilitating disease of the oral cavity characterized by inflammation and progressive fibrosis of the submucosal tissues (lamina propria and deeper connective tissues).  Oral submucous fibrosis results in marked rigidity and an eventual inability to open the mouth.  The buccal mucosa is the most commonly involved site, but any part of the oral cavity such as the soft palate, pterygomandibular raphe, the anterior pillars of fauces and even the pharynx can be involved.The condition is well associated with areca nut chewing; a habit practiced predominately in Southeast Asia and India. Worldwide, estimates of oral submucous fibrosis indicate that 2.5 million people are affected, with most cases concentrated on the Indian subcontinent, especially southern India.  The rate varies from 0.2-2.3% in males and 1.2-4.5% in females in Indian communities.  The migration of endemic betel quid chewers has also made oral submucous fibrosis a public health issue in many parts of the world, including the United Kingdom, South Africa, and many Southeast Asian countries.
A variety of aetiological factors including capsaicin, betal nut alkaloids, hypersensitivity, autoimmune genetic predisposition  and malnutrition have been suggested by various authors.The importance of this disease lies in its inability to open the mouth and dysplasia giving rise to malignancy.  The incidence of malignant change to squamous cell carcinoma in patients with OSMF ranges from 2 to 10%. Submucous fibrosis typically affects the buccal mucosa, lips, retromolar areas and the soft palate. Occasional involvement of the pharynx and esophagus is seen. Early lesions present as a blanching of the mucosa, imparting a mottled, marble-like appearance. Later lesions demonstrate palpable fibrous bands running vertically in the buccal mucosa and in a circular fashion around the mouth opening or lips.  As the disease progresses the mucosa becomes stiff, causing difficulty in eating and considerably restricting the patient's ability to open the mouth (trismus). If the tongue is involved, it becomes stiff and  atrophic.
Mucosal petechiae are seen in more than 10% of cases and most patients complain of a burning sensation, often aggravated by spicy foods.  Salivary flow is diminished and blotchy melanotic mucosal pigmentation is often seen. More than a fourth of affected persons develop precancerous leukoplakia of one or more oral surfaces. Once present, oral submucous fibrosis does not regress, either spontaneously or with cessation of betel quid chewing.Histologic findings in oral submucous fibrosis are generally characterized by diffuse hyalinization of the subepithelial stroma, atrophic epithelium and intercellular oedema, with or without keratosis, epithelial dysplasia, chronic inflammation and fibrosis in the minor salivary glands in the area of quid placement; and atrophy of the underlying muscle.
MATERIALS AND METHODS
  A total of 65 histologically proven cases of advanced oral submucous fibrosis having a mouth opening of less than 10 mm were treated by Diode laser excision. The procedure involved (1) bilateral release of fibrotic bands  .(2)extraction of bilateral upper and lower molars .3) Temporary acrylic prosthesis insertion between upper and lower molars bilaterally. 4) after six weeks patients were given spring prosthesis for extensive physiotherapy. 5)  Pre and post operative inter-incisors distances were measured  by caliper and recorded.6) supportive IV fluids, vitamins. and antioxidants therapy was continued for further three months. 7) Regular followup was done and results were assessed by comparing the  different modalities  responses in preoperative & postoperative maximum mouth opening.8) No grafting  procedures were  done in our series for the scarred tissue. 9) Post op healing was excellent.
        TABLE-1, Age group & sex Ratio:
       
     
  TABLE-2. Clinical staging and grouping:
Group I Earliest stage witht mouth opening limitations with an interincisal distance of greater than 35 mm.
Group II Patients with an interincisal distance of 26-35 mm.
Group III Moderately advanced cases with an interincisal distance of 15-26 mm.
Group IVA   Trismus is severe, with an interincisal distance of less than 10 mm
Group IVB Disease is most advanced, with  dysphagia  & premalignant and malignant changes in the mucosa
 
      TABLE-3 Grouping of Different modalities treatment result wise:
Grouping Procedure Cases Result
Group 1 Simple diatheramy excision                 25 cases             Triusmus opening short term with scarring
Group 2 Skin grafting                                       15 cases             High failure because of flap necrosis
Group 3       Tongue flaps                                       10 cases             Initial results good but very cumbersome for pts. Tongue flap necrosis, speech problems
Group 4 Diode laser therapy                           15 cases             Excellent long term results with improved                                    

Pre-Operative pictures of OSMF
                                       
Early branching in OSMF                Depapilliation of Tongue             severe blanching of tongue                   Bud-like uvula in OSMF        
                                                                   
                                     
        Buccal Lesion                                  Lower lip lesion                                Tongue lesion                              Retromolar lesion.

      Diode Laser Procedure:.

Under GA with tonsillectomy position the mouth gag(Boyle Davis) is inserted inthe oral cavity and the last upper and lower molars on either sides extractedand haemostasis secured. Next with Diode Laser the fibrotic bands from retromolar region to soft palate ,anterior and posterior tonsillar pillars and uvula were excised.Laser beam with ideally 5 watt power is directed. to the fibrotic bands .The excision of fibrous bands was followed by forcible separation of mucosa.using  Boyl davis gag  and oral cavity  opening stretched .at its maximum..After complete haemostasis the oral cavity is cleaned and temporary acrylic prosthesis is inserted  bilaterally between the last  upper and lower molars., and maintained for full 10 days.Post operative Ryles tube feedig continued for ten days . During the post op period  oral hyegine was mainted. After the oral mucosa has healed well ,the patients were given  spring props for for extensive physiotherapy.for further six weeks.All Patients were instructed to stop chewing betel nuts or other addictive habits. Patients were taught mouth opening exercises with this spring props six to eight times day along with chewing gums exercises.The inter- inscisor measurement were recorded. All patients were given i.v.antibiotics,anti inflammatory drugs for controlling the oral sepsis and pain . Patients were followed at an interval of 2 months, 6 months and 1 year where by interincisor distance was measured and documented..
Results
In our series  65 patients were studied from age 16 years to 60 years (Mean 32years) who were habitual betel nut chewers. Males had preponderance of 68%and females of 32%. The Mean maximum mouth opening of the patients preoperatively was 10 mm. The Mean intra operative interincisor distance after fibrotic band excision was 32mm. Two months postoperatively the average mouth opening was 34 mm.and at 6 months 36 mm, and at 1 year 34mm. Patients were very happy and satisfied after this treatment.Since there is no effective treatment for oral submucous fibrosis and the conditionis  is irreversible  we have decided to adopt to Diode laser therapy which gave excellent results. All patients were kept for periodical observation and  surface leukoplakias are handled by close follow-up and  repeat biopsies for malignment change.

Submucous Fibrosis pictures showing post-operative
                                                     
  Diode laser instrument            Pre-surgical Markings             Intra of Temporary prosthesis                   Post of spring prosthesis       Post operative final opening    
     Discussion
Oral submucous fibrosis is a chronic debilitating disease associated with restricted mouth opening and poor oral hygiene.   The treatment aims at good release of fibrosis and provides long term results in terms of mouth opening.  The various conservative treatments with intralesional injections of steroids,  (kenacort) ,hyaluronidase, placental extract and physiotherapy are not beneficial to provide a long-term effect in advanced cases of oral submucous fibrosis   Surgical intervention is required in these cases.  The surgical treatment commonly followed is the release of bilateral fibrotic bands with  surgery and various grafts, tongue flaps, etc were tried but not much of benefit.
A mucosal graft is the best treatment for oral submucous fibrosis, as it is ideal graft to cover the oral mucosa, but is limited by the quantity of oral mucosal available for grafting.  Thick mucosa taken from the cheek may result in scar formation, whereas a uniform thin graft removed with a microtome from the cheek is costly and complicated.  Split-skin grafting has been tried but it has a high failure rate as fibrotic areas have less vascular supply besides retaining the coloration of skin.  Also seen is the growth of hair and sweat glands.  Skin is not suitable for grafting in elderly people due to atrophy and inelasticity.
A nasolabial flap has also been used by some surgeons and has a good survival rate, but sometimes it may be too small to cover the whole defect.  It also causes a visible scar on the face and requires a second surgery for division.   Tongue flaps are bulky and when used bilaterally causes disarticulation, dysphagia and increases the chance of aspiration.   In addition, the tongue is involved with the disease process in 38% cases   The use of bilateral, small, bipaddical radial forearm flaps for reconstruction of bilateral buccal defects requires two flaps with two microsurgeries.  The procedure is more time consuming and technically demanding, and it involves two forearm donor sites with sacrifice of the radial arteries in both the right and left hands.  Island palatal flaps again have limitation to reach posteriorly.
Of the 65 cases of OSMF studied, males were more than females. A literature survey shows a wide variation in age and sex distribution of OSMF. Some of the epidemiological surveys in India have shown a female predominance in the occurrence of this entity. A male predominance in OSMF cases was shown by Sinor et al.in India. We also observed a male predominance and the male to female ratio was 6:1. Half of the study population was in the age group of 20-29 years. This observation is different from that of Pindborg et al. who reported the maximum number of OSMF cases in the age group of 40-49 years in their study. Increase in the chewing habit of the areca nut without any tobacco and the use of various commercial products containing areca nut may explain the decrease in the age of OSMF cases due to various chewing habits. The mean age of occurrence was lower in males than in females and the difference was statistically significant (P<0.009).

Recent epidemiological studies in India and evidence from Indians living in South Africa point to the habit of chewing areca nut as the major aetiological factor of OSMF. In recent years, commercial preparations like paanmasala have become available in India and abroad. The main ingredient of these products is areca nut along with lime and catechu wrapped in a betel leaf with or without tobacco. Many patients with OSMF give a history of chewing paanmasala for very long time.
Seventy-five per cent of the patients in stage II had a habit of chewing commercially available areca nut products-"Paanmasala" and 50% of the total study population were in the age group of 20-29 years. It has been documented that paanmasala chewing was preferred by people in younger age groups (11-30 years). In addition, onset of OSMF changes occurred earlier with paanmasala chewing compared with areca nut / quid chewing. Absence of betel leaf, which has anti-oxidant properties and a consequently higher dry weight proportion of areca nut were responsible for early development of OSMF. These findings are of great concern because younger individuals are at greater risk as it has been well established that OSMF is a premalignant and crippling condition of the oral mucosa.
 The treatment aims at good release of fibrosis and provides long term results in terms of mouth opening.  The various conservative treatments with intralesional injections of steroids, hyaluronidase, placental extract and physiotherapy are not beneficial to provide a long-term effect in advanced cases of oral submucous fibrosis   Surgical intervention is required in these cases.  The surgical treatment commonly followed  in our series is the release of bilateral fibrotic bands with Diode Laser.with extraction of both upper lower molars  followed by post op.props for  extensive physiotherapy. We have achieved excellent results in all cases.
Conclusion
In this study, the occurrence of OSMF was higher in the younger age group of 20-29 years. The prevalence of OSMF was more in males than in females with a ratio of 6:1. The number of patients with a paanmasala chewing habit (68.0%) was higher than the number of patients with betel nut (17.4%) or betel quid chewing habits (14.6%). The chewing of paanmasala was associated with earlier presentation of OSMF as compared to betel nut chewing. Significant and direct correlation to the manifestation of OSMF was seen with frequency rather than duration of chewing.

The maximum number of patients (74.3%) as well as most of the paanmasala chewers were in clinical stage II. Although various degrees of epithelial dysplasia were observed, malignant transformation was not seen. There was no correlation between clinical staging to histopathological grading. This observation could be explained by the fact that patients with higher histopathological grading could have had more collagenous bands in the posterior region, which restricted the mouth opening. Chronic inflammatory cell infiltrate was observed in a large number of cases in histopathological grade I but less so in higher histopathological grades, possibly due to a stabilisation of the lesion and a decrease in the levels of proinflammatory mediators.   The follow up examinations after the surgery showed significant improvement in  mouth opening . The key point was extensive physiotherapy to sustain the mouth opening.  There is no doubt that diode laser surgery is very effective and less invasive technique to treat Submucous fibrosis and offers great relief to the terrible state the patients suffer because of this disease .This technique has less morbidity and is suitable for Asian population as it requires less hospital stay and less followup as compared to other surgical methods.
 
References:
1. Schwartz J. Atrophia Idiopathica Mucosae Oris. London: Demonstrated at the 11th Int Dent Congress; 1952.
2. Joshi SG. Submucous Fibrosis of The Palate And Pillars. Ind J Otolaryng 1953; 4:1-4.
3. Canniff JP, Harvey W, Harris M. Oral submucous fibrosis: its pathogenesis and management. Br Dent J. 1986 Jun 21;160(12):429-34.
          4. Cox SC, Walker DM. Oral submucous fibrosis. A review. Aust Dent J. 1996 Oct;41(5):294-9.   5.
          5. Aziz SR. Oral submucous fibrosis: an unusual disease. J N J Dent Assoc. 1997 Spring;68(2):17-9.  6.
          6. Paissat DK. Oral submucous fibrosis. Int J Oral Surg. 1981 Oct;10(5):307-12.
7. Paul RR, Mukherjee A, Dutta PK, Banerjee S, Pal M, Chatterjee J, Chaudhuri K, Mukkerjee K. A novel wavelet neural network based pathological stage detection technique for an oral precancerous condition.  J Clin Pathol. 2005 Sep;58(9):932-8.
8. Pindborg JJ. Lesions of the oral mucosa to be considered premalignant and their epidemiology. In: Mackenzie IC, Dabelstein E, Squire CA, eds.: Oral premalignancy. Iowa City, IA: University of Iowa Press, 1980: 2-12.
9. Sinor PN, Gupta PC, Murti PR, Bhonsle RB, Daftary DK, Mehta FS, Pindborg JJ. A case-control study of oral submucous fibrosis with special reference to the etiologic role of areca nut.  J Oral Pathol Med. 1990 Feb;19(2):94-8
10. Pindborg JJ. Oral submucous fibrosis: a review. Ann Acad Med Singapore. 1989 Sep;18(5):603-7.
           11. Khanna JN, Andrade NN. Oral submucous fibrosis: a new concept in surgical management. Report of 100 cases. Int J Oral Maxillofac Surg. 1995 Dec;24(6):433
12. Rao Venkat & Raju P.N: A preliminary report of the treatment of submucous fibrosis of oral cavity with cortisone. Ind. Jour. Otolarng. 1954. 59-330.  
13. Kakar PK, Puri RK, Venkatachalam VP. Oral submucous fibrosis--treatment with hyalase. J Laryngol Otol. 1985 Jan;99(1):57-9.
14. Pruitt BA Jr, Levine NS. Characteristics and uses of biologic dressings and skin substitutes. Arch Surg. 1984 Mar;119(3):312-22
15. Kavarana NM, Bhathena HM. Surgery for severe trismus in submucous fibrosis. Br J Plast Surg. 1987 Jul;40(4):407-9.
16. Pindborg JJ, Bhonsle RB, Murti PR, Gupta PC, Daftary DK, Mehta FS. Incidence and early forms of oral submucous fibrosis. Oral Surg Oral Med Oral Pathol. 1980 Jul;50(1):40-4.  
17. Lee JT, Cheng LF, Chen PR, Wang CH, Hsu H, Chien SH, Wei FC. Bipaddled radial forearm flap for the reconstruction of bilateral buccal defects in oral submucous fibrosis. Int J Oral Maxillofac Surg. 2007 Jul;36(7):615-9. Epub 2007 May 11.  
18. Gupta RL, et al: Role of collagen sheet cover in burns—A clinical study. Indian J Surg.  40:646, 1978
19. Gupta RL, et al: Fate of collagen sheet for artificial created wounds. Indian J Surg.   40:641, 1978
20. AAlexander JM, Rabinowitz JL. Microfibrillar collagen (Avitene) as a hemostatic agent in experimental oral wounds. J Oral Surg. 1978 Mar;36(3):202-5
21. GGüngörmüş M, Kaya O. Evaluation of the effect of heterologous type I collagen on healing of bone defects.
J Oral Maxillofac Surg. 2002 May;60(5):541



Nasopharyngeal rhabdomyosarcoma

Abstract
Nasopharyngeal Rhabdomyosarcoma, a common soft tissue sarcoma in children, is a rare identity, so far only four–cases have been reported as per the review of literature. Ours is the fifth case presented exclusively in the nasopharnx, which is, hereby reported. The prognosis of this clinical entity is always gloomy and the modality of the treatment is always surgery, chemotherapy and radiotherapy.

Case report
A 5 year old boy was referred to ENT department of K.E.M. hospital with the complaints of nasal obstruction, noisy breathing and mouth breathing for over four months. There was no history of bleeding from the nose or any other significant ENT problems. Clinical examination revealed both nostrils clear with no evidence of any nasal mass occluding the nasal passage. Airways were blocked. Posterior rhinoscopy revealed huge polypoidal and grape like mass arising from the nasopharynx and hanging in the oropharynx pushing the soft palate downward and forward. Palatal movements were normal and there was no neurological involvement. Rest of the ENT examination was insignificant. Rest of the ENT examination was insignificant. Routine hematological tests and urine exam was normal. X–ray nasopharynx and compromising the nasal airway. Provisional diagnosis of nasopharyngeal malignancy was made. CT scan could not be done because of poor economical status of the patient. He was advised surgery and was subjected to excision under G. A. with routine tonsillectomy position. With Boyle–Davis gag, mouth was opened and with adenoid curette the whole mass was curetted. Bleeding was not alarming and controlled with post nasal packing. Postoperative recovery was uneventful. The mass sent for histopathology revealed rhabdomyosarcoma. Patient was advised post–op radiation and chemotherapy. Post operative recovery was uneventful and his symptoms of mouth breathing noisy breathing and nasal obstruction was cleared.

Dissussion
Rhabdomyosarcoma is a highly malignant tumor of childhood arising from the rhabdomyosarcoma of mesenchymal connective tissue. It frequently involves the head and neck region as orbit, nasopharynx, cheek, external ear, maxilla, tonsillar area and soft palate.

The presentation in the nasopharynx is very rare. Only four cases so far have been reported in the literature. Ours is another rare case which exclusively presented in the nasopharynx.

The first mention of this pathological entity was made by Weber (1855), Mason and Soule (1956). The histological classification was made by Wills R. A. (1967). Histologically, the tumor is characterized by round or spindle cells with distinct granular vacuolated cyto plasm.

The Rhabdomyosarcomas can be divided into four subtypes
  • Embryonal and embroyonal botryoid.
  • Alveolar.
  • Pleomorphic.
  • Mixed.
The embryonal is most frequently encountered rhabdomyosarcoma of childhood (80%). The disease usually runs a rapid course unless diagnosed and treated promptly. Clinically, it may be mistaken as antrochoanal polyp or angiofibroma or hypertrophic adenoid. The tendency of lymph node metastasis is more in embryonal rhabdomyosarcoma. Hemotogeneous spread to lung and bones are also not uncommon. Prior and Stoner (1957), Vieta et al (1962). Mason and Soule (1963).

The prognosis of rhabdomyosarcoma is very gloomy with less than 10% long time survival. The treatment modality adopted is a multi modality therapy. This entails surgery, chemotherapy and radiotherapy.

References

  • Horn R. C. Jr. Enterline HT (1958). Rhabdomyosarcoma a clincopathological study and classification Cancer11, 181.
  • Masson J. K. and Soule EH (1965). Embryonal rhabdomyosar coma of Head and Neck. American Journal of Surgery 110,585.
  • Stout A. D. (1946). Rhabdomyosarcomas of the skeletal muscles. Annals of Surgery 123,447.
  • Vieta L. R. Guraieb SR and Obregon MA (1962) Archives of Otolaryngology 75,248.
  • Wilis R. A. (1967) Pathology of Tumors 4th edition. Editorial Butterworth, London.


Management of Myasthenia Gravis in ENT Practice

Dr.K.K.Desarda. Leena Parulekar .Hemant Todmal

Abstract:
Myasthenia gravis is a neuromuscular disorder characterized by variable weakness of voluntary muscles, which often improves with rest and worsens with activity. The condition is caused by an abnormal immune response.
In myasthenia gravis, weakness occurs when the nerve impulse to initiate or sustain movement does not adequately reach the muscle cells. This is caused when immune cells target and attack the body's own cells (an autoimmune response). This immune response produces antibodies that attach to affected areas, preventing muscle cells from receiving chemical messages (neurotransmitters) from the nerve cell.
The cause of autoimmune disorders such as myasthenia gravis is unknown. In some cases, myasthenia gravis may be associated with tumors of the thymus (an organ of the immune system). Patients with myasthenia gravis have a higher risk of having other autoimmune disorders like thyrotoxicosis, rheumatoid arthritis, and systemic lupus erythematosus.
Myasthenia gravis affects about 3 of every 10,000 people and can affect people at any age. It is most common in young women and older men.


Introduction

Myasthenia gravis is a neuro–muscular disorder characterized by marked weakness and fatigue of muscles affecting the motor apparatus at the Myoneural junction. The management of this disease in ENT practice has been outlined briefly below. This disorder involves all age groups and any muscles in the body, but the disease shows special affinity for muscles innervated by bulbar nuclei (face, lips, eyes, tongue and neck). It is because of this affection that patients of this disorder first go to see the Otolaryngologists.

The cause of the myasthenia gravis is not known, but it is said to be a metabolic disorder. Thymus gland abnormalities have been described in some patients. It has also been said that myasthenia gravis is an auto immune disease, since multiple auto antibodies have been found in the sera of patients of this disorder. The women are more often affected than men and the disease appears between 20 and 40 years of age.


Twenty cases of proved myasthenia gravis were being treated at KEM hospital in Pune since 1978. Out of this, three patients required thymectomy, two patients went into respiratory failure and had to be subjected for tracheotomy and were subsequently put on the respirator. They died ultimately. Fourteen cases were managed on medical therapy e.g. neostigmine, alternate day steroids and supportive therapy.



Three interesting cases which were presented to our department are given below. The patients were diagnosed for oropharyngeal complaints like dysphagia, dyspnoea, dysphonia, hoarseness, diplopia and ptosis, mimicking the presentation of bulbar palsy, cricopharyngeal and nasopharyngeal malignancies, with neurological involvements.

Case Reports:

Case 1
A 40 year old man complaining of difficulty in swallowing, heaviness of speech and inability to open his eye, was referred by his practitioner to ENT OPD as the symptoms were rapidly progressing. The detailed examination of his nose, pharynx, nasopharynx and larynx did not reveal any abnormality except weak movements of palate (soft), pharyngeal wall and vocal cords. There was no evidence of any malignancy either.

Routine blood, urine, X–ray chest, ECG and lipid profiles were within normal limits. The only positive findings were ptosis, diplopia and marked fatigue. The nasal regurgitation ,dysarthria and the patient had difficulty in swallowing even liquids. A provisional diagnosis of Myasthenia gravis was made and confirmed by a positive neostigmine test.

Case 2
A 40 year old female was admitted for difficulty in breathing, hoarseness of voice and difficulty in swallowing. A routine ENT examination was done. Her nose, nasopharynx and larynx looked normal except for weakness in palatal movements, pharyngeal musculature and sluggish mobility of the cords. She had ptosis though diplopia and fundi were normal. Routine blood, urine and serum electrolyte studies did not reveal any abnormality. An X–ray of the chest revealed huge shadows in the mediastinum suggestive of thymoma. Provisional diagnosis of myasthenia gravis was confirmed by positive neostigmine test.

Case 3
A 35 year old female was attending an ENT clinic for slurring of speech, nasal regurgitation and change of voice for six weeks. She would feel fine in the mornings, but as the day advanced, the symptoms would progress and at the end of the day, she would become dyspneic and dysphonic with marked fatigue in articulation.

A routine ENT examination did not reveal any marked abnormality except weakness in palatal movements, nasal twang, and weak movements of vocal cords. Even routine hemogram, urine and chest X–ray did not reveal any abnormality. A provisional diagnosis of myasthenia gravis was made and confirmed by a positive neostigmine test.


Clinical Presentation

Most of these patients come to an otolaryngologist for their nasal or pharyngeal symptoms.
Clinical presentation is as follows:
Occular Symptoms: Ptosis, diplopia or both.
Oropharyngeal, symptoms: Dysphagia, dysarthria, dyspnoea.
Weakness of muscles of mastication and facial muscles.
Nasal twang (nasal speech), nasal regurgitation and marked fatigue in articulation.
Pharyngeal musculature weakness may cause dysphagia for liquids and solids.
Laryngeal muscle weakness may cause dyspnea, dysphonia and hoarseness of voice and weak cough reflex.
Involvement of respiratory muscle may cause respiratory striders.
Diagnosis
It is based on a detailed history of the clinical course of the disease, clinical examination and laboratory investigation. The positive finding like ptosis, diplopia, facial weakness and progressive fatigue in chewing, swallowing and speaking. The diagnosis is confirmed by a positive neostigmine test.

The hysteria, disseminated sclerosis, bulbar lesions, and polymyositis do mimic the picture of myasthenia gravis and should be excluded by appropriate tests and laboratory investigations. It is also stressed that malignancy of oropharyngeal, nasopharyngeal spaces should be kept in mind in evaluating the diagnosis. The importance of positive neostigmine test is the main stay in the diagnosis of the myasthenia gravis.


Treatment
There is no known cure for myasthenia gravis. However, treatment may result in prolonged periods of remission.
Lifestyle adjustments may enable continuation of many activities. Activity should be planned to allow scheduled rest periods. An eye patch may be recommended if double vision is bothersome. Stress and excessive heat exposure should be avoided because they can worsen symptoms.
Some medications, such as neostigmine or pyridostigmine, improve the communication between the nerve and the muscle. Prednisone and other medications that suppress the immune response (such as azathioprine, cyclosporine, or mycophenolate mofetil) may be used if symptoms are severe and there is inadequate response to other medications.
Plasmapheresis, a technique in which blood plasma containing antibodies against the body is removed from the body and replaced with fluids (donated antibody-free plasma or other intravenous fluids), may reduce symptoms for up to 4 - 6 weeks and is often used to optimize conditions before surgery.
When other treatments do not improve systems, patients may receive intravenous immunoglobulin.
Surgical removal of the thymus (thymectomy) may result in permanent remission or less need for medicines.
Patients with eye problems may try lens prisms to improve vision. Surgery may also be performed on the eye muscles.
Several medications may make symptoms worse and should be avoided. Therefore, it is always important to check with your doctor about the safety of a medication before taking it.


Medical treatment
Most of these patients are well maintained on a regime of medical drugs.
Neostigmine bromide 15 mg orally four times/day and may be increased upto 180 mg/day.
Edrophonium chloride (Tensilon) 10 mg 1/v may relieve myasthenic weakness in 20 to 30 seconds. 25–30 mg  im daily gives relief for several hours.
Ephenrine sulphate: 12 mg/day. The side effects of these drugs like abdominal cramps, nausea, vomiting may be prevented by adding an atropine like drug.
Corticosteroids and corticotropine: Encouraging results have been reported. Taking alternative day steroids orally is the recommended therapy.

Surgical treatment



Myasthenia gravis with Thymus tumor can be subjected for Thymectomy in females under 40 years, where medical treatment has failed. Complete remission may occur in 1/3rd of patients. The surgical result in men are uncertain.




Deep X–ray therapy

Those patients whose medical treatment has failed and those who have refused surgery, may be subjected for deep X–ray therapy (3000 R) to thymus in 10 to 12 divided doses. Partial remission may occur in half the patients.

Emergency Treatment

The Myasthenic crisis is an emergency and should be attended to immediately. Sudden inability to breath or swallow may occur any time. These patients should carry two ampules of 0.5 mg. of Neostigmine methysulphate to be given immediately S/c or 1/m if severe symptoms develop.

Progressive and potentially dangerous respiratory stridor may take place and will require immediate emergency tracheostomy. Therefore both, a tracheostomy oxygen and suction unit should be kept near the patient. If these facilities are not available, then the patient should be transferred to an intensive care unit for management where respirator facilities will be available. During this emergency treatment body fluids and electrolytes should be maintained.

Prognosis:

As regards the prognosis of this condition, it is always guarded. The spontaneous remission occurs frequently but a relapse is the rule. Myasthenic crisis and sudden death may also occur. The most critical period following the onset is two years.There is no cure, but long-term remission is possible. There may be minimal restriction on activity in many cases. Patients that only have eye symptoms (ocular myasthenia gravis), may progress to have generalized myasthenia over time.
Pregnancy is possible for a woman with myasthenia gravis but should be closely supervised. The baby may be temporarily weak and require medications for a few weeks after birth but usually does not develop the disorder.
            Possible Complications
  Restrictions on lifestyle (possible)
  Side effects of medications (see the specific medication)
  Complications of surgery
  Myasthenic crisis (breathing difficulty), may be life threatening
.


           References
Brumner N. G. (1972). ‘Corticosteroids in Myasthenia Gravis’. Neurology 22, COC.
Cape C. A., Utterback R. A. (1973). ‘Maintenance of ARTH in Myasthenia Gravis’. New England Journal Med. 27,288.
Kreal et al. (1967). ‘Role of Thymectomy in the management of Myasthenia Gravis’. Ann Surgery 165,111.
Warmolts J. R., Engel W. K. (1972). ‘Benefit from alternate prednisone in Myasthenia Gravis’. New England J Med. 17,286.

           Contributed by

           Dr. K. K. Desarda
         Prof.& Head otolaryngology,
         KEM Hospital Pune.



             MALIGNANT FIBROUS HISTEOCYTOMA
.
                         DR.K.K.DESARDA.  DR.S. PUNTAMBEKAR  KEM HOSPITAL PUNE.




INTRODUCTION

Malignant fibrous histiocytoma (MFH) is a tumor derived from mesenquimal tissue composed by 5 cell types  The term MFH was suggested to describe a tumor histiocytic-like with fibrous tissue . Its origin is believed to be from totipotent cells for presenting 2 different cell types. In histological terms, MFH is divided into 5 subtypes.

MFH is usually malignant when occurs on profound soft tissue. Around 3% of such tumors occur in the area of head and neck, and it is rare in larynx. New immunohistochemical and by electronic microscopy techniques have increased accuracy of histopathological diagnosis.
We described a rare case of MFH in the larynx and discussed its diagnosis and treatment.


CASE REPORT

54-year-old-male patient, smoker for 30 years was  seen in ENT OPD at KEM Hospital, Pune. in 2005 with the complaints of change of voice,odynophagia, and Foreign body   sensations in the throat for 18 months duration. There was no signs of respiratory distress or he neither had symptoms of dyspnoea, dysphagia nor important medical medical problems. Videolaryngoscopy, revealed smooth polypoidal mass on the anterior two third of left vocal cord with no paresis . There was no palpable cervical lymphadenopathy. In June, 2005, a microlaryngoscopy with polyp excision was performed and the biopsy taken from the mass was subjected for histopathological study, which was reported as  malignant fibrous histcytoma.He was then advised for surgical excision.and frontolateral left  laryngectomy was performed. The post operative recovery was excellent.He was reviewed at monthly intervals for almost two yrs, and there was recurrence seen.
 


  Videolaryngoscopy showing polypoidal mass over the left vocal cord


             Light microscopy showing large proliferation of fibroblasts mixed in several histiocytic, multinucleated cells.



DISCUSSION

The term MFH was suggested by Kauffman and Stout in 1961 in order to describe a histiocytic-like tumor with predominance of fibroblasts

MFH on head and neck area is more common in men, in a proportion of 3:1 and it affects this area only in 3% of the cases  on the skin and soft tissues. Nose, paranasal sinus and mandible are the most common areas  with malignant feature when in profound areas  There are 30 cases reported  and most of them are subglotti , preferably in men on glottic area and in women in subglottic  Patient with glottic MFH are usually older and smoker, opposing from subglottic MFH.

It is histologically divided into 5 types: pelomorphic, myxoid, inflammatory, giant cells and angiomatous  It is derived from mesenquimal tissue composed by 5 cell types: fibroplast, histiocytic, indistinguished, giant multinucleated and xantomatous cells  Its origin is believed to be from totipotent cells for presenting two different cell types. Structural analysis through electronic microscopy, immunohistochemical techniques and culture of tissue help in order to differ this type of tumor . The immunohistochemical anti-vimentin V9 was positive in this case.

This tumor often presents only clinical symptoms, such as dyspnoea, dysphagia, hemoptysis, stridor and tiredness when achieves larger sizes . Diagnosis for it is usually established after the removal of the tissue mass.

Differential diagnosis should be performed with pleomorphic rhabdomyosarcoma, fibrosarcoma, spinocellular carcinoma, angiosarcoma, hemangiopericytoma, pleomorphic liposarcoma and lymphoma

Surgery is the therapy with block resection of tumor. The hemilaryngectomy technique was chosen, though tumor was limited to left vocal fold. Cervical emptying was not performed because physical exam. did not presente lymphonode involvement. Radiotherapy is reserved to high risky patients, recurrence of non-operated patients and cases of metastasis to distance . Some authors suggest chemotherapy for other types of sarcomas .  Adjuvant radiotherapy and chemotherapy with surgery requires more studying.

MFH of the larynx is a rare type of tumor, but a high recurrence, fast growth, unexpected clinical behaviour and it tends to local and distance metastasis, especially to brain and lungs There is a lack of prognosis when there is sign of metastasis and glottic MFH, what differs from subglottic MFH


CONCLUSION

MFH is a rare type of tumor and there are few reported cases in the literature,thus makes its prognosis and therapy  difficult. Surgical therapy is the one with ample resection of tumor. It has high recurrence rate and hence periodical review is mandatory.


BIBLIOGRAPHY

1. Barnes L, Kanbour A. Malignant fibrous histiocytoma of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:1149-56.

2. Bernaldez R, Nistal M, Kaiser C, Gavilán J. Malignant histiocytoma of the larynx. J Laryngol Otol 1991;105:130-3.

3. Blitzer A, Lawson W, Biller HF. Malignant fibrous histiocytoma of the head and neck. Laryngoscope 1977, 87:1479-99.

4. Canalis R, Green M, Donard H, Hirose F, Cooper S. Malignant fibrous xanthoma of the larynx. Arch Otolaryngol 1975, 101:135-7.

5. Enzinger FM, Weiss SW. Soft Tissue Tumours. St. Louis: C.V. Mosby Company; 1983. pp. 125-35, 170-98.

6. Ferlito A, Nicolai P, Recher G, Name S. Primary laryngeal malignant fibrous histiocytoma: review of the literature. Laryngoscope 1983;93:1351-8.

7. Godoy J, Jacobs JR, Crissman J. Malignant fibrous histiocytoma of the larynx. J Surg Oncol 1986;31:62-5.

8. Kauffman SL, Stout AP. Histiocytic tumors (fibromas, xanthoma and histiocytoma) in children. Cancer 1961;14:469-82.

9. Keenan J, Snyder G, Toomey J. Malignant fibrous histiocytoma of the larynx. Otolaryngol Head Neck Surg 1979;87:599-603



 INVERTED PAPILLOMA OF NOSE &     PARANASAL   SINUSES:

                                   


 Dr.K.K.Desarda.  Prof. & Head otolaryngology KEM Hospital Pune.

                                                           
Abstract:



 Inverted papilloma (Schneiderian papilloma) is a primarily benign lesion that occurs in the nasal cavity and paranasal sinuses. Clinical problems include a tendency towards local destruction,recurrence and malignant transformation into squamous cell carcinoma. Hence, complete surgical removal is the therapy of choice and a meticulous follow-up is mandatory. The different histological types of nasal papilloma, their pathogenesis and the clinical and histopathological diagnosis., staging systems, therapeutic approaches, and surgical concepts are discussed.

The medical management is limited. Historically, radiotherapy was used in the management of inverted papillomas. Inverted papillomas have not shown to be radiosensitive. With radiotherapy in recent times, it has been used in patients with synchronous squamous cell carcinoma. Currently, medical management is used as an adjunct to specific complications, such as sinusitis. Surgical management is the mainstay of treatment of inverted papilloma. Selection of the surgical procedure is based on its extent, location, and the presence of concurrent malignancy.



Introduction:


Inverted Papilloma is a benign neoplasm originating from the Schneiderian membrane of the nose and paranasal sinus cavities. It has varied clinical and histological features involving the lateral nasal wall, septum, ethmoids, maxillary and sphenoid sinuses and at times involves the skull base. Three  cses interesting cases are reported below because of varied presentation of inverted papilloma.
Since Inverted Papilloma is associated with chronic sinusitis, patients always had nasal and sinus surgery. Most authorities will consider this a true neoplasm because of its transformation into carcinoma as transitional cell, papilloma or squamous papillary epithelioma. Early clinical diagnosis and thorough evaluation by biochemical tests, high resolution coronal CT scanning MRI studies and radical surgery is advocated. For its recurrence and malignant transformation few authorities advocate deep X–ray therapy following radical surgery. Fifty six  cases of proved inverted papilloma were treated  by different surgical procedures during 1980 to 2000 at KEM Hospital  Pune .
The advantages and disadvantages of various techniques  are discussed. No single technique gives better result so combinations were tried for better results.
The etiology of inverted papilloma is still unknown, but a number of nonspecific causes, as seen in the slide to the right, have been implicated. It is most often considered a true epithelial neoplasm as its intense proliferation of epithelium is its dominant histologic feature. Finally, a viral etiology has also been suggested. The support for viral etiology includes its multifocal origin and its high rate of recurrence, as well as the fact that it has been found to be responsible for other papillomas in other areas of the body. Human papilloma virus is most often associated with inverted papilloma. HPV DNA has been identified in 32 percent of inverted papillomas by in situ heparinization and PCR. Among inverted papillomas associated with carcinomas, HPV is present in 58 percent. Type 16 is the most prevalent in inverted papillomas,
 Because of its varied presentation inverting papillomas can be difficult to distinguish from other nasal tumors and they tend to recur after limited operation and also tend to transform into carcinoma. Hence, it is impossible to predict which inverting papillomas will become malignant.

.Sinonasal papillomas are characterized by being in general unilateral, although bilateral papillomas occur infrequently. Second, they have a destructive capacity with an ability to extend on into adjacent areas by spreading along a mucosa. Third, they have a tendency to recur, and they will recur even if completely excised. Finally, they have the potential for malignant degeneration.The clinical appearance of the nasal mass resembles an allergic polyp looking like a gray and red nasal mass.

The incidence of associated malignancy has been estimated to be approximately 10 % to 15%  Lawson and Allen in 2003 reported that 7 percent of patients have associated malignancy with synchronous carcinoma and 4 with metachronous carcinoma. These develop three to eight years after initial diagnosis. In their literature review of 26 series published between 1970 and 2001, consisting of over 1400 patients, 8.9% were found to have associated malignancy, 67% with synchronous, and 32% with metachronous











Grading of Inverted Papilloma



Grade I  Lesions involving nasal cavity only.
Grade II  Lesions involving nasal cavity + Paranasal sinuses.
Grade III
 Lesions involving  nasal cavity + Paranasal sinuses +Intracranial  extension
.




Management:

 Key to diagnosis is a detailed history, of course. Patients may have a history of unilateral symptoms or history of multiple surgical procedures for nasal polyps. Second, a thorough physical exam where a unilateral mass may be seen or endoscopic exam may reveal multiple polypoid masses with multiple digitations located laterally to the middle turbinate. Three, biopsy is key. Given the similar appearance and possible presence of concurrent polyps, histologic examination is critical. Multiple biopsies may be necessary, as seen in our case, due to inadequate sampling or sampling of concurrent polyp or inflammatory tissue or error in diagnosis.
Finally, radiographic evaluation is critical. CT scan is considered to be the study of choice. The most common CT profile is a unilateral mass with a lobulant surface occupying the middle meatus and extending into one or more of the adjacent sinuses. Opacification, mucosal thickening, of the paranasal sinuses may be seen, as well as bony thinning remodeling or erosion caused by inverted papilloma growth. Disadvantages of the CT scan are primarily due to its difficulty to differentiate inspissated mucus, polyps, or mucoperiostial thickening from inverted papilloma. MRI may also be used in the diagnosis of inverted papilloma. It is superior to CT scan for distinguishing papillomas from underlying inflammation and provides better delineation of lesions in contrast to surrounding soft tissue.
The medical manageent is limited. Historically, radiotherapy was used in the management of inverted papillomas. Inverted papillomas have not shown to be radiosensitive. With radiotherapy in recent times, it has been used in patients with synchronous squamous cell carcinoma. Currently, medical management is used as an adjunct to specific complications, such as sinusitis. Surgical management is the mainstay of treatment of inverted papilloma. Selection of the surgical procedure is based on its extent, location, and the presence of concurrent malignancy.
Three procedures that have been used to treat inverted papillomas are lateral rhinotomy and medial maxillectomy. Currently, the gold standard for the treatment of inverted papilloma is midfacial degloving and endoscopic sinus surgery. Lateral rhinotomy medial maxillectomy is particularly useful for inverted papillomas that are perilacrimal, nasofrontal, supraorbital, ethmoidal, or in the orbit. A curvilinear incision beginning just below the medial aspect of the eyebrow is made inferiorly half-way between the medial canthus and the nasion. The incision is extended inferiorly along the lateral aspect of the nose around the ala. The incision includes a full thickness skin down to periosteum. The periosteum is then elevated as far lateral as the lateral aspect of the maxillary antrum, as far superior as the orbital rim exposing and preserving the infraorbital nerve, and along the nasal bone in the ascending process of the maxilla. The periorbit is then undermined off the lamina papyracea, dislocating the lacrimal sac out of the lacrimal fossa and transacting the lacrimal duct as far distal as possible. The periorbit is then further undermined off the medial floor of the orbit. The anterior and posterior ethmoid artery is identified. These are the most constant landmarks for the frontoethmoid suture line. Staying below the suture line is critical to avoid entrance into the anterior cranial fossa. An opening is made through the anterior wall of the maxillary antrum, and the entire front wall of the maxillary antrum is removed up to the orbital rim. Then osteotomies are made, first along the floor of the nose through the bone between the antrum and the nasal cavity; second, through the frontoethmoid suture line below the level of the anterior ethmoid artery; and finally, along the medial floor of the orbit to the posterior wall of the antrum. The lateral nasal wall is then removed by cutting through the middle inferior turbinate attachments and then all mucosa is removed from the maxillary antrum. Then the sphenoid sinus is opened and its mucosa also removed. Dacryocystorhinostomy is then performed to avoid epiphora, a common postoperative complication of this procedure. It can be accomplished in two ways—either by catheterization of the lacrimal duct using an indwelling silicone or incising the lacrimal sac along it long axis and then suturing the edges in place to adjacent tissues. The main advantages of this approach include a radical excision, access to all lateral sinuses, the skull base, nasopharynx, and orbit. Disadvantages include, obviously, a surgical scar, possible CSF leak, epiphora, injury to orbit, and mucocele formation.
The contraindications to a purely endoscopic resection of inverted papillomas include the concomitant presence of squamous cell carcinoma, massive skull base erosion, intradural or intraorbital extension, and extensive involvement of the frontal sinus.
Midfacial degloving is particularly useful for inverted papillomas that are bilateral nasal in origin. Four types of incisions are required in this approach. The first is bilateral intercartilaginous incisions, bilateral septocolumellar incisions, a complete transfixion incision, and then a gingivobuccal incision. This is made from one maxillary tubercule to another. The soft tissue is then incised around the piriform aperture and nasal floor is undermined as high as the orbital rims. These incisions facilitate the exposure of a piriform aperture in the lateral nasal wall. A medial maxillectomy is then performed . and this can be combined with the frontal sinus osteoplastic flap for access to the frontal sinus. Its main advantages are that there is no external scar, invisibility, and bilateral access. Its disadvantages include insufficient access to more distant areas, such as vestibular, orbital, ethmoid cells; and complications include vestibular stenosis, oral antral fistula, epistaxis, and nasal congestion.
Endoscopic sinus surgery is particularly useful for inverted papillomas in the lateral nasal and nasal cavity, middle meatus, maxillary sinus, and the anterior and posterior ethmoid cells. The procedure largely consists of tumor debulking through a microdebridder until the origin is identified, and then lesions are usually excised en bloc from the identified tumor attachment sites with a wide cup of normal mucosa. Frozen sections are obtained, negative margins confirmed, and bone may be removed from underlying sites of attachment Although traditionally endoscopic surgery is used more for small lesions, bulky lesions have made endoscopic surgery difficult.  However, a new technique has been described for treatment of massive tumors with attachments within the maxillary sinus. It is called SSES (Sequential Segmental Endoscopic sinus Surgery). Basically, this involves sequential excision of larger tumors into massive segments, four segments usually. First, the nasal cavity; second, the middle meatus, including portions of the ostiomeatal complex; third, the maxillary sinus ostium and antrum along with the maxillary sinus medial wall if an endoscopic medial maxillectomy is performed; and fourth the frontal or sphenoid sinus .Advancements in diagnosis and treatment of sinus disease have led to successful use of endoscopic techniques in the treatment of inverted papilloma .

Case presentations:

Case No. 1
Mr. D. K. aged 68 had complaints of left nasal obstruction, headache, epiphora and bleeding from nostrils (epistaxis). He had nasal polypectomy and intranasal antrostomy done seven years ago. An ENT examination revealed a large greyish mass occupying the left nasal cavity pushing the septum to right side. A probe could not be passed around the nasal mass since it was coming from the lateral nasal wall. The nasal vault was tender and there was a evidence of nasolacrimal duct obstruction. Left maxilla was tender and mass did not bleed on touch. A provisional diagnosis of recurrence of nasal polyposis was made with the view of malignancy in mind.

The routine bio–chemical tests were within normal limits, X–ray paranasal sinuses revealed opacity and left maxillary sinuses with medial wall destruction. A CT scan also revealed a lesion of maxillary sinus with erosion of medial wall and a soft tissue mass in the left nasal cavity extending to the nasopharynx.
In view of the recurrence and erosion of the medial wall, the patient was subjected to lateral rhinotomy with medial maxillectomy procedure. The mass was removed with maxillary clearance. Histopathology confirmed the diagnosis of inverted papilloma with no evidence of malignancy. Post operative recovery was uneventful. The patient was reviewed in a follow up clinic and had no recurrence for the last one and a half year.

Case No. 2
Mrs. M. L. aged 48 had a bilateral nasal obstruction, mouth breathing and headache. She was non–diabetic, non–hypertensive and had nasal polypectomy two years ago. A routine clinical examination revealed bilateral polypoidal masses occupying both the nostrils. The color of the mass was grayish and firm in consistency and did not bleed on touch. Both maxillary sinuses were tender. Postnasal space did not show any soft tissue mass..
Clinical diagnosis of bilateral nasal polyposis was made (Recurrence) with associated maxillary sinusitis and no extension to nasopharynx. CT PNS revealed extensive opacity of maxillary and ,ethmoid sinuses without any erosion of orbital cavity. Endoscopic  sinus surgery  was planned under GA .  Post operative recovery was uneventful. Histopathology of the biopsy revealed inverted papilloma. A review after 18 months was satisfactory without recurrence.
Case No. 3
Mr. A. K. aged 28 came to the ENT Clinic with the chief complaints of nasal obstruction, bleeding from left nostril, headache, and proptosis of left eye for over six months. A clinical diagnosis of the left nasal polyposis was made after a thorough clinical examination. A routine hemogram was performed and this including biochemical tests were within normal limits. A CT scan revealed a lesion involving the entire left ethmoid, nasal cavity, maxillary sinus, nasopharynx and anterior wall of sphenoid sinus.A planned endoscopic ethmoidectomy and transantral maxillary clearance was done. The post operative period was uneventful. and  the patient’s left proptosis decreased . Histopathology report revealed inverted papilloma. Post–operative review after six months was quite satisfactory and without recurrence.
Discussion:
The search for an ideal surgical approach to removing inverted papillomas has been fairly controversial, with proponents of radical surgery vying with those who support endoscopic endonasal procedures. The external approaches include medial maxillectomy, frontoethmoidectomy, mid facial degloving and Caldwell-Luc surgeries. Recurrence rates following these procedures have been reported to range from 4 to 35%.  The short-term complications of these approaches include epiphora, dacryocystitis, diplopia, transient blepharitis, lid edema, and cerebrospinal fluid leak. Late complications include persistent crusting, pain, nasocutaneous fistula, frontal sinus mucocele, vestibular stenosis, unacceptable scarring, and nasal collapse

Inverted papillomas are relatively uncommon tumors of the nasal cavity comprising approximately 0.5% to 4% of all primary nasal tumors. Its incidence ranges from 0.75 to 1.5 cases per 100,000 per year. They occur approximately 15 th as often as inflammatory polyps. Age ranges widely from 6 to 89 years, and most are usually diagnosed in the 5 th to 7 th decade. Average age of diagnosis is 53 years. There is a male predominance 3:1, and it affects primarily Caucasians.
Sinonasal papillomas have been categorized in to three distinct subtypes based on histologic appearance. Inverted papillomas (70%),cylindrical cell papilloma (19%), and fungiform  papillomas  (11%),although all these are histologically benign in nature but inverted and cylindrical  papillomas  may be associated with malignancy which ranges from 4% to 17 % for inverted papillomas and 9% to 13% for cylindrical papillomas..
Symptoms are nonspecific often mimicking sinusitis. The most common is unilateral nasal obstruction seen in over 60% of patients. Nasal discharge, headache, facial pressure and pain, epistaxis, and anosmia may also be seen. Signs may include a polypoidal mass filling the nasal cavity extending from the vestibule to the nasopharynx. The nasal septum is often bowed to the contralateral side due to slow expansile growth. Proptosis and facial swelling  is also seen in these patient  at late stages.
Sinonasal papillomas are characterized by being in general unilateral, although bilateral papillomas occur infrequently. Second, they have a destructive capacity with an ability to extend on into adjacent areas by spreading along a mucosa. Third, they have a tendency to recur, and they will recur even if completely excised. Finally, they have the potential for malignant degeneration Although most of the lesions arise from the lateral wall, middle meatus and ethmoid complex, they may sometimes arise from the septum and underlying perichondrium, the cartilage, lateral nasopharyngeal wall, maxillary sinus, sphenoid sinus and may involve the base skull. The distribution is: lateral wall 68%, ethmoid complex 57%, septum 28% intracranium 4%. The highest recurrence rate  is 70%.
 Advantages and Disadvantages of endoscopic surgery.
It is a less invasive procedure and you have a multiangle visualization and absence of facial scarring. In terms of disadvantages - orbit injury, CSF leak, and periorbital ecchymosis. It is especially difficult for the larger tumors that fill the entire nasal cavity. This is increasingly circumvented by the ability to first debulk the tumor and then perform excision in segments. The main controversy in the treatment of inverted papilloma lies in the final advantage and disadvantage: seemingly contradictions, comparable versus greater recurrence rate. This inherent contradiction is reflected in the debate that still exists regarding whether less extensive procedures result in incomplete excision of the inverted papilloma and, thus, more recurrence



Acknowledgement

I am thankful to  Dr. B.J. Coyaji Chief Medical officer,KEM Hospital Pune for permitting me to publish this paper.I extend sincere thanks to  Nursing staff and staff ENT Dept for their kind co-operation in  the preparation of this manuscript.


Bibillography.
Frank, C. A., Oliver D. J. and Jack, G (1985): Usual anatomic presentation of inverted papilloma – Head and neck Surgery, 243:45.
Feinmessar, R., Goy. I., Weessel, J. M. and Ben–Bessat H. (1985): Malignant Transformation of inverted Papilloma. Ann Otol 94:39–43.
William, M. Menden, H., Rodney, R., Million, N. J. and Cassissi Kenal, P. K. (1985): Biological aggressive Papilloma of Nasal Cavity – Role of radiation therapy. Laryngoscope, 95:344–347.
Thomas, C. and Calceterra, W. T. (1980): Varied presentation of inverted Papilloma, 90:53–60.

 Alba JR, Diaz MAA, Perez A, Rausell N, Basterra J. Inverted papilloma of the sphenoid sinus. Acta Oto-Rhino-Laryngologic Belg 2002:56:399-402.
Baruah P, Deka RC. Endoscopic management of inverted papillomas of the nose and paranasal sinuses. ENT-Ear Nose Throat J 2003;82:317-320.
Benninger MS, Roberts JK, Sebek BA, Levine HL, Tucker HM, Lavertu P. Inverted papillomas and associated squamous cell carcinomas. Otolaryngol Head Neck Surg 1990;103:457-461.
Buchwald C, Lindeberg H, Pedersen BL, Franzmann M-B. Human papilloma virus and p53 expression in carcinomas associated with sinonasal papillomas: A Dutch epidemiological study 1980-1998. Laryngoscope 2001;111:1104-1110.
Bull TR. Color Atlas of ENT Diagnosis. New York: Thieme.
Calcaterra TC, Thompson JW, Paglia DE. Inverting papillomas of the nose and paranasal sinuses. Laryngoscope 1980;90:53-60
Cummings BW, Goodman ML. Inverted papillomas of the nose and paranasal sinus. Arch Otolarygol 1970;92:445-449.
Eisen MD, Buchmann L, Litman RS, Kennedy DW. Inverted papilloma of the sphenoid sinus presenting with auditory symptoms: A report of two cases. Laryngoscope 2002;112:1197-1200.
Gulya AJ, Wilson WR. An Atlas of Ear, Nose and Throat Disorders. New York: Parthenon Publishing Company.

Contributed by Dr. K. K. Desarda,





Cryosurgery Treatment for Hemangioma of Tongue:
A Case Report

Dr.K.K. Desarda .Dr. Nilima,Dr.Sheetal.


ABSTRACT

Hemangiomas are frequently seen benign tumors which based on vascular tissues. These lesions are mainly identified in to two groups which are namedas capillary and cavernous hemangiomas due to vascularization of the lesions.Capillary hemangiomas consist of small capillary vessels which show lobulesformation. Cavernous hemangiomas consist of large dilated vessels and theycan reach to large sizes. Several treatment modalities including sclerotherapy,embolisation, laser surgery and cryosurgery have been described forhemangiomas. In this significant case, the cryosurgery treatment of a 32 yearsold female patient who was suffering from the huge hemangioma in the rightside of her tongue is presented with complete regression of haemangioma without any tongue deformity or atrophy.or post op morbidity.


KEYWORDS


Hemangioma, Cryosurgery

 Contact Author

Dr. K.K.Desarda-Prof.Emeritus-& Head ORL KEM Hospital, Pune.

E-mail : kdesarda@gmail.com


INTRODUCTION


The author using cryoapplicator of his own modification presents cryosurgery as a successful surgical technique in cases of haemangiomas involving , tongue and oral cavity. From 1980 to 2012 in the ENT-Clinic ,KEM Hospital,Pune. Over 60 patients were operated upon using this method. Most of the cases were treated under local anaesthesia. The results were very encouraging. Total regression of haemangiomas and regeneration of normal mucosa with no noticeable scar resulted. A method of cryosurgery especially suitable for very young patients or for those who are poor risks because of associated disease and advanced age is suggested.

Hemangiomas are benign tumors ofvascular tissue which are most likely to be seen at the head and neck region. They are the most common tumors of the childhood. They
show higher prevalence in women. Most of these lesions are described hamartomas instead of tumors. Vast majority of hemangiomas are known to be regressive(1).Hemangiomas are classified as capillary and cavernouson the basis of the vascularization system. (1). Capillary hemangiomas consist of small capillary bodies that organize lobularly. Cavernous hemangiomas consis of wide and dilated vessels and can reach to large size..Hemangiomas can be encountered intraorally; on,tongue, anterior gingival and buccal mucosa. Especially the lesions on the cheeks and tongue can be traumatized by chewing and bleeding can occur.  (1-4).It is known that deep hemangiomas could be seen as blue-purple lesions during intraoral examination. For the diagnosis of cavernous hemangiomas, bidigital palpation of the region and detection of disappearance of the blood due to finger pressure and after removal of finger pressure observation of revascularization are important signs. Furthermore, if the lesion has an arterial origin, pulse can be obtained by finger pressure(1,2).

Although most hemangiomas of the tongue are asymptomatic, they could sometimes cause significant bleeding, pain or difficulty in chewing, speaking, and  even swallowing, if they are large enough. Small lesions can be excised with impunity.  Large lesions, if excised by surgery could result in significant functional  disability.  This is why several modalities of less invasive treatment have recently been advocated (Argon laser, Nd:YAG laser,radiofrequency,scelotherapy, cryosurgery and superselective embolization to avoid functional disability caused by tissue loss).

Age and general condition of the patient has great importance as well as the size and characteristics ofthe lesion in the treatment of hemangiomas. Small lesions can be surgically excised while larger ones require specific surgical interventions. These treatment methods are sclerotherapy, embolisation, laser surgery, radiofrequency and cryosurgery (1-5)
.
Amongest all modalities Cryosurgery is a very effective method for thetreatment of intraoral cavernous hemangiomas. It can be applied under basic local anesthesia. Freezing should be repeated twice for each area for three to five minutes to ensure adequate effect. The ice ball should extend a little beyond the limits of the lesion so that about three-quarter of the diameter of the observed circumference of the frozen tissue will be at a cell lethal temperature of – 15° C of normal tissue. Sloughing of some of the central tissue usually takes places at about one week post operatively and healing may be expected with 4-6 weeks.


CASE REPORT


A 32  year old female patient presented to ENT dept KEM Hospital Pune who was suffering from progressive huge swelling  on her right lateral border of tongue,pain while swallowing,distorted speech and at time occasional bleeding for over six months.She was treated conservatively by the medical practioner,She was also seen by general surgeon who advised surgery (hemiglossectomy) which patient refused.



Clinical examination :

Revealed wide, bluish purple lesion was detected on the right lateral side of the patients tongue (Figure 1). The signs of revascularization after finger pressure was determined and no pulsations were obtained from the lesion and the lesion was diagnosed with hemangioma. Rest of the ENT examination was WNL
.A clinical diagnosis of tongue haemangioma was confirmed  and patient advised for crysurgery treatment,which she has accepted since it was less invasive procedure.Routine lab study and general fitness was taken and patient was posted for cryosurgical intervention .
...
Under local anaesthesia 10cc (xylocaine + Adr.1:200000) infiltration all around the haemangiomatous mass was done with 3mm normal tongue margin. Liquid nitrogen via large contact cryo tip was applied to the entire lesion for 3 to 5 minutes .including 3mm peripheral normal tissue margin (Fig 1 & 2) Following this process, the area was washed with NaCl 0.9% and the patient was prescribed with antibiotics, analgesic, anti-inflammatory agents and tantum mouth wash.. Necrotic and sloughing area were observed in the postoperative first week (Figure 3 & 4 )and within four to six weeks  the lesion was successfully  and completely healed (Figure 5 & 6)



Pre operative & post operative results of cryosurgery shown in these pictures.







 
Fig.1.Pre op Tongue Haemangioma     Fig.2. cryo-freezing.of Haemangioma.
                                                           
                                                                   




 

 Fig.3.   Intra op cryosurgery lesion           Fig.4.    Intra op cryosurgery lesion
 
Fig.5   Post cryosurgery result 4 wks     Fig.   Post cryosurgery result 6 wks


.
DISCUSSION

The diagnosis and the classification of the vascularmalformations have a great importance on the treatment plan of the lesions. Mulliken and Glowackiproposed a terminology for classifying these lesionsthat is based on clinical and microscopic features(6) .This system broadly classifies vascular lesions into hemangiomas and vascular malformations. The hemangioma is the true vascular tumor that results from a neoplastic overgrowth of normal vascular tissue.The hemangioma grows by endothelial proliferation.In distinction to hemangiomas, vascular malformation results from abnormal vascular or lymphatic vesselmorphogenesis, not as the result of abnormal endothelial growth. Hemangiomas are usually present at birth and can be diagnosed by 1 year, where asvascular malformations are present at birth but often not diagnosed until second decade of life.Hemangiomas show rapid growth until 6-8 monthsand involute by 5-9 year
 Vascular malformations show slow growth throughout life with increase in response to infection, trauma, or hormonal fluctuation and they do not involute. Osseous involvement of the hemangiomas is rare but 35% of the vascular malformations show osseous involvement .A wide variety of methods are utilized for the treatmentof intraoral hemangiomas.
 Embolisation technique which is one of them has been utilized since early nineties. However, embolisation technique has two major disadvantages. One of these disadvantages is the risk of embolisation material reaching cranial cavity via external and internal carotid arteries and the need for an experienced radiologist in order to perform this procedure. The other disadvantage is the temporary blockage of flow . It would be proper to utilize this method prior to the surgical excision of large hemangiomas in order to reduce to risk of bleeding

Another method for the treatment of hemangiomas is sclerotherapy. In this method, a sclerotic agent is injected into or peripheral to the vein that the hemangioma originates from. This method is successfully utilized in the treatment of extra oral lesions. However, pressured bandage can not be applied to the region after the injection of sclerotic agent inintraoral lesions. Thus, sclerotherapy is recommended to be applied together with other treatment methods Subzero temperatures can cause so-called ‘frosbite’whilst temperatures above the coagulation point of proteins results in ‘burns’. Thermal surgery employs these effects in carefully controlled manner: the use of temperatures below freezing point constituting cryosurgery while heating effects are obtained by theuse of lasers normally functioning in the infrared range of wavelengths.
The results of the cryosurgery can be explained as acold-induced coagulative necrosis.

 Cryodestruction of a normal or benign neoplastic tissue normally requiresthe attainment of a temperature of at least -15°C (thetemperature at which intracellular ice forms) while total ablation of malignant tumor tissue calls for somedegree of overkill at level of -50°C .For the management of oral benign, premalignant and malignant lesions liquid nitrogen is used as a freezing agent and delivered by either probes or sprays  Inthis case, probes are preferred in order to have limited effect on the lesion.In cryosurgery tissue regeneration is remarkably betterthan the other surgical techniques. When the body spontaneously separates the coagulated slough there is a powerful stimulus to cellular division, hyperplasia and apparent hypertrophy; this may be associated with concomitant cytokine release .Laser surgery is another effective method used for the treatment of intraoral hemangiomas  But, it has several disadvantages when compared with cryosurgery. Laser surgery is a much more complex process compared with cryosurgery


Laser surgery is a much more complex process compared to cryosurgery and requires general anesthesia.(5) Nerve damage is less in cryosurgery and regeneration is quicker.Postoperative scar formation is less in cryosurgery.Laser surgery application can be hazardous around salivary gland ducts, which should be taken into consideration. Laser surgery costs much more expensive compared to cryosurgery. However in laser surgery, postoperative edema is less and the procedure does not require to be repeated. Additionally, laser surgery is a faster and more dramatic technique



CONCLUSION

A variety of methods of treatment are thus available for intraoral hemangiomas. The majority of theselesions can be regarded as capillary-cavernous hemangiomas. In this case, cryosurgery was preferred for the treatment of the cavernous hemangioma since it has multiple advantages like being an easy, cheap,successful method which can be done under local anesthesia and can be repeated if required for residual lesion.


REFERENCES

1. Langdon JD, Patel MF: OperativeMaxillofacial Surgery, pp393-396. London
Chapman&Hall Medical, 1998.
2. Tal H. Cryosurgical treatment of hemangiomas of the lip. Oral Surg Oral
Med Oral Pathol 1992;73:650-54.
3. Hartmann PK, Verne D, Davis RG.Cryosurgical removal of a large oral
hemangioma. Oral Surg 1984;58:280-82.
4. Reischle S, Schuller-PetrovicS.Treatment of capillary hemangiomas of
early childhood with a new method ofDept. of Oral and Maxillofacial Surgery
Faculty of Dentistry, Ankara UniversityAnkara, Turkey.
5.Mulliken JB, Glowacki J. Hemangiomasand vascular malformations in infants andchildren: A classification based on
endothelial characteri





BERA STUDY IN 150 CHILDREN UNDER 5 YRS AGE

Dr. K. K. Desarda
Professor Emeritus & Head, Otolaryngology.KEM Hospital Pune.
&
Dr. A. N. Sangekar
Audiologist and Speech Pathologist,
KEM Hospital, Pune


BERA (BSER OR BAEP)

Abstract

OBJECTIVES: The brainstem evoked response audiometry (BERA) is an objective neurophysiological method for the evaluation of the hearing threshold and diagnosing retrocochlear lesions. The aim of the study was to investigate the hearing level in children with suspected hearing loss or pathological speech development. PATIENTS AND METHODS: The BERA diagnostic procedure was applied in 150 children ranging from 1 to 5years of age at KEM Hospital Pune.. RESULTS: We found profound hearing loss (deafness) in 15 children, severe hearing loss in 10 children, moderate hearing loss in 35 children, mild hearing loss in 30 children, and normal hearing level in 60 children. Out of the children suspected for hearing loss, 42% actually had some level (mild-moderate) of hearing loss. Out of the children with delayed speech, 63% had some level (mild-profound) of hearing loss which actually caused the delay in speech development; 37% had normal hearing, but inadequate verbal communication affected their language acquisition and speech development. CONCLUSIONS: These results illustrate the necessity to test children hearing even with the slightest suspicion by the parent or doctor of hearing loss.
INTRODUCTION
BERA has  proved to be a useful tool in diagnosing hearing impairments in children which could be conductive or sensorineural in nature. Thanks to early detection, rehabilitative procedures could be started early which will help speech and language development.

We have studied 150 cases below five years of age, and our observations are recorded with case history profiles like high risks, referral for adaptions, congenital malformations and delayed speech. We feel BERA is the only tool which can present an accurate picture of hearing sensitivity.

BERA (Brainstem evoked response audiometry), ABR (Auditory brain stem response), BAER (Brainstem auditory evoked response audiometry).

BERA is an electro-physiological test procedure which studies the electrical potential generated at the various levels of the auditory system starting from cochlea to cortex. This investigation was first described by Jewett and Williston in 1971.
Procedure: The stimulus either in the form of click or tone pips is transmitted to the ear via a transducer placed in the insert ear phone or head phone. The waves of impulses generated at the level of brain stem are recorded by the placement of electrodes over the scalp.

Electrode placement: Since the electrodes should be placed over the head, the hair must be oil free. The patient should be instructed to have shampoo bath before coming for investigation. The standard electrode configuration for BERA involves placing a non inverting electrode over the vertex of the head, and inverting electrodes placed over the ear lobe or mastoid prominence. One more earthing electrode is placed over the forehead. This earthing electrode is important for proper functioning of preamplifier.
BERA is resistant to the effects of sleep, sedation, sleep and anesthesia. Its threshold has been found to be within 10dB as elicited by conventional audiometry.

The waves detected in BERA tests
1. Auditory nerve
2. Cochlear nucleus
3. Superior olivary complex
4. Lateral lemniscus
5. Inferior colliculus
6 and 7. Medical geniculate body

.
Hearing problems are common among the children which could be conductive or sensorineural in nature. Early diagnosis of hearing impairment is important as the rehabilitative procedure can be started early which will help speech and language development.

Various audiological test procedures are used to assess the hearing sensitivity of children. Some of the common ones are: Behavioral Observation.
Free Field Audiometry.
Peep Show.
Pediatric Tester.
All the above techniques are useful in estimating hearing sensitivity but have their own limitations. The major difficulties involved in the use of these techniques are:
Co–operation of children.
Consistency of responses.
Subjectivity on the part of the tester.
Assessment of children with multiple handicaps.
BERA, (BSER or BAEP) has proved to be a useful objective tool in diagnosing hearing impairments in children. The use of Auditory Brainstem Response (ABR) (1) audiometry as an audiological tool focused on two principal areas:
The assessment of hearing sensitivity and slope of loss, (if any) in patients, who are unable or unwilling to participate in standard psychoacoustic test procedures.
To study the neurological integrity of acoustic nerve and brainstem pathway.
To achieve this objective, the paradigm employed is to collect appropriate ABR data for a case, analyze it and compare the data with relevant norms and draw inferences.

Material and Methods

In this study, an attempt is made to study the findings of BERA in children below the age of five years. These children reported to us or were referred to us for the following reasons:
Delay in speech and language development.
Inconsistent responses to sound or inability to respond to sound.
History of high risk factors – Deafness in the family, consanguineous marriage, difficult/obstructed labor, pre–term/premature labor, administration of antibiotic drugs during pregnancy, diseases contracted by the mother such as Rubella, Meningitis or Hyperbilirubinemia.
To rule out hearing impairment before adoption.
To rule out the extent of malformation anomalies especially in Atresia.

Test Procedure

All patients were administered the test procedures with prior appointment. An ENT check up was done to rule out the possibility of wax, ear infection, middle ear problems etc. The parents were instructed to wash the scalp of the child thoroughly as a requirement of the test. Prior to the test, each child was examined by the pediatrician and the dosage for sedation was prescribed. Drugs used for sedation were Trichloryl and Phenergan in combination.

Test was carried out in pre–cooled, quiet (not treated) room. The instrument used was Nicolet EP Four Compact which is a fully computerized machine with the facility of artifact rejection. The skin was cleaned with spirit and OMEN abrasive skin preparatory paste. The silver electrode were placed as follows: Cz–vertex, A–1 LF mastoid, A–2 values was not more than 1ohms. Electrode electrolyte gel was used and electrodes were fixed. Acoustically shielded THD 32 ear phones were placed on the ear and head bands were adjusted. The clicks of 11.4/sec duration were used as stimulus. The filter settings used were a 50Hz–300Hz. The polarity used was alternate and the analysis time was 10m/sec. About 4,000 responses were averaged. First, stimulus was given at 105 dBnHL level (i.e. maximum intensity level available). If peak V was detected at a particular level, intensity was increased by 5dB. The existence of peak V was considered as sound stimulus heard and perceived by the auditory mechanism. The threshold for each ear was confirmed. The guidelines used for the confirmation of peak V were as follows:
1.Peak V occurs around latency of 5.7 m/sec with S.D. of 0.25 (as per our norms).
     2.With decrease, an intensity level latency of peak V increases and its amplitude decreases.
     3.Reproduction of peak in re–run.
     4.Peculiar in shape.
     5.Use of a neutral run.
Since the measurement of hearing sensitivity in children under five years of age was the only aim of this study, the latency values and interpeak intervals even though measured, are not considered. Each child's hearing sensitivity was assessed, and they were sub–grouped in the following categories.
Normal hearing sensitivity. Hg. thresholds up to 25dB level and below.
Mild hearing impairment. Hg. thresholds between 30dB to 45dB.
Moderate hearing impairment Hg. thresholds between 50dB to 65dB.
Severe hearing impairment. Thresholds between 70dB to 85dB.
Profound hearing impairment. Thresholds above 90dB.

Observation
We have studies BERA findings of 150 children (below the age of five yrs) for this study.

Given below is the age–wise distribution of these children:

AGE No.
0–1 52
1–2 52
2–4 24
4–5 22

The case history profile of these cases is as follows:

High risk clinics. 82 cases
Referred for adoption. 31 cases
Patients with ear anamolies (congenital). 3 cases
Patients brought by atresia. 34 cases
Patients with complaint of delayed speech or inability to respond to sound.

The BERA findings of 15 cases studied:
Normal hearing sensitivity. 53
Mild hearing impairment. 12
Moderate hearing impairment. 21
Severe hearing impairment. 30
Profound hearing impairment. 34
Total 150

Out of 53 patients with normal hearing sensitivity, 33 were sent for adaption, 26 patients were found to be mentally subnormal and 5 patients had multiple anomalies. Out of 12 patients with mild hearing impairment nine patients had history of ear discharge and out of 21 patients with moderate hearing loss 10 had history of ear discharge either in one ear or in both ears and three patients had congenital anomalies of the ear and four had a history of high risk factors. Out of 30 patients with severe hearing impairment 22 had high risk factors contributing to their history and of 34 patients of profound hearing loss, 23 had high risk factors contributing to their history.

Out of 82 high risk cases, 22 had severe hearing loss, 23 had profound hearing loss, four had moderate hearing loss, 33 had normal hearing sensitivity. All the above cases were sent for further rehabilitative procedures as per their requirement.

Conclusion

BERA is a very useful in early detection of hearing loss and planning rehabilitative procedures. In case of multiple handicaps, BERA is the only test which can give accurate picture of hearing sensitivity. In cases of high risk babies, BERA should be carried out as a routine procedure to detect hearing loss. BERA test helps us to conclude regarding the cause of delay in speech and language development. BERA is the only tool which can confirm the normal sensitivity of hearing whenever required.

References
Chaturvedi V. N., Chaturvedi P. (1980): Assessment of hearing in small children. Indian Journal of Paediatrics. 27: 827–831.
Jerger J and Hall (1980): Effects of age and sex on Auditory Brainstem response. Archives of otolaryngology.
Jerger and Mauldin. (1978): Prediction of S N Hearing loss from BERA. Archives of otolaryngology.
awson S., Mc Cromic B., Wood S. (1995): BERA in children and normative study.
Kilney (1982): Auditory brainstem responses as indicators of hearing aid performance. Annals of otology, Rhinology and Laryngology pp 91.
Alberti P. W., Hyde M. L., Riko K., Corbin H., Abramovich S. (1984): Laryngoscope BERA in high risk neonates.
Contributed by Dr. K. K. Desarda(This paper was read in AOI conference.)