|Year : 2015 | Volume
| Issue : 1 | Page : 67-71
Bezold's fistula: An unusual presentation of cholesteatoma
Department of Otolaryngology and Head and Neck Surgery, King Hamad University Hospital, Busaiteen, Kingdom of Bahrain
|Date of Web Publication||10-Mar-2015|
Busaiteen Viresh Arora
Flat No 74, Building 2354, Street 2834, Zip Code 228 Muharaq, Busaiteen
Kingdom of Bahrain
Source of Support: None, Conflict of Interest: None
Cholesteatoma presenting with a Bezold's fistula is extremely rare, and such presentation has been reported. It is infrequent these days in this era of antibiotics, due to early institution of treatment. Fistula results in untreated and inadequately managed Bezold's abscess. The treatment of Bezold's abscess is with prompt intravenous antibiotics, followed by drainage of cervical abscess, but management that of Bezold's fistula is primarily surgical in which a mastoidectomy is done to eradicate the disease with exposure of the mastoid tip with laying open the fistulous tract. One such unusual case of a Bezold's fistula with cholesteatoma is reported in a teenager with a long-standing history of ear discharge, but without any other complications. The patient had being managed conservatively with ear drops since childhood. High resolution computed tomography (CT) mastoids revealed extensive cholesteatoma is destroying the mastoid and perforating the tip with erosion of the tegmen tympani with extra cranial extension. Patient underwent modified radical mastoidectomy with reconstruction of the mastoid bowl with bone pate. Patient was successfully treated and was followed to 1-year with CT scan without recurrence.
Keywords: Bezold′s abscess, Cholesteatoma, Fistula, High resolution computed tomography mastoids, Mastoidectomy, Mastoiditis
|How to cite this article:|
Arora V. Bezold's fistula: An unusual presentation of cholesteatoma. Indian J Otol 2015;21:67-71
| Introduction|| |
Cholesteatoma otherwise benign, is a locally destructive disease of the middle ear cleft with recurrence tendency, has been known to be associated with extracranial and intracranial complications. Mastoiditis and subperiosteal abscess are the commonest extracranial complications, which can extend to the surrounding major vessels in the neck causing life-threatening complications. 
Bezold's abscess typically develops as a sequel to suppurative complication of acute mastoiditis following acute otitis media (AOM), which leads to impeding drainage of the middle ear cleft due to entrapped inflamed mucosa of mastoid air cell system. Pus tracks from the eroded mastoid cortex medial to the attachments of sternocleidomastoid and the posterior belly of digastric muscles, through the digastric groove to the upper part of the neck to appear as a nonfluctuant swelling in the neck. Since it is located deep in the neck obscured by overlying muscles, it escapes detection. 
Chronic mastoiditis usually occurs in association with a long-standing cholesteatoma or as a complication to an infection following placement of a ventilating tube or when a long established uninfected central perforation becomes infected and extends to the mastoid. In the case of cholesteatoma, the debris may obstruct the infection emptying into the external auditory canal and leading to erosion through the mastoid tip resulting eventually in a fistula. 
Cholesteatoma may lead to subsequent bone destruction and other complications such as meningitis, brain abscess, labyrinthitis, and facial nerve paralysis.  Fistulous presentation in chronic mastoid disease with cholesteatoma is rarely seen, but when present can lead to sinister sequel if ignored. With the advent of antibiotics, otogenic complications have decreased but incomplete treatment has resulted in more insidious progression of the disease.
Here, a case report of a rare presentation is reported.
| Case Report|| |
An 18-year-old male presented to the ENT outpatient department with oozing bloody discharge on and off from the right neck wound, for past 8 months. History revealed foul smelling right ear discharge since childhood. There were no associated symptoms such as headache, seizures, fever, neck rigidity or otalgia. Patient was emanating stench, which was appreciable the time he entered the examination room.
On inspection, exuberant granulations were seen in the neck on right side along the anterior border of the upper third of the sternocleidomastoid muscle, which on probing lead to bleeding [Figure 1]. Pinna was normal in position with no visible postaural scar. There was no mastoid or spura metal tenderness. Right ear otoscopy revealed whitish cholesteatomatous debris in the posterior quadrant extending to the attic eroding the scutum, whereas the left ear was unremarkable. Tuning fork showed weber lateralized to the right side with Rinnes negative to the right side. Audiometry revealed 45 dB hearing loss in the right ear. Culture swab was taken from the right ear, and high resolution computed tomography (HRCT) scan of the temporal bones was requested, which revealed extensive destruction of the right mastoid with eroded mastoid tip [Figure 2] and [Figure 3]. Magnetic resonance imaging T1-weighted images showed cholesteatoma breaching the tegmen tympani with enhancement of the meninges, but without any intracranial extension [Figure 4].
|Figure 2: Preoperative computed tomography coronal view showing eroded mastoid tip|
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|Figure 3: Preoperative high resolution computed tomography axial section showing erosion of mastoid and tegmen tympani|
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|Figure 4: Magnetic resonance imaging T1-weighted without contrast showing intracranial extension of cholesteatoma after breaching tegmen tympani|
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Patient was worked up for general anesthesia and complete blood count, blood culture and coagulation profile was done. Right postural incision was given 1.5 cm behind the postural sulcus since exposure of the mastoid tip was required. The intraoperative findings revealed granulations and extensive cholesteatoma with erosion of the posterior canal wall. Mastoid was drilled from inside out and followed back toward the aditus antrum to reach the mastoid tip. Posterior metal wall was found eroded. Modified radical mastoidectomy (CNWD) was done, and cholesteatoma removed. The epithelium was tracked going into the mastoid tip, which was drilled to expose the fistulous track leading in the neck. The postaural incision was extended to the fistulous opening, and the track was excised. No ossicles except the footplate of stapes were present, and no reconstruction was attempted at this stage. A large cavity thus created after exteriorization and the sump formed was filled with the bone pate collected during the drilling procedure.
An adequate meatoplasty was done, and the middle ear was packed with gel foam and the cavity with antibiotic impregnated ribbon gauze for 10 days. Simultaneously the neck track was closed. Patient received 2 doses of Augmentin 1.2 g intravenous and was discharged on the 3 rd postoperative day. The cavity remained infected for the 1 st month, which necessitated cleaning twice weekly till the cavity was well epithelized. The patient was then followed monthly for first 3 months [Figure 5]. HRCT was repeated after 6 months, which showed well developed tip showing osteogenesis with no evidence of recurrence of remaining disease [Figure 6] and [Figure 7]. The patient remained under follow-up till 1-year, for persistence of complaints and recurrence of disease.
|Figure 6: Postoperative high resolution computed tomography coronal section showing osteogenesis of tip after bone pate obliteration|
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|Figure 7: Postoperative high resolution computed tomography axial section of mastoid|
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| Discussion|| |
Chronic otitis media (COM) is defined as persistent infection or inflammation of the middle ear and mastoid air cells.  In these cases with refractory Eustachian tube More Details dysfunction, the tympanic membrane is weakened by retraction process, increasing the likelihood of atelectasis and cholesteatoma formation.
The cholesteatomas are cystic lesions of the middle ear cleft lined by stratified squamous epithelium filled with keratin, with a tendency for bone erosion and recurrence. They are found to be located anywhere in the pneumatized temporal bone including the petrous apex. 
Cholesteatoma is histologically a benign disease, but its aggressive local behavior and invasive properties cause significant morbidity and mortality.  Once established in the middle ear, the gradual expansile keratin accumulation causes osseous erosion as well as adjacent structures, leading to facial nerve paralysis, labyrinthine fistula, as well as other complications. 
The proposed mechanisms of bony destruction, although remain controversial, are expanding mass effect with pressure necrosis, and osteoclastic action, causing enzymatic bony destructions.  Cholesteatomas can also erode the outer mastoid cortex by bony resorption, leading to the development of a cutaneous fistula.  Due to slow destructive and insidious behavior of cholesteatoma, it may cause, intracranial and extracranial complications, leading to severe sequele. Intracranial complications range from meningitis, extradural abscess, lateral sinus thrombosis and cerebellar and temporal abscess. ,
The extracranial complications though less serious than the intracranial, range from mastoid abscess, subperiosteal abscess, Bezold's abscess, petrositis and labyrinthine fistula. The infection can spread caudally from the mastoid tip to involve the great vessels tracking along the subclavian artery and reach the mediastinum leading to life-threatening emergencies.  The abscesses can also spread along the fascial planes, into spaces such as suprasternal, parapharyngeal, and even the contralateral neck. 
Prior to the antibiotic era, mastoiditis was a common complication of AOM and intracranial complications frequently resulted in death.  However, postantibiotic era has drastically changed the course of mastoiditis with reduction of complications.  The predilection of a middle ear infection to spread into adjacent spaces is influenced by various factors, including organisms virulence, its sensitivity to antibiotics, and host resistance. Immunocompromised patients are at risk to develop complications of otitis media. 
Subperiosteal abscess formed by lateral extension of suppuration is the commonest extra temporal complication, which results either when the infectious process within the mastoid air cells extends into the subperiosteal space from erosion of the cortex secondary to acute or coalescent mastoiditis, , or as a result of vascular extension secondary to phlebitis of the mastoid veins. , It's more common in small children with AOM but may also occur in patients with COM with or without cholesteatoma.  Development of subperiosteal abscess with or without fistula provides reduction in pus pressure within the mastoid air cells, which in turn reduces the chance of infection spreading intracranially. 
Bezold's abscess was first described in 1881 by a German Otologist, Bezold as an abscess formation in the neck, strictly by definition and differentiated from the subperiosteal abscess since suppuration develops from the violation of its tip as opposed to the lateral cortex in subperiosteal abscess.  Thus, by definition, a Bezold's abscess may be considered as such only if it is formed by extension of the suppuration from the inferior portion of the mastoid to the neck.  Similarly subperiosteal abscess, the Bezold's abscess also occurs more frequently in AOM. In the case of cholesteatoma, the debris may obstruct the infection emptying into the external auditory canal and leading to erode through the mastoid tip and eventually results in a fistula. 
Only 27 cases have been reported in the English literature from 1966 to 2001  and Uchida et al.  reported 18 cases of Bezold's abscess between the period 1960 and 2002, the most likely reason being widespread use of antibiotics which has prevented complications.
Mastoid pneumatization is considered an important predisposing factor in the genesis of Bezold's abscess, and aeration of the mastoid bone resulting in thinning of its walls is an important predisposing factor. , Pneumatization takes place shortly after birth and is complete by 10 years of age. In the absence of pneumatization, the mastoid's bony walls are thick and hinder the erosion process and for this reason, this type of abscess is rarely found in children, where pneumatization of the mastoid process is still not complete. In well pneumatized bone, the spaces between the thin septa enhance the spread of the disease while in a sclerotic mastoid the thick bony walls prevent the eroding process. Hence, it is found in older children and adults. 
The thicker lateral mastoid process with the confluence of the neck muscles also serves as a strong barrier against erosion of pus laterally. Instead, pus in the mastoid erodes the area of least resistance, the digastric incisures and extends between the digastric and sternocleidomastoid muscle during the process of evolution of Bezold's abscess.  Although it is more commonly a complication of AOM with mastoiditis in children, it is a known complication of COM with cholesteatoma.  Antecedents of cholesteatoma and mastoidectomy are also considered predisposing factors for the development of Bezold's abscess. 
Because of location and rarity Bezold abscess requires a high index of suspicion, with contrast enhanced computed tomography imaging of the temporal bone and neck providing vital information for diagnosis and surgical intervention.  Prognosis is excellent if optimally managed with surgery.
| Conclusion|| |
Bezold's fistula presenting with cholesteatoma is extremely rare. However, its possibility in long-standing and neglected COM cases should be kept in mind. Recognition of involvement of the mastoid process in the presence of a neck abscess dictates the need for concurrent surgical exploration of the neck and the temporal bone.
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