Congenital mastoid cholesteatoma of adult patient masquerading as complicated otitis media – A case report and literature review

Balaji Ramamourthy; Anurag Snehi Ramavat; Neemu Hage; Arun Govind

doi:10.4103/indianjotol.indianjotol_243_20

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CASE REPORT

Year : 2022 | Volume : 28 | Issue : 2 | Page : 149-152

Congenital mastoid cholesteatoma of adult patient masquerading as complicated otitis media – A case report and literature review

Balaji Ramamourthy, Anurag Snehi Ramavat, Neemu Hage, Arun Govind
Department of Otolaryngology and Head and Neck Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission	06-Nov-2020
Date of Decision	20-Jan-2021
Date of Acceptance	22-Mar-2021
Date of Web Publication	21-Sep-2022

Correspondence Address:
Dr. Balaji Ramamourthy
Department of Otorhinolaryngology and Head & Neck Surgery, All India Institute of Medical Education and Research (AIIMS), Bibinagar- 500 088
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/indianjotol.indianjotol_243_20

Abstract

The study highlights the management protocol of a large mastoid cholesteatoma presenting with sudden-onset facial palsy mimicking a complicated otitis media and literature review. Congenital cholesteatoma may affect various regions of the temporal bone, the mastoid process being the least frequently involved. The diagnosis is often missed, specifically when the patient presents with features of mastoiditis. Radiology plays an important role in clenching the diagnosis. Congenital (mastoid) cholesteatoma manifesting with features of complicated otitis media is a rare occurrence and needs familiarity to facilitate early diagnosis and appropriate management.

Keywords: Congenital cholesteatoma, facial palsy, mastoid abscess, temporal bone

How to cite this article:
Ramamourthy B, Ramavat AS, Hage N, Govind A. Congenital mastoid cholesteatoma of adult patient masquerading as complicated otitis media – A case report and literature review. Indian J Otol 2022;28:149-52

How to cite this URL:
Ramamourthy B, Ramavat AS, Hage N, Govind A. Congenital mastoid cholesteatoma of adult patient masquerading as complicated otitis media – A case report and literature review. Indian J Otol [serial online] 2022 [cited 2023 Feb 6];28:149-52. Available from: https://www.indianjotol.org/text.asp?2022/28/2/149/356450

Introduction

In 1965, Derlacki and Clemis^[1] defined congenital cholesteatoma (CC) for the first time by outlining criteria for its diagnosis, which was later modified in 1986 by Levenson et al.^[2] The incidence of CC reported in the literature is 0.12 per 100,000.^[3] Approximately 1%–3% of the childhood cholesteatoma cases, and 1%–5% of the cholesteatoma in general, are congenital.^[4] CC may originate within the temporal bone in various locations, including the petrous apex, the middle ear, the area of the geniculate ganglion, the mastoid process, the jugular foramen, and the squamous portion of the temporal bone.^[1] However, occurrence in the mastoid process is the least frequent.^[5] Here, we report a rare case of the CC of the mastoid in an adult patient who presented with otalgia and facial nerve palsy, confounding our diagnosis with that of acute mastoiditis.

Case Report

A 50-year male patient presented to the emergency department with complaints of left-sided otalgia and left-sided facial weakness for a week. The ear pain was dull aching, mild to moderate in severity, intermittent, and nonradiating. Facial weakness was acute onset and nonprogressive. The patient denied any history of ear discharge, vertigo, or tinnitus; however, he reported fullness of the ear and mild diminution of hearing on the affected side. There was no history of fever, headache, altered sensorium, seizures, or difficulty in ambulation.

The patient had no other ear complaints in the past, and there was no history of trauma. The patient received a course of oral antibiotics before presenting to our institution.

On examination, the patient was fully conscious, oriented, and afebrile. He had House Brackmann Grade V facial paresis on the left side. No mastoid or cymba concha tenderness was elicited; however, the patient had tragal tenderness. On otoscopic examination, the cartilaginous part of the external auditory canal was unremarkable; however, the deep part was congested. The tympanic membrane was intact and dull. There was congestion over the posterosuperior quadrant of the tympanic membrane. A working diagnosis of complicated acute otitis media was made.

A high-resolution computed tomography (CT) of temporal bone revealed a huge coalescent cavity with the destruction of mastoid septae. The disease was also found to be eroding the overlying cortical bone, posteriorly the sigmoid plate; however, the tegmen appeared intact. The soft-tissue density also involved the attic, but no widening of the aditus was appreciated, and the ossicles were also unaffected. The disease was in close relation with the posterior cranial fossa [Figure 1]. Hence, contrast-enhanced magnetic resonance imaging (MRI) was done, which showed a T1 isointense and T2 hyperintense lesion involving the mastoid region. The lesion encroached the cerebellum, but fat planes with the dura were maintained [Figure 2]a and [Figure 2]b.

Figure 1: Congenital cholesteatoma of mastoid showing the destruction of posterior fossa plate, sigmoid sinus, and closely abutting cerebellum

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Figure 2: (a) T1-weighted image showing hypointense lesion. (b) Showing T2-weighted image showing hyperintense lesion of the mastoid closely abutting cerebellum

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Pure-tone audiometry of the patient showed moderate mixed hearing loss in the left ear and normal hearing sensitivity in the right. A facial nerve excitatory stimulation was done preoperatively.

An emergency mastoid exploration was performed with intraoperative findings of a huge cholesteatoma sac behind an intact tympanic membrane which had replaced the mastoid air cells, caused the destruction of mastoid cortical bone, sigmoid plate, and exposure of the posterior cranial fossa dura [Figure 3]a. Tegmen and ossicular chain were intact. The bony Fallopian canal ^{More Details} was intact in its tympanic segment, whereas it was dehiscent in its entire course in the mastoid segment. Attic and aditus were normal except for edematous mucosa. The semicircular canals were intact.

Figure 3: (a) Huge mastoid cavity after removal of cholesteatoma showing the intact posterior fossa dura. (b) Completely healed postoperative cavity at 18-month follow-up

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A modified radical mastoidectomy was done, which resulted in a huge cavity. The cavity was obliterated using fat and bone pâté. Wullstein Type III tympanoplasty was performed.

Postoperatively, the patient had persistent ear discharge, which was managed conservatively. The cavity was dry after a year of vigilant follow-up. The facial paresis, however, persisted. He was last reviewed at 18 months post surgery and has been rendered disease-free [Figure 3]b. We intend to keep him on a longer follow-up.

Discussion

CC arises from an epidermoid cyst from a congenital remnant of the keratinizing squamous epithelium of the temporal bone.^[6]

CC of mastoid origin has the following characteristics:

The initial presentation is usually pain in the neck and mastoid region
CT scan shows an expansile, cystic lesion occupying the mastoid process without involving the middle ear, which may compress the cerebellum and the dural sinuses adjacent to it and/or erode the bony labyrinth
MRI scanning shows little or no peripheral enhancement on postcontrast T1-weighted images and hyperintensity on T2-weighted images.^[1],[7],[8]

Similar presenting complaints and radiological appearance can be noted in our case as well [Figure 1] and [Figure 2]. However, facial nerve palsy as a presenting complaint with features of acute mastoiditis is rare.

Hidaka et al. reported a similar case where the patient presented with postauricular swelling and pain as the presenting complaint. Along with mastoid abscess, the patient also had an isolated mastoid CC with the erosion of posterior fossa bony plate and sigmoid plate similar to the findings in our patient.^[8]

As in most patients of CC, mastoid was well pneumatized in our patient. Pnematization is not affected because inflammation is absent in CC. Whereas, in acquired cholesteatoma inflammation and pathologic changes of mucosa are more evident leading to sclerotic mastoid. Kojima et al. analyzed temporal bone pneumatization in 53 patients of CC, 27 (50.9%) and 16 (30.2%) had very good and good pneumatization, respectively. They also found that otorrhea or otalgia was an initial symptom in 17.5% of the patients.^[9]

Potsic et al. described a staging system for CC, which are defined as follows:

Stage I – Single quadrant: No ossicular involvement or mastoid extension
Stage II – Multiple quadrants: No ossicular involvement or mastoid extension
Stage III – Ossicular involvement: It includes erosion of ossicles and surgical removal for eradication of disease; no mastoid extension
Stage IV – Mastoid extension (regardless of findings elsewhere).

Their study showed a strong association between stage and residual disease, ranging from a 13% risk in Stage I to 67% in Stage IV.^[10] Our patient belongs to Stage IV according to their staging system, emphasizing the need for a regular long time follow-up.

Warren et al. presented a case series of nine patients who they divided into mastoid-only cholesteatoma and mastoid and middle ear cholesteatoma groups. The former group tends to be clinically silent as the lesion does not impinge upon any critical neurovascular structures and shows a higher incidence of dural exposure, facial nerve exposure, and auditory canal erosion.^[11] Our patient fits into the mastoid-only group and had both dural exposure and facial nerve exposure.

Giannuzzi et al., in their study, concluded that many cases, which were reported as mastoid cholesteatoma in the past, were not true mastoid cholesteatoma and thereby detailed the following characteristics of mastoid CC which differentiated it from middle ear CC.

Presenting complaint – Neck and temporal region pain
Age at the presentation – Adulthood
Findings at otoscopy – No abnormalities
Findings of imaging restricted to the mastoid process.

Based on their literature review, only 12 of the 20 reported cases were true mastoid cholesteatomas. Our case fits the description of true mastoid cholesteatoma in all aspects defined by Giannuzzi et al.^[12]

Our literature review on patients of CC who presented as complicated otitis media is summarized in [Table 1]. We were able to find four such cases in the literature; however, one was deliberately omitted, as it was not in English.

Table 1: Reported cases of congenital cholesteatoma presented as complicated otitis media

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^[13],[14]

Informed consent

Informed consent was obtained from the patient included in the study.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published, and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Conclusion

CC of mastoid origin is very rare and has specific characteristics like late age of presentation, destruction of adjoining structures like posterior fossa bone, facial nerve canal, sigmoid plate, tegmen, and intact ossicular chain
CC presenting as mastoiditis, though a rare phenomenon, has to be kept in mind as these cases pose special surgical challenges to the surgeon
Imaging of the temporal region helps the surgeon (a) to arrive at the diagnosis preoperatively, (b) to see the extent of disease, and (c) to do surgical planning.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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2.	Levenson MJ, Parisier SC, Chute P, Wenig S, Juarbe C. A review of twenty congenital cholesteatomas of the middle ear in children. Otolaryngol Head Neck Surg 1986;94:560-7.
3.	Bennett M, Warren F, Jackson GC, Kaylie D. Congenital cholesteatoma: Theories, facts, and 53 patients. Otolaryngol Clin North Am 2006;39:1081-94.
4.	Koltai PJ, Nelson M, Castellon RJ, Garabedian EN, Triglia JM, Roman S, et al. The natural history of congenital cholesteatoma. Arch Otolaryngol Head Neck Surg 2002;128:804-9.
5.	Nager GT. Pathology of the ear and temporal bone. Williams & Wilkins; Waverly Press, Inc., Baltimore.1993.
6.	Mevio E, Gorini E, Sbrocca M, Artesi L, Lenzi A, Lecce S, et al. Congenital cholesteatoma of the mastoid region. Otolaryngol Head Neck Surg 2002;127:346-8.
7.	Luntz M, Telischi F, Bowen B, Ress B, Balkany T. Imaging case study of the month. Congenital cholesteatoma isolated to the mastoid. Ann Otol Rhinol Laryngol 1997;106:608-10.
8.	Hidaka H, Ishida E, Kaku K, Nishikawa H, Kobayashi T. Congenital cholesteatoma of mastoid region manifesting as acute mastoiditis: Case report and literature review. J Laryngol Otol 2010;124:810-5.
9.	Kojima H, Tanaka Y, Shiwa M, Sakurai Y, Moriyama H. Congenital cholesteatoma clinical features and surgical results. Am J Otolaryngol 2006;27:299-305.
10.	Potsic WP, Samadi DS, Marsh RR, Wetmore RF. A staging system for congenital cholesteatoma. Arch Otolaryngol Head Neck Surg 2002;128:1009-12.
11.	Warren FM, Bennett ML, Wiggins RH, Salzman KL, Blevins KS, Shelton C, et al. Congenital cholesteatoma of the mastoid temporal bone. Laryngoscope 2007;117:1389-94.
12.	Giannuzzi AL, Merkus P, Taibah A, Falcioni M. Congenital mastoid cholesteatoma: Case series, definition, surgical key points, and literature review. Ann Otol Rhinol Laryngol 2011;120:700-6.
13.	Santhi K, Tang IP, Nordin A, Prepageran N. Congenital cholesteatoma presenting with Luc's abscess. J Surg Case Rep 2012;2012:rjs026.
14.	Tabook SM, Abdel Tawab HM, Gopal NK. Congenital cholesteatoma localized to the mastoid cavity and presenting as a mastoid abscess. Case Rep Otolaryngol 2015;2015:305494.