Delayed Presentation of a Congenital Cholesteatoma in a 64-year-old Man: Case Report and Review of the Literature (original) (raw)
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Unusual cases of congenital cholesteatoma of the ear
The Journal of Laryngology & Otology, 1999
Congenital cholesteatoma may originate at various sites in the temporal bone. For example, in the petrous apex, the cerebellopontine angle, the middle ear cavity, the mastoid process or the external auditory canal. The least common site being the mastoid process. We present two cases of congenital cholesteatoma of the mastoid process, each presenting with different symptoms and at different ages. Both patients underwent surgical treatment, which confirmed the diagnosis and radiological findings.
Congenital Cholesteatoma of the Mastoid Temporal Bone
The Laryngoscope, 2007
Congenital mastoid cholesteatomas are rare lesions of the temporal bone. The clinical presentation of these lesions is variable, making them difficult to identify preoperatively. We evaluated our series of mastoid congenital cholesteatomas (CCs) in an effort to better define the clinical presentation, imaging characteristics, and surgical challenges specific to this lesion. Retrospective chart and radiologic study review. The medical records of patients with the diagnosis of mastoid CC on radiologic imaging over a 15-year period were reviewed. All had surgical and pathologic confirmation. Eight patients underwent preoperative computed tomography (CT). Six also underwent magnetic resonance (MR) scanning. Demographic information, clinical presentation, imaging results, and operative findings were recorded. Nine patients with the diagnosis of mastoid CC satisfying the inclusion criteria were found. Clinical findings were variable, with the most common presentation being an incidental finding. Imaging findings were more uniform. All CT scans demonstrated an expansile, well-circumscribed mass centered within the mastoid portion of the temporal bone. All MR scans showed a well-circumscribed mass with high intensity on T2-weighted images with precontrasted T1 sequences showing the lesion to be isointense or slightly hyperintense to cerebrospinal fluid (CSF). Operative findings included lateral mastoid cortex erosion, sigmoid sinus exposure, ossicular destruction, facial nerve exposure, and associated postauricular abscess. Management of these lesions is reviewed. Congenital mastoid cholesteatomas have a variable and nonspecific clinical presentation. Surgical challenges arise from the indolent nature of this clinical entity, which belies the extent of otologic involvement. Imaging with CT and magnetic resonance imaging are diagnostic, defines the extent of these lesions, and facilitates preoperative surgical planning.
Congenital cholesteatoma of mastoid origin: A multicenter case series
Vojnosanitetski pregled, 2014
Background/Aim. The mastoid is the rarest site for the onset of congenital cholesteatoma (CC). The symptoms are atypical and minimal. The aim of this multicenter retrospective descriptive study was to define this extremely rare condition and its clinical presentation, diagnosis and management. Methods. We analyzed data files for a 15- year period in 4 tertiary otology centers and discovered 6 patients with the diagnosis of CC of the mastoid. Results. The clinical presentation of CC varied from incidental findings in patient to patient with otogenic meningitis. The most common findings during surgical procedures were mastoid cortex erosion, sigmoid plate dehiscence, dural exposure and external canal wall destruction. Conclusion. CC of mastoid origin tends to occur in adult patients probably because of minimal symptoms and the delayed diagnosis. It can exist for years in a nonaggressive state and develop to giant sizes. In children it is almost incidentally diagnosed. Early imaging is...
A Unified Classification of Middle Ear and Petrous Bone Congenital Cholesteatomas
The Journal of International Advanced Otology
BACKGROUND: The aim of this study was to classify congenital cholesteatoma along an entire spectrum of involvement ranging from the middle ear to petrous apex. METHODS: A total of 131 patients (85 adults and 46 children) underwent operations for congenital cholesteatoma over the duration of 27 years. RESULTS: For most cases, middle ear mucosa was normal, the first ossicle eroded by the mass was the stapes, and the mastoid air cell system was well-pneumatized on intraoperative and radiographic views. Totally 34% of patients presented with facial nerve weakness and 45% of these cholesteatomas arose from the supralabyrinthine area (32.8%) and from the petrous apex (12.2%). CONCLUSION: In this unified classification system, the otologist sees congenital cholesteatoma as a continuum, with facial nerve involvement and anacusis as part of the picture. This system of congenital cholesteatoma accommodates the supralabyrinthine and petrous bone locations of the disease.
Atypical presentation of congenital cholesteatoma in an adult case with good hearing result
Annals of Medicine and Surgery, 2015
Introduction: Congenital cholesteatoma is thought to be caused by inadequate folding of the epidermoid formation inside the middle ear cleft. During development of the middle ear mucosa, stratified squamous epithelium accumulates in the embryonic life. Its typical appearance is a "pearl" beneath the anterosuperior quadrant of the tympanic membrane. Presentation of case: We report 28 years-old case with congenital cholesteatoma in the posterosuperior quadrant of middle ear cavity. The main complaint was the hearing loss which had developed slowly over several years. Discussion: The case was surgically treated. Postoperative hearing result was satisfactory. Conclusion: Congenital cholesteatoma may occur in atypical locations and ages. Many authors prefer canal wall down tympanomastoidectomy. But it can also be treated successfully by intact canal wall tympanomastoidectomy with good hearing results.
Cholesteatoma: Congenital and Acquired
Military Medicine, 1978
CHOLESTEATOMA, a progressively enlarging accumulation of keratinizing squamous epithelium within the temporal bone, is a disease process which is of common interest to the pediatrician, family practitioner, and otologist. These lesions may be classified into congenital and acquired varieties,1,4,9 as noted in Table I. While the acquired types are relatively common, congenital cholesteatomas remain asymptomatic until erosion into important structures in the temporal bone, or secondary infection arouses suspicion of their presence. Those arising in the middle ear can frequently be diagnosed by otoscopy prior to the onset of significant destruction. Four cases of congenital cholesteatoma are reported. The diagnosis, pathophysiology, and management of this condition is reviewed in an effort to stimulate renewed awareness of congenital cholesteatoma, and facilitate early diagnosis and treatment prior to the development of extensive otologic disease. R~port of Cases Case J. A two year old male was referred for evaluation of recurrent episodes of bilateral, acute otitis media. There was no past history of otorrhea or hearing loss. Examination showed bilateral pearly white masses in the anterior-superior quadrant of each mesotympanum (Fig. 1). The tympanic membranes were intact, translucent and slightly retracted. Surgical exploration of the right middle ear disclosed an easily removable three to four mm spherical mass attached to the anterior portion of the neck of the malleus (Figs. 2 and 3). Six weeks later, surgical exploration of the left middle ear showed a similar lesion. Histological examination of both masses was compatible with the diagnosis of cholesteatoma. Case 2. A 26 month old male was referred because of an abnormal otologic examination. There were no symptoms of otologic disease. Examination revealed a white mass in the anterior-superior quadrant of the right mesotympanum. Surgical exploration disclosed a three to four mm mass attached to the undersurface of the tympanic membrane which was removed without difficulty. By histologic examination, the diagnosis was cholesteatoma. Case 3. A five year old male was referred in 1971 for an evaluation of serous otitis media. The tympanic membranes were intact, dull, and retracted. Pure tone audiometry showed a 25dB conductive loss in the right ear and 15dB loss in the left. Myringotomies were performed. Ten months later, hearing in the left ear deteriorated to a 53dB conductive loss. The tympanic membranes were opaque and tympanosclerotic, and myringotomies were again performed. Two years later, due to persistant conductive loss and "thickening" of the posterior-superior quadrant of the left tympanic mem-From the
Congenital cholesteatoma of the middle ear - uncommon clinical presentation
Vojnosanitetski pregled, 2014
Introduction. Congenital cholesteatoma of the middle ear is un uncommon and yet not well-defined disease. Only few cases of cholesteatoma in the fossa ovalis with unusual clinical presentation have been reported in medical literature. Case report. We reported a 16-year-old girl with congenital cholesteatoma in the fossa ovalis with minimal clinical presentation. A small mass was found occluding the fossa ovalis and mimicking otosclerotic process within tympanic cavity. The operation started as stapedotomy, and when the process was confirmed it converted to mastoidectomy via the retroauricular approach. Conclusion. The diagnosis of congenital cholesteatoma in children should always be considered, even if the clinical symptoms imitate other ear disorders, in our case otosclerosis.
Mastoid and Epitympanic Bony Obliteration in Pediatric Cholesteatoma
Otology & Neurotology, 2008
Objective: The primary goal of cholesteatoma surgery is complete eradication of the disease. To lower the recurrence rate in the pediatric population in canal wall up techniques and to avoid the disadvantages of canal wall down techniques, the bony obliteration technique with epitympanic and mastoid obliteration has been developed. The objective of this study was to evaluate the long-term surgical outcome and recurrence rate of this technique in children. Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Fifty-two children (G16 yr) were operated on in 90.4% (n = 47) for a primary or recurrent cholesteatoma and in 9.6% (n = 5) for an unstable cavity. Intervention: In all cases, we closed the tympanoattical barrier and the posterior tympanotomy with sculpted cortical bone and then completed obliteration of the epitympanum and mastoid with bone pâté. A reconstruction of the middle ear was performed by means of an allograft tympanic membrane including the malleus handle and a sculpted allograft malleus or incus for columellar reconstruction. Main Outcome Measures: Recurrent rate; residual rate; functional outcome; hygienic status of the ear; long-term safety issues. Results: The mean follow-up time was 49.5 months (range, 12Y101.3 mo). Recurrent cholesteatoma occurred in 1.9% (n = 1). Residual cholesteatoma was detected in 15.4% (n = 8) of the cases. Postoperative hearing results revealed a median gain on pure-tone averages of 14.3 dB and a median postoperative air-bone gap of 25.6 dB. Conclusion: The mastoid and epitympanic BOT is an effective technique to lower the recurrence rate of cholesteatoma in the pediatric population. Follow-up by magnetic resonance imaging provides a safe, noninvasive method for postoperative detection of residual cholesteatoma. Key Words: Bone pâtéVCholesteatomaVMastoid obliterationVMastoidectomy. Otol Neurotol 00:00Y00, 2008.
The Journal of International Advanced Otology, 2016
Congenital cholesteatoma within the tympanic membrane is an uncommon entity, with only few cases being documented. The aetiopathogenesis of this lesion is still unknown; however, when cholesteatoma develops in subjects without any history of previous ear inflammation, as in the case we report here, an embryologic origin is deeply suspected. An acquired origin is hypothesized in patients with a previous history of an inflammatory process of the external or middle ear because of the proliferation of the basal cell layer of the tympanic membrane epithelium. We report a rare case of congenital cholesteatoma of the tympanic membrane in an adult patient and review the literature KEYWORDS: Congenital intratympanic cholesteatoma, intratympanic cholesteatoma, congenital cholesteatoma of the tympanic membrane, cholesteatoma of the tympanic membrane