Congenital cholesteatoma is a rare entity. It 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 in the external auditory canal. The least common site being the mastoid process. Most common presentation is a retrotympanic pearly white mass with no previous history of ear discharge, perforation or any ear surgery. It can lead to various complications, both intracranial and extracranial, some of which may be life threatening. Bezold's abscess is an extracranial complication which is usually seen in children following acute otitis media with mastoiditis. Here we present a rare case of a 60 year old patient with congenital cholesteatoma complicating to Bezold's abscess. After necessary investigations patient underwent surgery for complete removal of cholesteatoma and the abscess drainage.
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http://dx.doi.org/10.1007/s12070-011-0226-6 | DOI Listing |
J Int Adv Otol
November 2024
Department of Otolaryngology, Head and Neck Surgery, Kobe University Faculty of Medicine, Hyogo, Japan.
Bilateral congenital cholesteatoma (BCC) is rare among congenital cholesteatoma (CC) cases, and bilateral surgery poses a significant psychophysical burden on pediatric patients. Here, we present 2 pediatric cases of BCC that were successfully managed using simultaneous bilateral transcanal endoscopic ear surgery (TEES). The surgical approach provided clear visualization of the middle ear structures, enabling successful cholesteatoma removal with minimal invasiveness and a short operative time.
View Article and Find Full Text PDFDiagn Interv Radiol
December 2024
Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Radiology, Seoul, Korea.
Purpose: To investigate the differences in computed tomography (CT) features between closed-type congenital cholesteatoma (CCC) and open-type congenital cholesteatoma (OCC) of the middle ear and to evaluate the usefulness of preoperative CT examination for staging workup of congenital cholesteatoma (CC) in correlation with the surgical findings.
Methods: We retrospectively reviewed the preoperative CT scans of the temporal bone obtained from 80 patients with surgically confirmed CC of the middle ear. All patients had a solitary lesion, except for one patient with two lesions, resulting in 81 CCs, which formed the basis of this study.
Am J Med Genet A
December 2024
Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", Trieste, Italy.
Alteration in the ubiquitin-proteasome system results in human disorders with neurological and/or autoinflammatory presentation. Haploinsufficiency of PSMD12, which encodes a subunit of the core component of the proteasome, causes Stankiewicz-Isidor syndrome (STISS), characterized by intellectual disability, autism spectrum disorder, craniofacial dysmorphisms, with or without other congenital anomalies, and autoinflammation. We described six patients (four adults) from two unrelated families carrying a known p.
View Article and Find Full Text PDFAm J Case Rep
November 2024
Department of Otorhinolaryngology-Head and Neck Surgery, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.
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