title: Diagnostic and surgical challenge: middle ear dermoid cyst in 12 month old with branchio-oto-renal syndrome and multiple middle-ear congenital anomalies.
abstract: Described is the first case report, to our knowledge, of a middle-ear dermoid in a child with branchio-oto-renal (BOR) syndrome. Radiographic, pathologic, and intraoperative figures are shown. This was a diagnostic and surgical challenge as the presentation was similar to a congenital cholesteatoma and the child had numerous significant temporal bone abnormalities. After the intraoperative findings suggested a non-destructive process, the treatment strategy was altered. This case reiterates the need for a cautious, flexible operative approach in a syndromic child. Included is a relevant review of the literature and a detailed clinical analysis.
authors.affiliation: Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, USA. dougjohnston3@gmail.com
authors.forename: D R
authors.initials: DR
authors.lastname: Johnston
authors.forename: K
authors.initials: K
authors.lastname: Whittemore
authors.forename: D
authors.initials: D
authors.lastname: Poe
authors.forename: C D
authors.initials: CD
authors.lastname: Robson
authors.forename: A R
authors.initials: AR
authors.lastname: Perez-Atayde
mesh_terms: D019280:Branchio-Oto-Renal Syndrome / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D003884:Dermoid Cyst / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D004428:Ear Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D004432:Ear, Middle; D006801:Humans; D007223:Infant; D008297:Male; D013701:Temporal Bone / Q000002:abnormalities*