Scimago Lab
powered by Scopus
eISSN: 1941-5923
call: +1.631.629.4328
Mon-Fri 10 am - 2 pm EST


Get your full text copy in PDF

Chronic Otitis Media Associated with Cholesteatoma in a Case of the Say-Barber-Biesecker-Young-Simpson Variant of Ohdo Syndrome

Bruno Galletti, Francesco Gazia, Francesco Freni, Rita Angela Nicita, Rocco Bruno, Francesco Galletti

(Adult and Development Age Human Pathology, Gaetano Barresi Unit of Otorhinolaryngology, University of Messina, Messina, Sicily, Italy)

Am J Case Rep 2019; 20:175-178

DOI: 10.12659/AJCR.913893

BACKGROUND: The Say-Barber-Biesecker-Young-Simpson (SBBYS) variant of Ohdo syndrome is characterized by congenital hypothyroidism, facial dysmorphism, postaxial polydactyly, and mental retardation. The SBBYS variant of Ohdo syndrome is extremely rare with only 19 cases previously reported in the literature. A case is presented of chronic otitis media associated with cholesteatoma in a six-year-old boy with the SBBYS variant of Ohdo syndrome.
CASE REPORT: A 6-year-old boy presented with perforation of the tympanic membrane and a cholesteatoma in the mesotympanic-attic region associated with chronic otitis media. The child had previously been diagnosed with the SBBYS variant of Ohdo syndrome. Following computed tomography (CT) and magnetic resonance imaging (MRI), tympanoplasty was performed with removal of the lesion.
CONCLUSIONS: This is the first case described in the literature of chronic otitis media associated with cholesteatoma in a patient with the SBBYS variant of Ohdo syndrome. This case demonstrates the importance of specialist otolaryngology referral for patient management.

This paper has been published under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially.
I agree