Refractory sleep-disordered breathing due to unilateral lingual tonsillar hypertrophy in a child with Proteus Syndrome

Publication date: Available online 14 February 2017
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Courtni R. Salinas, Brian A. Nuyen, Aria Jafari, Javan Nation
Proteus Syndrome (PS) is a rare congenital overgrowth disease affecting bones, skin, adipose and the central nervous system. The result is asymmetric, disfiguring hypertrophy which can manifest as craniofacial dysmorphia and aerodigestive tract abnormalities. We report the case of obstructive lingual tonsillar hypertrophy resulting in residual sleep disordered breathing after adenotonsillectomy in a child with PS, a previously unrecognized manifestation of the disease. Endoscopic treatment with coblation effectively and safely treated the obstructive symptoms.



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