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Case Reports in Otolaryngology
Volume 2015 (2015), Article ID 137589, 4 pages
http://dx.doi.org/10.1155/2015/137589
Case Report

Operative Management of OSAS in a Complex Case of Proteus Syndrome

1Department of Neuroscience, Reproductive and Odontostomatologic Science, Section of ENT, “Federico II” University, 80131 Naples, Italy
2CEINGE-Biotecnologie Avanzate Scarl, 80131 Naples, Italy

Received 27 March 2015; Revised 1 June 2015; Accepted 14 June 2015

Academic Editor: Rong-San Jiang

Copyright © 2015 Elena Cantone et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Obstructive sleep apnea syndrome (OSAS) is a common disorder in childhood with high prevalence in syndromic subjects with craniofacial malformations. Proteus Syndrome (PS) is a rare hamartoneoplastic disorder associated with disproportionate and asymmetric overgrowth of body parts and hypertrophy or malformation of lymphatic tissues, such as palatine tonsils. We report a case of a 12-year-old boy diagnosed with Proteus Syndrome (PS) and suffering from OSAS due to asymmetric palatine tonsillar hypertrophy, treated with partial resection of left tonsil. To avoid the risk of a general anesthesia and remove only the obstructive portion of the palatine tonsil bipolar radiofrequency-induced thermotherapy (RFITT) under local anesthesia was performed. Recovery of the obstructive respiratory disease was obtained. To our knowledge, this is the first case reported in the literature of partial tonsillar resection performed in a patient with PS suffering from OSAS under local anesthesia.