title: Operative Management of OSAS in a Complex Case of Proteus Syndrome.
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.
authors.affiliation: Department of Neuroscience, Reproductive and Odontostomatologic Science, Section of ENT, "Federico II" University, 80131 Naples, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Cantone
authors.affiliation: Department of Neuroscience, Reproductive and Odontostomatologic Science, Section of ENT, "Federico II" University, 80131 Naples, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: Cavaliere
authors.affiliation: Department of Neuroscience, Reproductive and Odontostomatologic Science, Section of ENT, "Federico II" University, 80131 Naples, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Castagna
authors.affiliation: Department of Neuroscience, Reproductive and Odontostomatologic Science, Section of ENT, "Federico II" University, 80131 Naples, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Marino
authors.affiliation: CEINGE-Biotecnologie Avanzate Scarl, 80131 Naples, Italy.
authors.forename: Luigi
authors.initials: L
authors.lastname: Del Vecchio
authors.affiliation: Department of Neuroscience, Reproductive and Odontostomatologic Science, Section of ENT, "Federico II" University, 80131 Naples, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Iengo