Proteus syndrome with huge tonsillar mass causing dysphagia: a rare case

Sahni, J. K.; Kumar, Sunil; Wadhwa, Vibhor; Kathuria, Geeta

doi:10.1017/s0022215106000430

Cited by 9 publications

(8 citation statements)

References 1 publication

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“…Previous studies reported asymmetric tonsillar enlargement in PS patient with OSAS treated with tonsillectomy under general anesthesia [ 12 ], but this is the first case of partial tonsillar resection under local anaesthesia. A retrospective study concludes that partial tonsillar resection is as effective as tonsillectomy for the long-term treatment of children suffering from OSAS due to hypertrophic tonsils [ 13 ].…”

Section: Discussionmentioning

confidence: 90%

Operative Management of OSAS in a Complex Case of Proteus Syndrome

Cantone

Cavaliere

Castagna

et al. 2015

Case Reports in Otolaryngology

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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.

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Section: Discussionmentioning

confidence: 90%

Operative Management of OSAS in a Complex Case of Proteus Syndrome

Cantone

Cavaliere

Castagna

et al. 2015

Case Reports in Otolaryngology

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“…These supraglottic mass were presumed to be lingual tonsillar hypertrophy and palatine tonsillar hypertrophy. Although there is no report of difficult intubation due to tonsillar hypertrophy in PS, a huge tonsillar mass causing dysphagia was reported as a manifestation of PS [ 9 ]. No case of PS involving a rugged surface or protruded masses of trachea has been reported, therefore it is not certain whether this feature is common or not.…”

Section: Discussionmentioning

confidence: 99%

Bronchoscopic concerns in Proteus syndrome: a case report

Hong

Lee

Kim

et al. 2016

Korean J Anesthesiol

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Proteus syndrome (PS) is a rare congenital hamartomatous disorder with multisystem involvement. PS shows highly clinical variability due to overgrowth of the affected areas, and several features can make anesthetic management challenging. Little is known about the airway problem associated with anesthesia in PS patients. An 11-year-old girl with PS was scheduled for ear surgery under general anesthesia. She had features complicating intubation including facial asymmetry and disproportion, abnormal teeth, limitation of neck movement due to torticollis, and thoracolumbar scoliosis. This study reports on a case of deformed airway of a PS patient under fiberoptic bronchoscopy.

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“…5 Evaluation includes skeletal survey with CT and magnetic resonance imaging in order to rule out any central nervous system manifestations.…”

Section: Discussionmentioning

confidence: 99%

“…Patients carry a social stigma due to the disfiguring effect of the syndrome. Evaluation includes skeletal survey with CT and magnetic resonance imaging in order to rule out any central nervous system manifestations 5 …”

Section: Discussionmentioning

confidence: 99%