title: Anesthetic management of a child with Langer-Giedion (TRPS II) syndrome.
abstract: We describe the anesthetic and perioperative management of a child with Langer-Giedion syndrome (trichorhinophalangeal syndrome type II). This is a very rare genetic syndrome caused by 8q chromosome deletion. The clinical features of this syndrome include craniofacial and urogenital abnormities, variable postnatal growth deficiency with mental retardation, multiple exostoses, hyperflexible joints, and recurrent respiratory tract infections. Potential perioperative problems are highlighted.
authors.affiliation: Department of Anaesthetics, Antrim Area Hospital, 45 Bush Road, Antrim BT41 2RL, UK/Northern Ireland.
authors.forename: Pavel
authors.initials: P
authors.lastname: Michalek
authors.forename: John T
authors.initials: JT
authors.lastname: Doherty
authors.forename: Michaela Michalkova
authors.initials: MM
authors.lastname: Vesela
mesh_terms: D000556:Ambulatory Surgical Procedures; D000768:Anesthesia, General; D002648:Child; D006801:Humans; D015826:Langer-Giedion Syndrome / Q000150:complications* / Q000601:surgery*; D017214:Laryngeal Masks; D008297:Male; D016343:Monitoring, Intraoperative; D019647:Oral Surgical Procedures; D019990:Perioperative Care; D014081:Tooth Extraction
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