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Anesthesia in a child with fibrodysplasia ossificans progressiva
Author(s) -
VASHISHT RITA,
PROSSER DYLAN
Publication year - 2006
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1111/j.1460-9592.2005.01837.x
Subject(s) - medicine , fibrodysplasia ossificans progressiva , abscess , tongue , ankylosis , heterotopic bone , surgery , pierre robin syndrome , airway management , airway , anesthesia , dentistry , heterotopic ossification , pathology
Summary We describe the anesthesia management of a 12‐year‐old girl, diagnosed with fibrodysplasia ossificans progressiva (FOP), who presented with a submandibular abscess. FOP is a rare, inherited disorder with heterotopic bone formation and progressive musculoskeletal disability. This disability ultimately confines patients to a wheelchair. Minor trauma following dental treatment may lead to ankylosis of the jaw. Subsequent to this disability, which resulted in poor dental hygiene, our patient developed a dental abscess. This spread along the mandibular margin and under the tongue. She presented with an impending airway compromise in an already difficult situation. The options for airway management in a child with limited mouth opening are discussed.