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Arthrogryposis multiplex congenita (AMC). A review of 32 years’ experience
Author(s) -
ZAMUDIO I.A.,
BROWN T.C.K.
Publication year - 1993
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.1460-9592.1993.tb00045.x
Subject(s) - medicine , arthrogryposis multiplex congenita , arthrogryposis , muscle contracture , intubation , retrospective cohort study , airway obstruction , surgery , anesthesia , pediatrics , airway
Summary Arthrogryposis multiplex congenita (AMC) is an uncommon condition with multiple congenital joint contractures of variable severity that is present in 1:3000 to 1:10 000 newborns. This abnormality results from a neuropathic or occasionally a myopathic lesion present in utero. Surgery is required for the correction of skeletal deformities and associated visceral anomalies. A retrospective study of the Royal Children's Hospital experience with AMC patients over the last 32 years is presented. The anaesthetic drugs and techniques used, complications, surgical procedures and mortality in 143 patients are reviewed. Thirteen patients were severely affected and died during their first week of life. One hundred and twenty‐eight patients underwent 928 procedures, the majority of them orthopaedic. Seventy complications were reported. Ten patients had a moderate increase in temperature (less than 2°C) out of 43 in whom it was recorded. There were 27 difficult intubations in 9 patients who had a total of 81 anaesthetics. Patients with AMC require careful preoperative assessment of their airway. Skill in gaining intravenous access and at intubation is essential. Monitoring of temperature, neuromuscular function and careful assessment of ventilation at the end of the anaesthetic is necessary.