关键词: Collagenopathy Difficult airway Kniest dysplasia

来  源:   DOI:10.1186/s40981-018-0178-x   PDF(Sci-hub)   PDF(Pubmed)

Abstract:
BACKGROUND: Kniest dysplasia is a type of chondrodysplasia characterized by severe craniofacial abnormalities including tracheomalacia, midface hypoplasia, and cleft palate.
METHODS: We previously described a 6-year-old girl with Kniest dysplasia, in whom glottic edema rapidly developed after tracheal intubation. At the age of 13 years, a reoperation was scheduled to correct talipes equinovarus but was subsequently canceled due to failure of tracheal intubation and subsequent glottic edema. Airway evaluation by endoscopy and computed tomography 1 month later revealed severe laryngeal narrowing. Therefore, the second anesthesia was maintained with spinal anesthesia combined with sciatic nerve block without tracheal intubation.
CONCLUSIONS: Careful perioperative airway evaluation is required in patients with Kniest dysplasia, and alternative strategies for airway management other than tracheal intubation should be considered.
摘要:
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