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Anaesthetic management of two paediatric patients with Hecht–Beals syndrome
Author(s) -
Nagata Osamu,
Tateoka Akiko,
Shiro Ryoji,
Kimizuka Mamori,
Hanaoka Kazuo
Publication year - 1999
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 - 10.1046/j.1460-9592.1999.00381.x
Subject(s) - medicine , general anaesthesia , kyphoscoliosis , intubation , anesthesia , muscle relaxation , surgery , airway management , arachnodactyly , muscle contracture , scoliosis , marfan syndrome
Summary We undertook the anaesthetic management of two children with Hecht–Beals syndrome for orthopaedic surgery under general anaesthesia. Both patients had arachnodactyly, kyphoscoliosis, and multiple congenital joint contractures, but limited mandible excursion was not obvious preoperatively in either, although mental retardation made it difficult for them to cooperate with mouth examination. They had no apparent difficulties with their mouths in daily activities. The anaesthesia records of one patient showed that intubation had been difficult in an earlier procedure. The other patient also had a history of difficult intubation, with slight tearing of the corners of her mouth during an intubation procedure. During slow induction of general anaesthesia with sevoflurane, face mask ventilation was easily performed. We attempted to visualize the larynx under anaesthesia with muscle relaxation, but we were unsuccessful because of the limited mouth opening. After several trials, blind oral intubations were fortunately successful in both patients. There were no postoperative problems with the airway.