Antila H, Laitio T, Aantaa R, Silvoniemi P, Pakkanen A
Department of Anaesthesiology, Turku University, Central Hospital, Finland.
Paediatr Anaesth. 1998;8(5):429-32. doi: 10.1046/j.1460-9592.1998.00763.x.
Marshall-Smith syndrome is a rare clinical disorder characterized by accelerated bone maturation, dysmorphic facial features, airway abnormalities and death in early infancy because of respiratory complications. Although patients with Marshall-Smith syndrome have several features with potential anaesthetic problems, previous reports about anaesthetic management of these patients do not exist. We present a case, in which severe hypoxia developed rapidly after routine anaesthesia induction in an eight-month-old male infant with this syndrome. After several unsuccessful attempts the airway was finally secured by blind oral intubation. After 2 weeks, laryngeal anatomy was examined with fibreoptic laryngoscopy which revealed significant laryngomalacia. Laryngoscopy was performed without problems with ketamine anaesthesia and spontaneous breathing. The possibility of a compromised airway should always be borne in mind when anaesthetizing patients with Marshall-Smith syndrome. Anaesthesia maintaining spontaneous breathing is safest for children with this syndrome. If tracheal intubation or muscle relaxation is required, precautions are needed to maintain a patent airway. Muscle relaxants should possibly be avoided before intubation.
马歇尔 - 史密斯综合征是一种罕见的临床疾病，其特征为骨骼成熟加速、面部畸形、气道异常，并因呼吸并发症在婴儿早期死亡。尽管马歇尔 - 史密斯综合征患者有一些可能存在麻醉问题的特征，但此前尚无关于这些患者麻醉管理的报道。我们报告一例，一名患有该综合征的8个月男婴在常规麻醉诱导后迅速出现严重缺氧。经过几次尝试均未成功后，最终通过盲探经口插管确保了气道安全。2周后，用纤维喉镜检查喉部解剖结构，发现明显的喉软化。在氯胺酮麻醉和自主呼吸下进行喉镜检查没有问题。麻醉患有马歇尔 - 史密斯综合征的患者时应始终牢记气道受损的可能性。对于患有该综合征的儿童，维持自主呼吸的麻醉最为安全。如果需要气管插管或肌肉松弛，需要采取预防措施以保持气道通畅。插管前可能应避免使用肌肉松弛剂。
