Dernedde G, Pendeville P, Veyckemans F, Verellen G, Gillerot Y
Department of Anaesthesiology, UCL Medical School, Saint-Luc Hospital, Brussels, Belgium.
Can J Anaesth. 1998 Jul;45(7):660-3. doi: 10.1007/BF03012097.
The Marshall-Smith Syndrome (MSS) is a rare disease characterized by orofacial dysmorphism, failure to thrive, accelerated osseous maturation and mental retardation. It has anaesthetic implications due to upper airway problems and possible atlanto-axial instability. We present the perioperative problems (difficult intubation, airway obstruction) encountered in a child with MSS who underwent several anaesthetics during his first two years of life.
At birth, the child presented with asphyxia due to obstructive apnoea. His trachea was, therefore, intubated immediately. The morphological diagnosis of MSS was confirmed by the pathognomonic radiological appearance of the bones (bone age was eight months at the age of four days). Upper airway difficulty was caused by functional problems at the level of the hypopharynx (inspiratory collapse at the level of the velum palatinum), and was solved by the use of a nasopharyngeal airway (NPA) during the induction of anaesthesia and early postoperative period.
The use of an NPA during both induction and recovery of anaesthesia may be particularly useful to prevent upper airway problems in children with MSS.
马歇尔 - 史密斯综合征（MSS）是一种罕见疾病，其特征为口面部畸形、生长发育迟缓、骨成熟加速和智力障碍。由于上呼吸道问题和可能的寰枢椎不稳定，它具有麻醉方面的影响。我们介绍了一名患有MSS的儿童在其生命的头两年接受多次麻醉时遇到的围手术期问题（插管困难、气道阻塞）。
出生时，该儿童因阻塞性呼吸暂停出现窒息。因此，他立即接受了气管插管。通过骨骼典型的放射学表现（出生4天时骨龄为8个月）确诊为MSS。上呼吸道困难是由下咽水平的功能问题（软腭水平吸气时塌陷）引起的，在麻醉诱导期和术后早期使用鼻咽气道（NPA）得以解决。
在麻醉诱导和苏醒期间使用NPA对于预防患有MSS的儿童出现上呼吸道问题可能特别有用。
