Belani K G, Krivit W, Carpenter B L, Braunlin E, Buckley J J, Liao J C, Floyd T, Leonard A S, Summers C G, Levine S
Department of Anesthesiology, University of Minnesota, Minneapolis.
J Pediatr Surg. 1993 Mar;28(3):403-8; discussion 408-10. doi: 10.1016/0022-3468(93)90240-l.
The perioperative care, morbidity, and mortality in 30 patients with mucopolysaccharidosis (MPS) are presented. They underwent a detailed preoperative assessment and were anesthetized 141 times. An intravenous induction technique was used in most patients. It was easier to see the vocal cords, during laryngoscopy, in children with Hurler syndrome (HS) when they were younger (23 v 41 months, P < or = .01) and smaller (12 v 15 kg, P < or = .05). Preoperative obstructive breathing was associated with a significantly higher incidence of postextubation obstruction (P < or = .05). A total of 28 children underwent bone marrow transplantation (BMT); this reversed upper airway obstruction and also reversed intracranial hypertension. In children with HS, the incidence of odontoid dysplasia was 94%; 38% demonstrated anterior C1-C2 subluxation. Head and neck manipulation was limited in children with cervical spine defects. None of the 30 patients experienced spinal cord morbidity. One child suffered an intraoperative stroke; another, pulmonary edema. Severe and extensive coronary obstruction was responsible for 2 intraoperative deaths. Coronary angiography underestimated coronary artery disease.
本文介绍了30例黏多糖贮积症（MPS）患者的围手术期护理、发病率和死亡率。他们接受了详细的术前评估，共接受了141次麻醉。大多数患者采用静脉诱导技术。在喉镜检查时，患有Hurler综合征（HS）的儿童在年龄较小（23比41个月，P≤0.01）和体重较轻（12比15千克，P≤0.05）时，更容易看到声带。术前阻塞性呼吸与拔管后梗阻的发生率显著较高相关（P≤0.05）。共有28名儿童接受了骨髓移植（BMT）；这逆转了上呼吸道梗阻，也逆转了颅内高压。在患有HS的儿童中，齿状突发育异常的发生率为94%；38%表现为C1-C2前脱位。颈椎有缺陷的儿童头部和颈部操作受限。30例患者均未出现脊髓并发症。1名儿童术中发生中风；另1名发生肺水肿。严重和广泛的冠状动脉梗阻导致2例术中死亡。冠状动脉造影低估了冠状动脉疾病。
