Bredenkamp J K, Smith M E, Dudley J P, Williams J C, Crumley R L, Crockett D M
Department of Surgery, University of California, Los Angeles School of Medicine.
Ann Otol Rhinol Laryngol. 1992 Jun;101(6):472-8. doi: 10.1177/000348949210100605.
A retrospective review of 45 children with mucopolysaccharidoses was performed to determine the frequency of complications related to the head and neck. In this series, every patient had at least one complication involving the head and neck region, and in over half, operative intervention by the otolaryngologist was required. Upper airway obstruction occurred in 17 (38%) and necessitated a tracheostomy in 7 (16%). Cervical spine instability occurred in 8 (18%), making airway management difficult. Recurrent respiratory infections occurred in 17 (38%), and chronic recurrent middle ear effusions were noted in 33 (73%). This review demonstrates that children afflicted with the mucopolysaccharidoses frequently have otolaryngologic-related complications that are common throughout their life span and often the primary management issue in their continuing care. The otolaryngologic management of these patients is outlined based on the results of this study and review of the relevant literature.
对45例黏多糖贮积症患儿进行了回顾性研究，以确定与头颈部相关并发症的发生率。在这组病例中，每位患者至少有一项涉及头颈部区域的并发症，超过半数的患者需要耳鼻喉科医生进行手术干预。17例（38%）出现上气道梗阻，其中7例（16%）需要行气管切开术。8例（18%）出现颈椎不稳定，导致气道管理困难。17例（38%）出现反复呼吸道感染，33例（73%）出现慢性复发性中耳积液。该研究表明，患有黏多糖贮积症的儿童经常出现与耳鼻喉科相关的并发症，这些并发症在其整个生命周期中都很常见，并且往往是其持续护理中的主要管理问题。基于本研究结果和相关文献综述，概述了这些患者的耳鼻喉科管理方法。
