Tomaski S M, Zalzal G H, Saal H M
Department of Pediatric Otolaryngology-Head and Neck Surgery, Children's National Medical Center, George Washington University School of Medicine, Washington, D.C. 20010, USA.
Laryngoscope. 1995 Feb;105(2):111-4. doi: 10.1288/00005537-199502000-00001.
Airway obstruction and feeding difficulties vary among patients with Pierre Robin sequence (PRS). Treatment is challenging and the appropriate management may not be readily identified, leading to delay in securing the airway. A retrospective review of 90 children with PRS was done to identify subgroups at a higher risk of developing severe airway obstruction using oxygen and apnea monitoring, sleep studies, and response to treatment. Patients with isolated PRS (group I, 27 patients) and Stickler syndrome (group II, 32 patients) do not suffer from debilitating airway and feeding difficulties when compared to those with unique syndromes (group III, 16 patients) and recognized named syndromes (group IV, 15 patients). Feeding difficulties were universal with the severity proportional to airway obstruction. Aggressive intervention should be considered early in group III and IV patients.
患有Pierre Robin序列征（PRS）的患者气道梗阻和喂养困难情况各不相同。治疗具有挑战性，且可能无法轻易确定适当的管理方法，从而导致气道保障延迟。对90例PRS患儿进行了回顾性研究，以通过氧气和呼吸暂停监测、睡眠研究以及对治疗的反应来确定发生严重气道梗阻风险较高的亚组。与患有独特综合征（第三组，16例患者）和已确认命名综合征（第四组，15例患者）的患者相比，孤立性PRS患者（第一组，27例患者）和Stickler综合征患者（第二组，32例患者）不存在严重的气道和喂养困难。喂养困难普遍存在，其严重程度与气道梗阻成正比。对于第三组和第四组患者，应尽早考虑积极干预。
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