Sisk E A, Heatley D G, Borowski B J, Leverson G E, Pauli R M
Division of Otolaryngology-Head and Neck Surgery, University of Wisconsin Medical School, USA.
Otolaryngol Head Neck Surg. 1999 Feb;120(2):248-54. doi: 10.1016/S0194-5998(99)70414-6.
To evaluate the prevalence of obstructive sleep apnea in a large population of children with achondroplasia and to evaluate the effectiveness of adenoidectomy and/or tonsillectomy as treatment.
Retrospective review of 95 children with achondroplasia.
Thirty-six patients (38%) had clinical evidence of obstructive sleep apnea. Thirty-four patients underwent surgery, with more than 1 procedure required in 10 children (29%). Adenotonsillectomy was the initial procedure for 22 of 34 patients, and further therapy was required in only 18% of this group. Adenoidectomy was the initial procedure for 10 of 34, with 90% requiring further surgery for recurrent obstructive sleep apnea. Tonsillectomy alone was performed in 2 patients: 1 was effectively treated and 1 later required adenoidectomy. Endotracheal intubation was accomplished in all patients without complication; 53% required a smaller endotracheal tube than would be predicted by their age. Eight postoperative complications were recorded.
Obstructive sleep apnea is very common in children with achondroplasia. Surgery is effective, but recurrent symptoms are common, particularly when the initial procedure is adenoidectomy. The complication rate is higher than that observed in a general pediatric population but is readily managed with standard therapy. Anesthesia can be given safely to these patients with special consideration for limited neck extension and appropriate endotracheal tube size.
评估大量软骨发育不全儿童中阻塞性睡眠呼吸暂停的患病率，并评估腺样体切除术和/或扁桃体切除术作为治疗方法的有效性。
对95例软骨发育不全儿童进行回顾性研究。
36例患者（38%）有阻塞性睡眠呼吸暂停的临床证据。34例患者接受了手术，10例儿童（29%）需要进行不止一次手术。腺样体扁桃体切除术是34例患者中22例的初始手术，该组中只有18%需要进一步治疗。腺样体切除术是34例中10例的初始手术，90%因复发性阻塞性睡眠呼吸暂停需要进一步手术。仅对2例患者进行了扁桃体切除术：1例得到有效治疗，1例后来需要进行腺样体切除术。所有患者均成功完成气管插管，无并发症；53%的患者所需气管插管尺寸小于根据其年龄预测的尺寸。记录了8例术后并发症。
阻塞性睡眠呼吸暂停在软骨发育不全儿童中非常常见。手术有效，但复发症状很常见，尤其是当初始手术为腺样体切除术时。并发症发生率高于普通儿科人群，但通过标准治疗很容易处理。对这些患者进行麻醉时可安全给药，需特别考虑颈部伸展受限和合适的气管插管尺寸。
