Wilson A C, Moore D J, Moore M H, Martin A J, Staugas R E, Kennedy J D
Department of Pulmonary Medicine, Women's and Children's Hospital, 72 King William Road, North Adelaide, South Australia 5006, Australia.
Arch Dis Child. 2000 Nov;83(5):435-8. doi: 10.1136/adc.83.5.435.
A retrospective review was carried out of 11 consecutive patients with the Pierre Robin sequence referred to a tertiary paediatric referral centre over a five year period from 1993 to 1998. Ten patients were diagnosed with significant upper airway obstruction; seven of these presented late at between 24 and 51 days of age. Failure to thrive occurred in six of these seven infants at the time of presentation, and was a strong indicator of the severity of upper airway obstruction. Growth normalised on treatment of the upper airway obstruction with nasopharyngeal tube placement. All children had been reviewed by either an experienced general paediatrician or a neonatologist in the first week of life, suggesting that clinical signs alone are insufficient to alert the physician to the degree of upper airway obstruction or that obstruction developed gradually after discharge home. The use of polysomnography greatly improved the diagnostic accuracy in assessing the severity of upper airway obstruction and monitoring the response to treatment. This report highlights the prevalence of late presentation of upper airway obstruction in the Pierre Robin sequence and emphasises the need for close prospective respiratory monitoring in this condition. Objective measures such as polysomnography should be used, as clinical signs alone may be an inadequate guide to the degree of upper airway obstruction.
对1993年至1998年五年间转诊至一家三级儿科转诊中心的11例连续患有皮埃尔·罗宾序列征的患者进行了回顾性研究。10例患者被诊断为严重上气道梗阻；其中7例在24至51日龄时出现较晚。这7例婴儿中有6例在就诊时出现生长发育迟缓，这是上气道梗阻严重程度的一个有力指标。通过放置鼻咽管对上气道梗阻进行治疗后，生长发育恢复正常。所有儿童在出生后第一周均由经验丰富的普通儿科医生或新生儿科医生进行了检查，这表明仅靠临床体征不足以提醒医生注意上气道梗阻的程度，或者梗阻是在出院回家后逐渐发展的。多导睡眠图的使用大大提高了评估上气道梗阻严重程度和监测治疗反应的诊断准确性。本报告强调了皮埃尔·罗宾序列征中上气道梗阻出现较晚的普遍性，并强调了对这种情况进行密切前瞻性呼吸监测的必要性。应使用多导睡眠图等客观测量方法，因为仅靠临床体征可能不足以指导上气道梗阻的程度。
