From the University of Alabama at Birmingham School of Medicine, Birmingham, AL.
Division of Plastic Surgery, Baylor College of Medicine, Houston, TX.
Ann Plast Surg. 2021 Jun 1;86(6S Suppl 5):S545-S549. doi: 10.1097/SAP.0000000000002702.
Pierre Robin sequence (PRS) is a triad of congenital facial abnormalities that can present as a syndrome (syndromic PRS [sPRS]) or an isolated entity (isolated PRS [iPRS]). Patients with PRS can develop airway and feeding problems that may result in failure to thrive. Mandibular distraction osteogenesis (MDO) is a method for improving the functional issues associated with breathing and feeding. There is a Paucity of literature evaluating the outcomes of MDO between sPRS and iPRS patients.
An institutional review board-approved retrospective review of PRS patients managed by a single surgeon and treated with MDO between January 2015 and December 2019 at a tertiary referral hospital was performed. The patients were stratified into iPRS or sPRS based on gene testing. Airway outcome measures included avoidance of tracheostomy, relief of sleep apnea, and oxygen saturation improvement. Primary feeding measures included achievement of full oral feeds and growth/weight gain. Statistical analysis included t tests and χ2 tests where appropriate using SPSS.
Over the study period, of the 29 infants with PRS, 55% identified as iPRS and 45% as sPRS. There were no significant differences in the patient characteristics, apnea-hypoxia index (22.27 ± 12.27) and laryngeal view (3 ± 0.79) pre-MDO. After MDO, 83% of the subjects achieved a positive feeding outcome and 86% achieved a positive airway outcome with no statistical significance between sPRS and iPRS (P = 0.4369). There was a statistically significant change post-MDO in apnea-hypoxia index (5.24 ± 4.50, P = 0.02) and laryngeal view (1.59 ± 1.00, P = 0.01).
Our recent experience would lead us to believe that sPRS patients have greater morbidities and challenging clinical developments that, when properly evaluated, can be managed by MDO. There is a potential role for MDO in reducing the need for traditional surgical interventions for respiratory and feeding problems in both iPRS and sPRS patients.
Pierre Robin 序列（PRS）是一种先天性面部畸形三联征，可表现为综合征（综合征性 PRS [sPRS]）或孤立实体（孤立性 PRS [iPRS]）。PRS 患者可能会出现气道和喂养问题，导致生长不良。下颌骨牵引成骨术（MDO）是一种改善与呼吸和喂养相关的功能问题的方法。目前，文献中评估 sPRS 和 iPRS 患者 MDO 结果的内容很少。
对 2015 年 1 月至 2019 年 12 月在一家三级转诊医院接受单外科医生治疗并接受 MDO 治疗的 PRS 患者进行机构审查委员会批准的回顾性研究。根据基因检测将患者分为 iPRS 或 sPRS。气道结果测量包括避免气管切开术、缓解睡眠呼吸暂停和改善氧饱和度。主要喂养措施包括实现完全口服喂养和生长/体重增加。使用 SPSS 进行适当的 t 检验和卡方检验进行统计分析。
在研究期间，29 名 PRS 婴儿中有 55%被确定为 iPRS，45%为 sPRS。MDO 前，患者特征、呼吸暂停低氧指数（22.27 ± 12.27）和喉视图（3 ± 0.79）无显著差异。MDO 后，83%的患者获得了积极的喂养结果，86%的患者获得了积极的气道结果，sPRS 和 iPRS 之间无统计学意义（P = 0.4369）。MDO 后呼吸暂停低氧指数（5.24 ± 4.50，P = 0.02）和喉视图（1.59 ± 1.00，P = 0.01）有统计学显著变化。
我们最近的经验使我们相信，sPRS 患者的发病率更高，临床发展更具挑战性，经过适当评估，MDO 可以治疗。MDO 有可能减少 iPRS 和 sPRS 患者因呼吸和喂养问题而需要传统手术干预的机会。
