Paes Emma C, van Nunen Daan P F, Speleman Lucienne, Muradin Marvick S M, Smarius Bram, Kon Moshe, Mink van der Molen Aebele B, Niers Titia L E M, Veldhoen Esther S, Breugem Corstiaan C
Department of Plastic, Reconstructive and Hand Surgery, Wilhelmina Children's Hospital, Utrecht, The Netherlands.
Department of Pediatric Plastic Surgery, Wilhelmina Children's Hospital Utrecht, PO Box 85500, 3508 GA, Utrecht, The Netherlands.
Clin Oral Investig. 2015 Nov;19(8):2101-14. doi: 10.1007/s00784-015-1407-6. Epub 2015 Feb 15.
Initial approaches to and treatments of infants with Robin sequence (RS) is diverse and inconsistent. The care of these sometimes critically ill infants involves many different medical specialties, which can make the decision process complex and difficult. To optimize the care of infants with RS, we present our institution's approach and a review of the current literature.
A retrospective cohort study was conducted among 75 infants diagnosed with RS and managed at our institution in the 1996-2012 period. Additionally, the conducted treatment regimen in this paper was discussed with recent literature describing the approach of infants with RS.
Forty-four infants (59%) were found to have been treated conservatively. A significant larger proportion of nonisolated RS infants than isolated RS infants needed surgical intervention (53 vs. 25%, p = .014). A mandibular distraction was conducted in 24% (n = 18) of cases, a tracheotomy in 9% (n = 7), and a tongue-lip adhesion in 8% (n = 6). Seventy-seven percent of all infants had received temporary nasogastric tube feeding. The literature review of 31 studies showed that initial examinations and the indications to perform a surgical intervention varied and were often not clearly described.
RS is a heterogenic group with a wide spectrum of associated anomalies. As a result, the decisional process is challenging, and a multidisciplinary approach to treatment is desirable. Current treatment options in literature vary, and a more uniform approach is recommended.
We provide a comprehensive and pragmatic approach to the analysis and treatment of infants with RS, which could serve as useful guidance in other clinics.
对于患有罗宾序列征（RS）的婴儿，最初的治疗方法和治疗手段多种多样且不一致。对这些有时病情危急的婴儿的护理涉及许多不同的医学专科，这可能使决策过程复杂且困难。为了优化对患有RS的婴儿的护理，我们介绍我们机构的方法并对当前文献进行综述。
对1996年至2012年期间在我们机构诊断为RS并接受治疗的75例婴儿进行了回顾性队列研究。此外，还结合近期描述RS婴儿治疗方法的文献对本文所采用的治疗方案进行了讨论。
发现44例婴儿（59%）接受了保守治疗。非孤立性RS婴儿比孤立性RS婴儿需要手术干预的比例显著更高（53%对25%，p = 0.014）。24%（n = 18）的病例进行了下颌骨牵张成骨术，9%（n = 7）的病例进行了气管切开术，8%（n = 6）的病例进行了舌唇粘连术。所有婴儿中有77%接受过临时鼻胃管喂养。对31项研究的文献综述表明，初始检查和进行手术干预的指征各不相同，且往往没有清晰描述。
RS是一组具有广泛相关异常的异质性疾病。因此，决策过程具有挑战性，需要多学科的治疗方法。文献中目前的治疗选择各不相同，建议采用更统一的方法。
我们提供了一种全面且实用的方法来分析和治疗患有RS的婴儿，这可为其他诊所提供有用的指导。
