A pragmatic approach to infants with Robin sequence: a retrospective cohort study and presence of a treatment algorithm

Abstract

Objectives: 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. Material and methods: 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. Results: 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. Conclusions: 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. Clinical Relevance: 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.