A pragmatic approach to infants with Robin sequence: a retrospective cohort study and presence of a treatment
Emma C Paes1,2, Daan P F van Nunen3, Lucienne Speleman4
1Department of Plastic, Reconstructive and Hand Surgery, Wilhelmina Children's Hospital, Utrecht, The Netherlands. emmapaes@gmail.com.
Insights
Robin sequence (RS) management varies, necessitating a multidisciplinary approach. This study offers a pragmatic institutional strategy for analyzing and treating infants with RS, aiming to guide clinical practice.
Area of Science:
- Craniofacial anomalies
- Pediatric surgery
- Neonatal care
Background:
- Robin sequence (RS) presents diverse clinical scenarios, complicating treatment decisions.
- Infant care for RS involves multiple specialties, leading to inconsistent management approaches.
- Optimizing RS infant care requires a standardized institutional strategy and literature review.
Purpose of the Study:
- To present an institution's comprehensive approach to Robin sequence (RS) management.
- To review current literature on the diagnosis and treatment of infants with RS.
- To provide guidance for optimizing the care of infants with RS.
Main Methods:
- Retrospective cohort study of 75 infants with RS (1996-2012).
- Analysis of institutional treatment regimens.
- Literature review of 31 studies on RS management.
Main Results:
- 59% of infants received conservative treatment; nonisolated RS cases more frequently required surgery (53% vs. 25%).
- Surgical interventions included mandibular distraction (24%), tracheotomy (9%), and tongue-lip adhesion (8%).
- 77% of infants received nasogastric tube feeding; literature review revealed variability in examination and surgical indications.
Conclusions:
- Robin sequence is a heterogeneous condition requiring a multidisciplinary approach due to its wide spectrum of anomalies.
- Current treatment options for RS vary significantly in the literature, highlighting the need for more uniform guidelines.
- The presented institutional approach offers a pragmatic framework for RS analysis and treatment, potentially serving as clinical guidance.
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.
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