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Mortality in Robin sequence: identification of risk factors
Robrecht J H Logjes1, Maartje Haasnoot2, Petra M A Lemmers2
1Department of Plastic and Reconstructive Surgery, University Medical Centre Utrecht, Wilhelmina Children's Hospital, Utrecht, The Netherlands. r.j.h.logjes-2@umcutrecht.nl.
European Journal of Pediatrics
|March 2, 2018
Summary
Robin sequence (RS) mortality is 10%, with syndromic RS and neurological anomalies being significant risk factors. Early multidisciplinary evaluation, including genetic testing, is crucial for identifying infants at higher risk.
Area of Science:
- Pediatrics
- Genetics
- Neonatology
Background:
- Robin sequence (RS) is a recognized condition associated with significant morbidity and mortality.
- Existing research indicates variable mortality rates for RS, ranging from 2% to 26%.
- Current clinical focus often emphasizes respiratory complications, potentially overlooking other critical risk factors.
Purpose of the Study:
- To investigate the mortality rate in infants with Robin sequence.
- To identify specific risk factors associated with mortality in this patient population.
Main Methods:
- Retrospective review of 103 infants with Robin sequence at Wilhelmina Children's Hospital (1995-2016).
- Analysis of mortality data, causes of death, and associated conditions.
- Statistical analysis to determine risk factors, including syndromic status and neurological anomalies.
Main Results:
- A 10% mortality rate was observed in the cohort (10 out of 103 infants).
- Mortality was significantly higher in infants with syndromic RS (15%) compared to isolated RS (2%).
- Neurological anomalies were strongly associated with increased mortality (40% vs. 7%), with an odds ratio of 8.3.
Conclusions:
- Robin sequence is a heterogeneous condition often linked to underlying syndromes.
- Syndromic RS and the presence of neurological anomalies are key predictors of increased mortality.
- A comprehensive, multidisciplinary approach including genetic evaluation and standardized neurological assessment is vital for risk stratification in infants with RS.
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