Unravelling Robin sequence

Considerations of diagnosis and treatment

Authors

  • Manouk J. S. van Lieshout MD,

    Corresponding author
    1. Department of Oral and Maxillofacial Surgery, Dutch Craniofacial Center, Erasmus Medical Center, Rotterdam, the Netherlands
    • Send correspondence to Manouk J. S. van Lieshout, Room D-234, Department of Oral and Maxillofacial Surgery, Erasmus MC, 3000 CA Rotterdam, the Netherlands. E-mail: m.vanlieshout@erasmusmc.nl

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  • Koen F. M. Joosten MD, PhD,

    1. Department of Pediatrics, Dutch Craniofacial Center, Erasmus Medical Center, Rotterdam, the Netherlands
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  • Hans L. J. Hoeve MD, PhD,

    1. Department of Otorhinolaryngology–Head and Neck Surgery, Dutch Craniofacial Center, Erasmus Medical Center, Rotterdam, the Netherlands
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  • Irene M. J. Mathijssen MD, PhD,

    1. Department of Plastic, Reconstructive and Hand Surgery, Dutch Craniofacial Center, Erasmus Medical Center, Rotterdam, the Netherlands
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  • Maarten J. Koudstaal MD, DDS, PhD,

    1. Department of Oral and Maxillofacial Surgery, Dutch Craniofacial Center, Erasmus Medical Center, Rotterdam, the Netherlands
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  • Eppo B. Wolvius MD, DDS, PhD

    1. Department of Oral and Maxillofacial Surgery, Dutch Craniofacial Center, Erasmus Medical Center, Rotterdam, the Netherlands
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  • The authors have no funding, financial relationships, or conflicts of interest to disclose.

Abstract

Objectives/Hypothesis

The airway management of children with Robin sequence is controversial. This study provides an overview of a single-center experience with the diagnosis and treatment of 59 children with Robin sequence.

Study Design

Retrospective cohort study.

Methods

We conducted a retrospective cohort study of 59 children (<1 year old) with Robin sequence managed between 2000 and 2010. Robin sequence was defined as the presence of mandibular hypoplasia and clinical signs of airway obstruction. Data were collected on demographic characteristics, the presence of a syndrome, the perinatal period, and the management of airway and nutritional problems.

Results

Eighteen children (31%) needed respiratory support because of severe respiratory distress, and a sleep study found obstructive apneas in another eight children who had been managed by prone positioning and/or monitoring. In the isolated group significantly fewer children needed respiratory support compared to the nonisolated group. After the age of 1 year, 10% of the Robin sequence cohort was still in need of treatment for obstructive symptoms. Almost half (47%) needed temporary nutritional support.

Conclusions

The prevalence of respiratory distress in children with Robin sequence is high. In most children, treatment with prone positioning was sufficient to relieve the airway obstruction. Successful treatment with prone positioning was significantly more often seen in children with an isolated Robin sequence. About one-third of all Robin sequence children needed respiratory support in the neonatal and/or infant period. However, in childhood, only 10% of the total Robin sequence cohort was still dependent on respiratory support.

Level of Evidence

4 Laryngoscope, 124:E203–E209, 2014

Ancillary