Management and outcomes of obstructive sleep apnea in children with Robin sequence, a cross-sectional study

Manouk J.S Van Lieshout,Marc P Van Der Schroeff,Karolijn Dulfer,Eppo B Wolvius,Koen F.M Joosten,Maarten J Koudstaal,Irene M.J Mathijssen

doi:10.1007/s00784-016-1985-y

Manouk J.S Van Lieshout, Marc P Van Der Schroeff + Show 5 more

Open Access

https://doi.org/10.1007/s00784-016-1985-y

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Abstract

ObjectiveThe objective of this cross-sectional study is to assess the prevalence, course, and management of obstructive sleep apnea (OSA) in children with Robin sequence (RS) aged 1–18 years.Materials and methodsA cross-sectional study was conducted in 63 children aged 1 to18 years with RS. Patient data were collected on baseline characteristics and management. OSA was evaluated by polysomnography.ResultsSixty-three children with RS were included (median age 8.0 years) and divided into two groups based on the initial treatment: prone positioning or respiratory support. Respiratory support was more often indicated in children with a non-isolated RS (p < 0.05). At cross section, in the prone positioning group (n = 32), one child was diagnosed with OSA. In the respiratory support group (n = 31), 13 children (42 %) had respiratory problems of whom 10 needed respiratory support.ConclusionsBetween the age of 1 and 18 years, almost one out of four children with RS still has respiratory problems. Children with RS, who can be treated with prone positioning only as an infant, are not likely to develop obstructive airway problems at a later age. In contrast, children who need respiratory support early after birth are at risk of continuing or re-developing OSA after the age of 1 year.Clinical relevanceThis study shows that those who need respiratory support at an early age need careful monitoring until adulthood.

Highlights

Robin sequence (RS) is a congenital facial condition occurring in 1 in 5600 to 1 in 30,000 newborns [1,2,3,4,5]
Children with RS, who can be treated with prone positioning only as an infant, are not likely to develop obstructive airway problems at a later age
Children who need respiratory support early after birth are at risk of continuing or redeveloping obstructive sleep apnea (OSA) after the age of 1 year

Summary

Introduction

Robin sequence (RS) is a congenital facial condition occurring in 1 in 5600 to 1 in 30,000 newborns [1,2,3,4,5]. The condition is classically characterized by an underdeveloped mandible (mandibular hypoplasia), backward displacement of the tongue (glossoptosis), and airway obstruction. Children with RS are at risk of developing obstructive sleep apnea (OSA) [8]. OSA is characterized by prolonged partial upper airway obstruction and/or intermittent complete airway obstruction, disrupting the child’s sleeping pattern [9]. Leaving OSA untreated may result in serious morbid consequences on the cardiovascular system, the metabolic system, and neurocognitive and behavioral functioning [9]. To establish the presence of OSA, polysomnography (PSG) is currently considered the gold standard [10]

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