Sleep in Pierre Robin Syndrome

doi:10.1378/chest.90.5.711

Chest

Volume 90, Issue 5, November 1986, Pages 711-715

https://doi.org/10.1378/chest.90.5.711 Get rights and content

Eight patients (aged 8 to 22 years) with the Pierre Robin syndrome underwent sleep studies. Seven demonstrated significant although minor degrees of increased sleep disturbances and apneas, and less time spent in the rapid-eye-movement (REM) stage of sleep. One patient who had previously undergone mandibular corrective surgery had major sleep abnormalities (central apnea index of 81.7 although an obstructive sleep apnea index of only 1.9). The patients had small mandibles, as demonstrated by lateral cephalometric roentgenography, and mildly increased right ventricular diastolic dimensions, as shown by M-mode echocardiography. Snoring was present in all of these patients and in 13 of 22 patients questioned from our Pierre Robin clinic. We conclude that minor abnormalities in sleep, mandibular size, and right ventricular size persist well into adolescence in the majority of patients with Pierre Robin syndrome. These appear to be clinically insignificant; however, a small percentage of such patients may continue to have major sleep disturbances.

Section snippets

Selection of Patients

Thirty-six consecutive patients with micrognathia, glossoptosis, cleft palate, and episodes of upper respiratory obstruction as neonates who were born between 1961 and 1974 inclusive were mailed questionnaires. They were questioned with regard to symptoms of obstructive sleep apnea,¹⁰ as to whether or not they snored, slept restlessly, performed poorly in school, had excessive daytime somnolence, excessive mood changes, or enuresis. Twenty of the 36 replied, and 13 of them reported chronic

Awake Studies

All patients had normal findings on physical, cardiovascular, and chest roentgenographic examinations. Electrocardiographic data were all within normal limits. The six measurable right-sided systolic time intervals were normal (mean, 0.26; range, 0.20 to 0.30). Right ventricular end-diastolic dimensions were all greater than the 50th percentile for weight (Fig 2). The chance of this occurring randomly is p<0.01. Five of the patients (as well as patient A) had values greater than two standard

DISCUSSION

Our patients, all of whom were chronic snorers, suffered minor degrees of sleep disturbances (a higher sleep disordered breathing index, more movement arousals and sleep stage changes, less time spent in REM sleep, and minor degrees of arterial oxygen desaturation compared to our normal subjects).

The most obvious cause for an increased incidence of such sleep disturbances in patients with the Pierre Robin syndrome is anatomic. Rivlin et al⁹ negatively correlated the apnea index with overall

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Polysomnography is considered the gold standard tool to establish the diagnosis and severity of obstructive sleep apnea (OSA) syndrome in children; however, it cannot provide information regarding the mechanism or location of upper airway obstruction during sleep. The present chapter will focus on the role of imaging modalities in evaluating childhood OSA and how they may advance our understanding of the anatomical and functional aspects of the disorder. Although routine imaging for children with OSA is not required, advanced imaging techniques are an important component of the evaluation for some children, including those with complex medical comorbidities, craniofacial anomalies, and persistent disease following first-line therapy. In this chapter, we emphasize studies using magnetic resonance imaging, since this technique is noninvasive and provides the most quantitative and reliable data without ionizing radiation. Lateral neck radiography, sleep fluoroscopy, computed tomography, and optical coherence tomography are also discussed.
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Cardinal features include significant airway obstruction in the first few weeks of life warranting airway management. Severe airway obstruction can cause RV enlargement and dysfunction.129 CV abnormalities include CHD (40% of individuals), in particular conotruncal malformations (interrupted aortic arch [50%], persistent truncus arteriosus [34%], tetralogy of Fallot, and ventricular septal defect).
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Children with craniofacial syndromes are at risk of sleep disordered breathing, the most common being obstructive sleep apnea. Midface hypoplasia in children with craniosynostosis and glossoptosis in children with Pierre Robin syndrome are well recognized risk factors, but the etiology is often multifactorial and many children have multilevel airway obstruction.
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This cohort study in 35 patients (13 children) evaluates the prevalence, severity and anatomical cause of obstructive sleep apnoea syndrome (OSAS) in patients with Treacher Collins syndrome. Ambulatory polysomnography was performed cross-sectionally to determine OSAS prevalence and severity. All upper airway related surgical interventions were evaluated retrospectively. In 11 patients, sleep endoscopy, and flexible and rigid endoscopy were applied to determine the level of anatomical obstruction of the upper airway. The overall prevalence of OSAS in Treacher Collins patients was 46% (54% in children; 41% in adults). Thirty-eight upper airway related surgical interventions were performed in 17 patients. Examination of the upper airway revealed various anatomical levels of obstruction, from the nasal septum to the trachea. Most significant obstruction was found at the level of the oro/hypopharynx. OSAS in Treacher Collins patients is an important problem so all patients should be screened for OSAS by polysomnography. Endoscopy of the upper airways was helpful in determining the level of obstruction. Surgical treatment at one level will not resolve OSAS in most patients because OSAS in Treacher Collins has a multilevel origin. Non-invasive ventilation (continuous positive airway pressure or bilevel positive airway pressure) or tracheotomy should be considered as a treatment modality.

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Chest

Clinical InvestigationsSleep in Pierre Robin Syndrome

Section snippets

Selection of Patients

Awake Studies

DISCUSSION

Am J Orthod

J Pediatr Surg

Lancet

Chest

La chute de la base de la langue considérée comme une nouvelle cause de gêne dans la respiration naso-pharyngienne

Bull Acad Med

Pierre Robin syndrome. Cause of respiratory obstruction, cor pulmonale and pulmonary edema

Am J Dis Child

Cor pulmonale and the Pierre Robin anomaly

Anaesthesia

Cor pulmonale in the Pierre Robin syndrome

Arch Dis Child

The Pierre Robin syndrome

Pediatrics

Glossoptosis revisited: on the development and resolution of airway obstruction in the Pierre Robin syndrome

Pediatrics

Growth of mandible in infants with micrognathia

Am J Dis Child

Devenir éloigné de quelques cas du syndrome de Robin du nouveau-né

Revue Stomatol

Upper airway morphology in patients with obstructive sleep apnea

Am Rev Respir Dis

Sleep disorders

Normal ECG standards for infants and children

Pediatr Cardiol

Clinical Investigations
Sleep in Pierre Robin Syndrome