Is there a correlation between sleep disordered breathing and foramen magnum stenosis in children with achondroplasia?

Children with achondroplasia have midface hypoplasia, frontal bossing, spinal stenosis, rhizomelia, and a small foramen magnum. Central sleep apnea, with potential resultant sudden death, is thought to be related to compression of the spinal cord at the cervicomedullary junction in these patients. Screening polysomnography and/or cervical spine MRI are often performed for infants with achondroplasia.

Quantitative computed tomographic scan and polysomnographic analysis of patients with syndromic midface hypoplasia before and after Le Fort III distraction advancement.

Background: Distraction advancement has been advocated for treatment of obstructive sleep apnea associated with congenital midface hypoplasia. The purpose of this study was to relate changes in maxillary position to changes in obstructive sleep apnea measures on polysomnography in a consecutive series of patients.

Methods: Among 26 syndromic pediatric patients undergoing Le Fort III distraction over a 5-year period, 15 had documented obstructive sleep apnea with an apnea hypopnea index greater than 5.

Sleep-disordered breathing in children with thoracic insufficiency syndrome.

Thoracic insufficiency syndrome (TIS) is a collection of chest and spine malformations that results in progressively restrictive pulmonary mechanics and an inability of the thorax to adequately support lung growth. Many children with TIS are too young to perform standard pulmonary function tests, yet need functional assessments of their restrictive thoracic disease. We report on the sleep architecture and frequency of sleep-related breathing abnormalities in 11 children with TIS who underwent overnight polysomnography from retrospective chart review.

Sleep outcomes in children with hemifacial microsomia and controls: a follow-up study.

Objective: Children with craniofacial anomalies are at high risk for sleep-disordered breathing (SDB), yet its prevalence among children with craniofacial conditions is not known. Children with hemifacial microsomia (HFM) are likely particularly vulnerable to SDB as a result of underdevelopment of the mandible and oropharynx. Nevertheless, most children with HFM are not referred for sleep studies.

Sleep-related breathing disorder in children with vagal nerve stimulators.

The effects of vagal nerve stimulation on sleep-related breathing have not been well-described in children. Vagal nerve stimulation was reported to cause decreases in airflow during sleep, although most studies reported this condition to be clinically insignificant. We present a retrospective case series of nine children who underwent polysomnography after vagal nerve-stimulator placement.

Sleep disturbances in 22q11.2 deletion syndrome: a case with obstructive and central sleep apnea.

The 22q11.2 deletion syndrome is characterized by wide phenotypic variability, frequently involving characteristic craniofacial features, cardiac malformations, and learning difficulties. Skeletal anomalies are also common and include an obtuse angle of the cranial base, retrognathia, and cervical spine abnormalities.

Response to nonprescription epinephrine inhaler during nocturnal asthma.

Background: Many health care professionals believe that a nonprescription epinephrine metered-dose inhaler is less effective and shorter acting and has more cardiovascular adverse effects than prescription beta2-agonists.

Objective: To determine if increasing the epinephrine dose improves efficacy safely.

Methods: Eight patients with nocturnal asthma (age range, 20-46 years) were treated in a randomized, crossover manner on 2 different nights while sleeping in a clinical research center.