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Klippel-Feil sequence and sleep-disordered breathing in two children
C L Rosen1, E J Novotny, L A D'Andrea
1Department of Pediatrics (Sections of Respiratory Medicine and Neurology), Yale University School of Medicine, New Haven, Connecticut 06525.
The American Review of Respiratory Disease
|January 1, 1993
Summary
Children with Klippel-Feil sequence can develop severe sleep-disordered breathing due to associated hindbrain anomalies. Early recognition and evaluation are crucial for timely neurosurgical intervention and improved outcomes.
Area of Science:
- Neurology
- Pediatrics
- Sleep Medicine
Background:
- Klippel-Feil sequence is a congenital vertebral anomaly.
- Hindbrain anomalies can occur alongside Klippel-Feil sequence.
- Sleep-disordered breathing is a potential complication.
Observation:
- Two children with Klippel-Feil sequence presented with severe sleep-disordered breathing.
- Both patients had associated minor vertebral and major hindbrain anomalies.
- One child developed fatal obstructive sleep apnea; the other presented with bradypnea and stridor.
Findings:
- Undiagnosed hindbrain anomalies in Klippel-Feil sequence can lead to severe respiratory issues.
- Sleep-disordered breathing, including obstructive sleep apnea, bradypnea, and stridor, is a significant risk.
- Prompt diagnosis of CNS disorders is vital.
Implications:
- Recommend vigilant monitoring for sleep-disordered breathing in Klippel-Feil sequence patients.
- Emphasize prompt polysomnography for sleep complaints and neuroimaging for neurologic signs.
- Consider unsuspected CNS disorders in pediatric stridor or sleep respiratory disturbances.