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Tracheobronchomalacia in Hunter's syndrome
1Department of Otolaryngology-Head and Neck Surgery, United States Air Force Medical Center, Lackland Air Force Base, TX 78236-5300.
International Journal of Pediatric Otorhinolaryngology
|April 1, 1993
Summary
Hunter's syndrome, a metabolic disorder, often causes pulmonary issues. This study identifies tracheobronchomalacia with major airway collapse as a key cause of these airway problems in patients.
Area of Science:
- Biochemistry
- Genetics
- Pulmonology
Background:
- Hunter syndrome is a rare lysosomal storage disorder due to enzyme deficiencies, leading to mucopolysaccharide buildup.
- Associated pulmonary complications include airway obstruction, sleep apnea, and difficult intubation.
Observation:
- Laryngotracheobronchoscopy findings in a Hunter syndrome patient with airway symptoms were analyzed.
- Previously reported cases of airway issues in Hunter syndrome were reviewed.
Findings:
- Tracheobronchomalacia with major airway collapse (MAC) is proposed as the pathological correlate for airway symptoms.
- Endoscopic findings characteristic of tracheobronchomalacia/MAC were observed.
Implications:
- Understanding tracheobronchomalacia/MAC is crucial for managing airway complications in Hunter syndrome.
- This insight aids in diagnosing and treating respiratory distress in affected individuals.