Critical Airway Stenosis in an Adolescent Male With Pompe Disease and Thoracic Lordosis: A Case Report.
- B Randall Brenn,
- Mary T Theroux,
- Suken A Shah,
- William G Mackenzie,
- Robert Heinle,
- Mena T Scavina
A A Case Rep 2017 May 23. Epub 2017 May 23.
From the Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware.
An adolescent male with late-onset Pompe disease (glycogen storage disease type II) presented with a history of restrictive airway disease and a near-cardiorespiratory arrest during anesthesia for a liver biopsy initially thought to be due to bronchospasm. During a subsequent posterior spinal fusion procedure, he suffered cardiorespiratory arrest resulting in the procedure being aborted. Bronchoscopy performed shortly after resuscitation revealed an undiagnosed narrowing of the distal trachea and bronchi. Read More