Vital Airway Stenosis in an Adolescent Male With Pompe Illness and Thoracic Lordosis: A Case Report.
A A Case Rep. 2017 Oct 01;9(7):199-203
Authors: Brenn BR, Theroux MT, Shah SA, Mackenzie WG, Heinle R, Scavina MT
An adolescent male with late-onset Pompe illness (glycogen storage illness kind II) offered with a historical past of restrictive airway illness and a near-cardiorespiratory arrest throughout anesthesia for a liver biopsy initially considered resulting from bronchospasm. Throughout a subsequent posterior spinal fusion process, he suffered cardiorespiratory arrest ensuing within the process being aborted. Bronchoscopy carried out shortly after resuscitation revealed an undiagnosed narrowing of the distal trachea and bronchi. That is the primary description of a affected person with late-onset Pompe illness with undiagnosed important tracheal stenosis as a result of development of thoracic lordosis, which was in the end relieved by posterior spinal fusion.
PMID: 28542051 [PubMed – indexed for MEDLINE]