Critical Airway Stenosis in an Adolescent Male With Pompe Disease and Thoracic Lordosis: A Case Report.
A A Case Rep
; 9(7): 199-203, 2017 Oct 01.
Article
in En
| MEDLINE
| ID: mdl-28542051
ABSTRACT
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. This is the first description of a patient with late-onset Pompe disease with undiagnosed critical tracheal stenosis due to the progression of thoracic lordosis, which was ultimately relieved by posterior spinal fusion.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Tracheal Stenosis
/
Glycogen Storage Disease Type II
/
Lordosis
Limits:
Adolescent
/
Humans
/
Male
Language:
En
Journal:
A A Case Rep
Year:
2017
Document type:
Article