Tracheal Resection for Critical Airway Obstruction in Morquio A Syndrome.

ABSTRACT:

Introduction

The primary cause of death in Morquio A syndrome (mucopolysaccharidosis (MPS) IVA) is airway obstruction, brought about by an inexorable and pathognomonic multilevel airway tortuosity, buckling, and obstruction. The relative pathophysiological contributions of an inherent cartilage processing defect versus a mismatch in longitudinal growth between the trachea and the thoracic cage are currently a subject of debate. Enzyme replacement therapy (ERT) and multidisciplinary management continue to improve life expectancy for Morquio A patients by slowing many of the multisystem pathological consequences of the disease but are not as effective at reversing established pathology. An urgent need has developed to consider alternatives to palliation of progressive tracheal obstru

SUBMITTER: Frauenfelder C 

PROVIDER: S-EPMC10171972 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature