Successful surgical repair of a massive window duct in a 1-month old with aniridia and pulmonary interstitial glycogenosis

Abstract

The window duct is a rare congenital anomaly that is physiologically similar to an aortopulmonary window but is extrapericardial at the distal pulmonary trunk. The diagnosis is challenging, and surgical management is complex. Our patient is the first and the youngest to be reported with successful closure and diagnosed by magnetic resonance imaging.

Pulmonary interstitial glycogenosis – A systematic analysis of new cases

“…Thus the demonstration of PIG on histology may be a general marker of developmental dysmaturity of the pulmonary interstitial compartment and not an entity in itself. Among the associated conditions, congenital heart defects and pulmonary hypertension were most common [11,13,16,[25][26][27][28][29]. More importantly from a clinical point of view, mucopolysaccharidoses (MPS) were overrepresented in our cohort with two out of eleven children.…”