Extrophy-Epispadias Complex
Pronunciation: EX-troh-fee ep-ih-SPAY-dee-us
A spectrum of congenital genitourinary malformations ranging from isolated epispadias to complete bladder extrophy requiring staged reconstructive procedures.
Full Definition
The Extrophy-Epispadias Complex represents a spectrum of rare congenital malformations affecting the genitourinary system and anterior abdominal wall. The spectrum includes isolated epispadias, classic bladder extrophy, and cloacal extrophy. Classic bladder extrophy involves exposure of the bladder mucosa through an abdominal wall defect, associated with epispadias and pubic diastasis. Surgical management typically involves staged reconstruction, beginning with bladder closure in the neonatal period, followed by epispadias repair and eventual bladder neck reconstruction. The complexity requires specialized pediatric urology and reconstructive surgery expertise.
Usage
Usage note: Use hyphenated form; distinguish from isolated epispadias in documentation.
In Context
- "The infant with extrophy-epispadias complex underwent primary bladder closure within 48 hours of birth." — Surgical operative note
- "Long-term outcomes in extrophy-epispadias complex depend on successful staged reconstruction." — Research publication