Prune Belly Syndrome and Tracheo-esophageal Fistula in a Premature Neonate.

Lance Potter,Charles J Rosser,S Iskandar,R Lawrence Kroovand

doi:10.1100/tsw.2004.78

Abstract

Prune Belly Syndrome, also known as Eagle-Barrett syndrome, is a well known triad of abdominal muscle deficiency or hypoplasia, urinary tract anomalies, and bilateral cryptorchidism. Tracheoesophageal fistula is a sporadically occurring defect estimated to occur in 1 out 4425 live births. Only one case of Prune Belly Syndrome, tracheo-esophageal fistula associated with VATER syndrome, and urethral atresia occurring in the same individual has been reported. We present a unique case involving a 33 week fetus with bladder distention and bilateral hydroureteronephrosis diagnosed in utero who postnatally was diagnosed with Prune Belly Syndrome, tracheo-esophageal fistula, and urethral atresia.

Highlights

Prune Belly Syndrome, known as Eagle-Barrett syndrome, is a well known triad of abdominal muscle deficiency or hypoplasia, urinary tract anomalies, and bilateral cryptorchidism.[1]
The Prune Belly Syndrome in females is a rarer occurrence than in males with an observed prevalence in males of 5/100,000 versus 1/100,000 in females. By definition this syndrome is only completely expressed in males(presence of cryptorchidism mandatory), three percent of the reported cases occur in genetic females and is generally known as Pseudo-Prune Belly Syndrome(PPBS).[4,5]
Our patient was found to have the typical phenotype of PPBS, lax abdominal musculature, large distended bladder, and urethral atresia as well as an unilateral absent ovarian structure, absent left fallopian tube and atretic vagina

Summary

Previously published in the Digital Urology Journal

Prune Belly Syndrome, known as Eagle-Barrett syndrome, is a well known triad of abdominal muscle deficiency or hypoplasia, urinary tract anomalies, and bilateral cryptorchidism.[1] Tracheoesophageal fistula is a sporadically occurring defect estimated to occur in 1 out 4425 live births.[2] Only one case of Prune Belly Syndrome, tracheo-esophageal fistula associated with VATER syndrome, and urethral atresia occurring in the same individual has been reported.[3] We present a unique case involving a 33 week fetus with bladder distention and bilateral hydroureteronephrosis diagnosed in utero who postnatally was diagnosed with Prune Belly Syndrome, tracheo-esophageal fistula, and urethral atresia

CASE REPORT

DISCUSSION