The phenotypic and pathological features of prune-belly syndrome


ŞAHİN D. , Çetiner H., GÜVEN Ş.

TURKISH JOURNAL OF PATHOLOGY, vol.26, no.2, pp.114-119, 2010 (Journal Indexed in ESCI) identifier identifier

  • Publication Type: Article / Article
  • Volume: 26 Issue: 2
  • Publication Date: 2010
  • Doi Number: 10.5146/tjpath.2010.01007
  • Title of Journal : TURKISH JOURNAL OF PATHOLOGY
  • Page Numbers: pp.114-119
  • Keywords: Prune-belly syndrome, Uretral obstruction, Urinary tract anomalies, Fetus, Fetal pathology

Abstract

Objective: Prune-belly syndrome is a rare congenital disorder characterized by musculature deficiency in the abdominal wall, lower urinary tract obstruction, other urinary tract anomalies, and bilateral cryptorchidism. The syndrome is commonly associated with pulmonary, skeletal, cardiac, and gastrointestinal defects. Over 95% of patients are male. Urinary tract disease is the major prognostic factor with the complications of pulmonary hypoplasia and end stage renal disease. The aim of this study was to determine phenotypic and pathologicalfeatures of fetuses with this syndrome.