Prune belly syndrome: current perspectives

Abstract

Angela M Arlen,1 Cayce Nawaf,1 Andrew J Kirsch21Yale University School of Medicine, Department of Urology, New Haven, CT 06520, USA; 2Emory University, Children’s Healthcare of Atlanta, Atlanta, GA 30328, USAAbstract: Prune belly syndrome (PBS) is a rare but morbid congenital disease, classically defined by a triad of cardinal features that includes cryptorchidism, urinary tract dilation and laxity of the abdominal wall musculature. Children often require numerous surgical interventions including bilateral orchidopexy as well as individually tailored urinary tract and abdominal wall reconstruction. Along with the classic features, patients with PBS often experience gastrointestinal, orthopedic, and cardiopulmonary comorbidities.Keywords: prune-belly syndrome, cryptorchidism, urinary tract dilation, abdominal wall laxity, abdominoplasty

Keywords

• Prune belly syndrome
• cryptorchidism
• abdominal wall
• abdominoplasty
• hypotonia