DOI: 10.36347/sjmcr.2022.v10i10.001

Pages: 992-995

Prune Belly syndrome (SPB) is an extremely rare and complex anatomo-radiological entity with a clear male predominance, characterized by the triad made up of aplasia or hypoplasia of the muscles of the anterior wall of the abdomen, dilatations of the urinary tract and bilateral cryptorchidism. This condition is associated in 75% of cases with pulmonary, skeletal, cardiac and gastrointestinal malformations. There are also so-called incomplete or partial forms that are more common in females and are known as Pseudo-Prune Belly Syndrome. The evolution of this entity is characterized by several complications and the prognosis depends mainly on the severity of the renal damage as well as the presence of pulmonary hypoplasia. Imaging confirms the observed abnormalities. We report the case of a 4-year-old male child presenting with sudden onset abdominal distension evolving for 7 days prior to admission in which the clinical examination found a crumpled and loose wrinkle of the abdominal wall more marked on the right. Abdominal wall muscle hypoplasia was confirmed by abdominal ultrasound and computed tomography.