A Rare Presentation of Prune Belly Syndrome in a Female Neonate: Case report and review of literature

Abstract

Prune Belly syndrome (PBS) is a rare congenital anomaly of uncertain etiology almost exclusive to males. There are variants of Prune Belly syndrome which are characterised by partial or unilateral hypoplasia of the abdominal wall muscles and may be associated with other malformations. These variants are described as incomplete or Pseudo Prune-Belly syndromes (PPBS). We report and discuss the case of a preterm female newborn, in whom clinical examination revealed deficient abdominal muscle, and she also had a soft systolic ejection murmur loudest over the pulmonic area. Abdominal ultrasound scan demonstrated no urinary tract anomalies; the presence of atria septal defect was confirmed on echocardiogram, and she had normal kidney function test results. She was referred to the paediatric surgical department and had follow-up cardiologist visits.

Keywords: Pseudo prune belly syndrome; Abdominal wall laxity; Atria Septal Defect; Female; Neonate