Antenatal Diagnosis of Prune Belly Syndrome

Abstract

AbstractPrune belly syndrome (PBS) is a rare congenital abnormality characterized by classical triad of urinary tract abnormalities, deficient abdominal musculature, and bilateral cryptorchidism. We present a prenatally diagnosed case of PBS identified at 12 weeks. Antenatal ultrasound study showed fetal bladder and abdomen to be enormously enlarged filling up whole of fetal abdomen with bilateral clubfeet. Gross examination showed very thin parchment-like defective anterior abdominal wall with protrusion of the abdominal contents. Microscopic examination showed hypoplasia of the abdominal muscle with overlying thin layer of epidermis. The histopathology examination of the abdominal wall muscles demonstrates a pattern of developmental arrest rather than one of atrophy consequent to early and severe obstruction. There is also absence of aponeurotic layer. Antenatal suspicion of PBS was corroborated on postnatal gross examination of the fetus and further confirmed on histopathological examination of the fetus.