Prune Belly syndrome in school patient

case report

Abstract

Prune Belly Syndrome (SPB) is a rare congenital disease (1: 40,000 live births) characterized by a triad of anomalies: absence, deficiency or hypoplasia of the abdominal wall, bilateral cryptorchidism, and urinary tract abnormalities. The diagnosis can be made even during prenatal care; however, it has a high perinatal mortality rate. Thus, the present study aimed to describe a case report about SPB in a school patient. VSSB, male, diagnosed with SPB. 2nd trimester US showed a polyhydramnio fetus with morphological alterations. At 38 weeks of gestation, cesarean section was performed due to evidence of severe oligoamnion and fetal malformation. At birth, plum abdomen and bilateral cryptorchidism were observed, suggesting SPB; Diagnostic suspicion was confirmed on the 13th day of life. The patient followed up with a multidisciplinary team. Due to the clinical condition, she required several hospitalizations throughout her life, dying in the last, at 7 years and 11 months. Therefore, it is undoubted to report the importance of quality prenatal care in the early diagnosis of congenital diseases and multidisciplinary follow-up. In addition, the longitudinality and comprehensiveness of care are crucial for increasing quality patient survival.

Keywords: Syndrome. Prune Belly. Case reports.