Современные подходы к диагностике и ведению больных с синдромом сливового живота (Prune belly syndrome)

Abstract

Prune belly syndrome (PBS) is a congenital disorder that is characterized by hypoplasia (aplasia) of the anterior abdominal wall muscles, various urinary tract anomalies, and bilateral cryptorchidism. The prevalence of PBS is approximately 1:40,000-1:45,000, and it is commonly diagnosed in male neonates. The possible genetic determinism of the disease is explained by the identification of 14 mutations in live-born children with PBS. Fetal ultrasound allows the diagnosis of PBS at 12 weeks’ gestation. In prolongation of pregnancy, along with specialized care and intensive care in neonatal units, renal replacement therapy, children with PBS need correction of diagnosed malformations and concomitant surgical pathology. This article presents current features of a multidisciplinary approach to the diagnosis and management of patients with this pathology to timely correct the condition and improve long-term outcomes. Key words: prune belly syndrome, megacystis, megaureter, cryptorchidism, abdominal muscle hypoplasia, wrinkled abdominal wall, renal replacement therapy