Вариабельность течения синдрома сливового живота (Prune belly syndrome) в практике врача-неонатолога и детского хирурга

Abstract

Prune belly syndrome (PBS) is a rare congenital disorder classically defined by the presence of urinary tract anomalies, hypoplasia of the abdominal wall muscles, and bilateral cryptorchidism. In addition to the above-mentioned pathological conditions, other may also be present, creating greater variability in clinical presentation of the disease. There are also phenotypic variants of PBS, which are not manifested by all the distinguishing symptoms. The wide variability of the syndrome is due to the variety of genetic mutations and chromosomal abnormalities that lead to its development. Surgical treatment is required both for correction of underlying pathologies and for concomitant or emergent conditions. PBS is diagnosed in the prenatal period and, if pregnancy is prolonged, determines the scope of diagnostic and therapeutic measures in neonatal units. This article presents two clinical cases of PBS. The condition of patients allowed refraining from renal replacement therapy. Nephrostomy and ureterocutaneostomy helped to improve the condition. One child underwent surgical correction of chylous ascites. Key words: prune belly syndrome, megacystis, megaureter, cryptorchidism, abdominal muscle hypoplasia, wrinkled abdominal wall appearance, renal replacement therapy