[Prune-belly syndrome and therapeutic problems].

UNLABELLED: Specific features of Prune-Belly syndrome (PBS) are deficiency of the abdominal muscles, urinary abnormalities and cryptorchidism. Diagnostics is possible prenatally. In order to inhibit kidney degradation process "in utero" intervention are performed.

THE AIM OF OUR STUDY: Presentation of diseases development and therapeutic problems in 4 boys with PBS.

RESULTS

In 3 cases this disease has been recognized before birth. In one case amniotic fluid has been supplemented. In other case abdominal-amniotic shunt has been introduced. Two children have been borne naturally, two children by cesarotomy in 31 weeks of gestation because there were intrauterine infection danger. After birth in all patients urinary infections and renal function have been observed. For three children surgical therapy has been applied. The following intervention: percutaneous nephrostomy (1 pt), cutaneocystostomy (2 pts), orchidopexy (1 pt), have been performed. One child died. Two children have renal disfunction (II degree of chronic renal disease).

CONCLUSIONS

Children with PBS need permanent interdisciplinary medical care. Multistage surgical therapy inhibits kidney degradation process and improves life quality.