[Prune-belly syndrome and therapeutic problems]. / Zespól suszonej sliwki--problemy terapeutyczne.

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.

[Megacalycosis as a diagnostic problem in children]. / Megacalicosis jako problem diagnostyczny u dzieci.

Megacalycosis is an uncommon, congenital renal abnormality, characterized by nonobstructive dilatation of the renal calyces with normal renal pelvis, ureter and bladder. This condition is due to underdevelopement or hypoplasia of Malpighie's pyramids. It usually occurs unilateraly as the isolated anomaly with strong male predominance. Megacalycosis itself does not impair the renal function but it can be the cause of urinary tract infections and calculus formation. This congenital defect is incidentally found by urography during the examination for urolithiasis or urinary tract infections or calculus formation. The images on radograms can be confused with obstructive or refluxing hydronephrosis or postinflamtory changes typical for pyelonephritis chronica. In case of megacalycosis surgical treatment is unnecessary. Patients with this condition should be followed-up with ultrasound and prevention of urinary tract infections or urolithiasis. Here we would like to report on the case of megacalycosis in a ten-year-old girl reffered to our deprtament due to UTI. Diagnosis of megacalycosis was established by typical urography findings: dilatation of renal calyces, no distention or obstruction of renal pelvic and ureter. The renal function was normal. No evidence of abnormality in cystoureterogram and uroflowmetry test was detected.