원발성 폐색성 거대요관: 임상관찰 13례

Abstract

Clinicopathological features were evaluated retrospectively for 17 ureters in 13 patients with primary obstructed megaureter. The mean age of patients was 8 years. Nine patients had unilateral and 4 had bilateral entity Clinical presentations included febrile UTI and flank pain in 5 patients respectively, and prenatally detected hydroureteronephrosis, abdominal mass and hematuria in each patient. Associated abnormalities of upper urinary system were found in 4 patients: UPJ obstruction with hypoplastic kidney, contralateral renal agenesis, contralateral hypoplastic kidney, and contralateral multicystic dysplastic kidney. Excision of obstructed segment and reimplantation with or without tapering was performed in 16 ureters. Also, correction of UPJ obstruction and nephrectomy for MCDK were needed. Thirteen ureters in 10 patients showed structural urographic improvement, but 3 ureters in 2 patients were not changed at mean follow up of 25 months. Reflux was seen postoperatively in 1 patient, which resulted in repeated reimplantation. Histopathologic examination of resected distal narrowed ureters showed ureteral muscular dysplasia, dense collagen infiltration and circular muscular hypertrophy in all specimens. These changes were seen not only in distal ureteral segment but also in upper dilated segment, and seemed to be more severe in patients who had associated upper tract problems. Primary obstructed megaureter showed various clinicopathological features and was frequently associated with ipsilateral or contralateral upper tract anomalies. Early meticulous imaging and surgical correction will lead to excellent result. Key word : Primary obstructed megaureter.