(Objectives) Congenital midureteral stricture (CMS), which develops from obstructive lesion between pyeloureteral junction and ureterovesical junction, is relatively rare and its clinical condition and therapeutic strategy have not yet been established. We analyzed the clinical characteristics and surgical outcomes of CMS. (Patients and methods) From November 2006 to December 2012, out of 137 patients presented with congenital hy-drohephrosis, we identified 4 pediatric patients diagnosed with CMS at our institutions. We retrospectively investigated clinical characteristics and surgical outcomes in these 4 patients. (Results) Three boys and one girl were identified in this study. All patients were detected hydronephrosis by fetal ultrasonography. The median age at the diagnosis of CMS was 1 year and 11 months. Three patients had obstructive lesion in left side and 1 patient in right CMSs were located at the level of L4 in 2 patients, and at the level of L5 and SI in each 1. Split renal function was decreased less than 45% in 3 of 4 patients. Ipsilateral pyeloureteral junction obstruction and ipsilateral hypoplastic kidney were identified in 2 and 1 patient respectively. One patient developed urosepsis and underwent nephrostomy. Partial ureterectomy and ureteroureterostomy, pyeloplasty were performed in 3 and 1 patient respectively. Extrinsic obstruction was detected in just 1 patient intraoperatively. In all patients, there were no protruded lesion and atrophied, fibrotic and ischemic muscles were not detected in pathological finding. Neither urinary tract infection nor recurrence of obstructive lesion was detected in all patients at the mean follow-up period of 3 years and 1 month. (Conclusion) We analyzed the clinical characteristics of 4 pediatric patients with CMS. In order to prevent critical infection and maintain renal function, it could be considered that surgical intervention is undertaken just after making the diagnosis of CMS.
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