Abstract
Congenital midureteral stricture (CMS) is a rare disease, but a detailed evaluation of the urinary tract is important for planning treatment strategies because about half of CMS patients present with other urinary tract abnormalities. In this report, we describe a case of functional solitary kidney with left polycystic dysplasia and right CMS. The patient developed acute postrenal insufficiency in the neonatal period and underwent surgical treatment in a stepwise fashion. The patient was a boy born at 38 weeks of gestation and diagnosed as having right hydronephrosis and hydroureter while still a fetus. At 7 days of age, the patient developed acute postrenal insufficiency and underwent percutaneous nephrostomy. The right CMS was found by postoperative pyelography, and a ureterocutaneous fistula was formed in the lower abdomen at 38 days of age to avoid problems with nephrostomy management. The patient underwent right vesicoureteral neoanastomosis in the same surgical incision at 9 months of age. In CMS, adequate attention should be paid to the possibility of the rapid deterioration of renal function in the neonatal period. Particularly in the case of a functional solitary kidney, surgical treatment should be planned at an early stage, with a view to long-term prognosis.
