Congenital giant megaureter associated with ipsilateral multicystic dysplastic kidney in newborn

Abstract

Congenital giant megaureter presents as abdominal mass and impose diagnostic difficulties. It can be associated with other upper urinary tract anomalies. A female newborn with antenatal diagnosis of polycystic kidneys was admitted at birth due to lower abdominal mass. Ultrasound and CT scans diagnosed a multiloculated cystic lesion in the mid and lower abdomen along with right side multicystic kidney. At laparotomy, an extaperitoneal, lobulated cystic swelling was found due to rightside giant megaureter. Its lower end was of normal caliber and orthotopic. End cutaneous ureterostomy was done. Intravenous urogram and isotope renograms showed nonfunctioning right kidney. She also had grade II vesicoureteral reflux on left side. Child suffered urinary infection twice. At 9m age, right nephroureterectomy was done. Histopathologic examination was consistent with cystic renal dysplasia and dilated ureter. This is the first case report of giant megaureter associated with ipsilateral multicystic dysplastic kidney in newborn.

Single System Ectopic Congenital Giant Megaureter Associated with Ipsilateral Multicystic Dysplastic Kidney

“…[3] The upper limit of normal size for non-obstructed ureter on unenhanced helical CT scan is 3 mm. [4] Hence, ureteral dilatation beyond 3cm is designated as giant megaureter.…”

“…The other described associated congenital anomalies are ureterocele, ectopic ureter, crossed ectopic ureter, ureteral atresia, supernumerary kidneys etc. [3,5] Multicystic dysplastic kidney (MCDK) is invariably associated with ipsilateral atretic ureter and most of these undergo involution within first 5 years of life. However, it can rarely occur in conjunction with ureteral dilatation and a ureterocele in both instances, the underlying mechanism is believed to be an obstructive insult to the developing metanephric mesenchyme at an early stage in gestation.…”