Projects

ntroduction:

Distal ureteral atresia is a rare urinary tract anomaly generally associated with ipsilateral renal dysplasia and abnormalities such as multicystic dysplastic kidney, hydronephrosis and megaureter in the contralateral kidney. Despite burgeoning investigation modalities, definitive preoperative diagnosis of this condition is rarely feasible, also the embryological disarray of events that result in the development of this anomaly and the associated malformations is not clearly understood.

Case presentation:

We hereby report two cases of distal ureteral atresia and discuss the diversity in their presentations, diagnosis, atypical associations and management and review the possible embryological mal-development.

Conclusion:

Distal ureteral atresia with urogenital sinus as in Case 1 has not been documented so far and a plausible embryological explanation is deduced regarding its occurrence. The course of the affected kidney following timely and adequate relief of obstruction in Case 2 is depicted, highlighting the eventual management.Case reports

Case 1

A two-year-old girl with an unremarkable perinatal history presented with abdominal distention, bilious vomiting and anuria during the last day and mild recurrent fever during the last year. She was malnourished, febrile, dehydrated, tachypnoeic and acidotic. The abdomen was grossly distended; an abdomino-pelvic mass occupied the left quadrant separate from the distended urinary bladder (Figure 1A). The perineum had a single vestibular orifice and a normally placed anus. Bladder catheterization through the vestibular orifice drained around 40 ml of turbid urine but the mass persisted. Blood investigations revealed anemia, leukocytosis, hyponatremia and marked acidosis. Blood urea and serum creatinine were 127 and 3.1mg/dl respectively. Ultrasonography showed right hydro-ureteronephrosis and an abdomino-pelvic multicystic lesion extending from the left renal fossa into the pelvis; a normal kidney on the left side was not identified.

With a provisional diagnosis of hydrometrocolpos secondary to urogenital sinus and obstructive uropathy, a cystogenitoscopy and tube vaginostomy was planned. Cystoscopy revealed a common urogenital channel of 2.5 cm, with mildly trabeculated bladder, normal right ureteric orifice but absent left hemitrigone and ureteric orifice. At laparotomy, the bladder was extrinsically compressed by a grossly dilated and tortuous blind ending left ureter without any communication with the bladder; the left kidney was just a nubbin (Figure 1B- a,b). The right ureter was moderately dilated; internal genitalia were normal. A left nephroureterectomy was done and the child had an uneventful recovery. A subsequent voiding cystourethrogram (VCUG) showed grade V vesicoureteral reflux on the right side and a significant post void residue. Dimercaptosuccinic acid showed solitary right kidney with scars. She was started on clean intermittent catheterization. Serum creatinine at discharge was 0.6 mg%. At one-year follow-up the child is thriving, on chemoprophylaxis and awaiting definitive urogenital sinus (UGS) reconstruction. Case 2

An asymptomatic 4.1 kg, four-month-old girl born by Caesarean section at term with antenatally detected left hydroureteronephrosis was evaluated. Post-natal scan confirmed hydroureteronephrosis. VCUG showed no reflux. Diuretic renogram revealed left vesicoureteric junction obstruction (Figure 2a) with split renal function of 27%, serum creatinine was 0.6 mg%. In view of the age, small bladder capacity and grossly dilated ureter, a decision for diversion was taken. At surgery, the left ureter was atretic with only a 3 cm fibrous cord communicating with the bladder (Figure 2b). Proximally it was grossly dilated and tortuous. Atretic distal segment was excised, and a cutaneous end ureterostomy fashioned.

At follow-up, left hydroureteronephrosis progressively decreased to mild pelviectasia. However, the left kidney remained small with renal function deteriorating from 27% to 8% and a moderate decrease in the urine output per ureterostomy. Considering a probable stomal stenosis, ureterostomy was revised. Over the next year no significant improvement was noted either in the urine output or in the function, hence left nephroureterectomy was done. The child is now six years old, well, with solitary functioning right kidney