Publications
Duplex sans duplex: a cryptic cause”: a case report
S. Rajkiran Raju, Jobin Pathrose, Dyan D’Souza & Attibele Mahadevaiah Shubha Background
Duplex kidneys represent an embryologic maldevelopment at time of renogenesis resulting in a spectrum of bifurcation anomalies of the reno-ureteric system. Though most are antenatally detected, recurrent urinary tract infections (UTIs), abdominal mass due to obstruction and incontinence are other common manifestations. Upper moiety ureter is usually obstructed and the lower moiety is refluxing. Management is guided by the percentage function of each of the moieties. A non-functioning system warrants a heminephrectomy. We report a toddler with right flank mass and a provisional diagnosis of right duplex system following investigations but met with a cryptic cause at surgery thereby altering the management.
Case presentation
A 2 ½ years girl with progressively increasing right abdominal mass for 6 weeks was found to have 12 × 10 cm right non-tender flank mass. Ultrasonography, contrast tomography and nuclear scans showed a right duplex system with obstructed, poorly functioning lower moiety. A lower moiety heminephrectomy was planned but at surgery, a densely adherent cystic structure displacing the right kidney superiorly was noted. On decompressing, the ureter was found to enter the cyst with discontinuation for a length of 6cms before being traced distally to its entry into the bladder. Retrograde pyelogram confirmed mid-ureteral transection and cystic urinoma. The cyst was excised and the ureter reconstructed with an appendicular interposition graft. Child recovered uneventfully and at 8 months follow up is well with good drainage across the conduit.
Conclusion
The case highlights a rare presentation of mid-ureteral transection with urinoma masquerading as a duplex system and its satisfactory management. Duplex kidneys comprise a spectrum of bifurcation anomalies of the reno-ureteric system. They commonly present with recurrent urinary tract infections (UTIs), abdominal mass due to obstruction or incontinence. Post-traumatic isolated ureteral injuries are extremely rare in children, due to anatomic concealment in the retroperitoneum. Ureteral transections following blunt abdomen trauma result in urinomas, often manifesting acutely with fever and toxicity due to early infection or with an abdominal mass. Management of ureteral injuries depend on the level, extent and completeness of disruption. Complete ureteral transections mandate surgical restoration of continuity.
We herewith report a rare presentation of isolated right ureteral transection with large urinoma in a two and half-year-old girl following unnoticed trivial trauma masquerading as a duplex right kidney with lower moiety obstruction and highlight the challenges encountered in management. 2 Case presentation
A two and-a-half-year-old girl was brought to hospital, with the mother noting a progressively increasing mass in the right abdomen for 6 weeks. She was otherwise well, but for two spells of low-grade fever which resolved with antipyretics. She weighed 10.2 kg, was afebrile and normotensive (Blood pressure 50th centile). A firm, non-tender 12 × 10 cm mass in right lumbar region extending to the right hypochondrium, abutting the anterior abdomen wall demonstrating right flank fullness with dullness was noted on abdominal examination. Genitalia, spine and rest of the systemic examination were unremarkable.
She was evaluated elsewhere with an abdominopelvic ultrasonography (USG) showing a right duplex kidney with mild hydronephrosis of the upper moiety and a large hydronephrotic lower moiety without ureteric dilatation. The contralateral left kidney was normal. A contrast-enhanced computed tomography (CECT) (Figs. 1, 2, 3A) revealed a large (89 × 83 mm) homogenous mass with peripherally enhancing rim without any contrast excretion (white arrow) at the lower part of mildly hydronephrotic right kidney (black arrow). The mass extended cephalad into subhepatic region, displacing the inferior vena cava (IVC) medially. The ureter was not delineated on right side, the contralateral left kidney and ureter were normal suggesting possible duplex right kidney with poorly functioning hydronephrotic lower moiety. The other possible differentials for pediatric flank mass with the above CT findings include cystic disease of kidney, retroperitoneal lymphatic cyst and tumors like Cystic nephromas
