CAKUT (Congenital anomalies of kidney and urinary tract) constitutes 20-30% of pediatric ESKD (End stage kidney disease) patients. Kidney transplant in such patients is challenging as majority requires bladder augmentation or urinary diversion procedures. Moreover, providing an appropriate dialysis access in these patients is arduous since pre-operative evaluation usually delays the transplant. We report a case of 9 years old female child who was symptomatic since the age of one and half years, however, was diagnosed with CAKUT and urogenital sinus at the age of five years. Despite sinus repair patient remained symptomatic and eventually developed ESKD requiring multiple blood transfusions and hemodialysis at 7 years of age. The dialysis access were tunneled catheters placed in the internal jugular and femoral veins, alternatively.  During transplant evaluation she was diagnosed with poorly compliant urinary bladder and outflow obstruction.


DSA (digital subtraction angiography) study was suggestive of chronic thrombosis of Infra-renal IVC (Inferior Vena Cava) extending to the right external iliac vein, likely related to the tunneled dialysis catheter. Her immunological workup was negative for any Donor Specific Antibodies. She was planned for a kidney transplant with her mother as the donor. Graft Kidney was implanted intraperitoneally onto the left common iliac vessels followed by bladder augmentation and a continent urinary diversion. Her post-operative course was uneventful with an adequate functioning allograft. She was discharged with a serum creatinine of 0.3 mg/dl on post-operative day 9. She is maintaining a good urine output of 2.5-3 liters through the diversion stoma at 3 weeks.