Bilateral Ureteropelvic Junction Obstruction Causing Anhydramnios
Naveen Nandanan MD, C.D. Anthony Herndon MD, Rebecca Zee MD
This female patient had a normal prenatal course until 21-weeks gestation, when prenatal US revealed anhydramnios and bilateral upper-tract obstruction with multicystic dysplastic kidneys. The patient was born at 37 3/7 weeks with significant pulmonary hypoplasia requiring bilateral chest tubes and immediate intubation. She was anuric and renal US showed severe bilateral hydronephrosis with cortical thinning. On DOL 3 she underwent bilateral percutaneous nephrostomy tube placement. Over the next two weeks, the patient had good urine output from the right PCN and minimal output from left PCN with improving creatinine and electrolyte profile. The left PCN was subsequently removed and she was extubated on DOL 15 with renal US showing decreased dilation of the left system and right PCN in good position with no dilation. The patient was taken to the OR at 1 month for bilateral retrograde pyelogram, which revealed severe right UPJO and patent left ureter with focal narrowing. A right-sided open pyeloplasty was performed. The patient had an extended inpatient course due to her CKD and feeding optimization but was discharged home 1 month later. Follow-up US at 6 months revealed mild pelvic dilation on the right and stable persistent dilation of major calyces on the left. Due to her stage 4 CKD, she was started on peritoneal dialysis and is currently undergoing evaluation for renal transplant.
Discussion and Conclusion
Bilateral UPJO, while rare, can cause pulmonary hypoplasia and kidney dysfunction leading to significant morbidity and mortality. Early detection allows for potentially lifesaving interventions until definitive repair can be performed.
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