Double Threat in a Newborn: High Anorectal Malformation with Pouch Colon and Bilateral Multicystic Dysplastic Kidneys - A Rare Case Report.

The coexistence of bilateral multicystic dysplastic kidneys (MCDKs) and high anorectal malformation with pouch colon is exceptionally rare. The combination results in a nonsurvivable condition due to complete renal failure, posing complex diagnostic and management challenges. A full-term male neonate presented with absent anal opening, limb and auricular deformities, and progressive anuria since birth. Antenatal history was unavailable. Clinical examination revealed bilateral clubfoot and normal male genitalia. Laboratory evaluation showed steadily worsening renal function. Ultrasound demonstrated bilateral MCDK with multiple cysts and gross hydronephrosis. A high-divided sigmoid colostomy was performed, and intraoperatively, a Type IV pouch colon was identified. Despite supportive management, including percutaneous nephrostomy, no urine output was achieved. Given the confirmed diagnosis of bilateral nonfunctioning kidneys and poor prognosis, the family opted for termination of care and support. This case highlights the diagnostic and ethical challenges associated with managing rare, fatal congenital anomalies in neonates. Early antenatal detection, when available, may facilitate better parental counseling and decision-making.