Bilateral Primary Obstructive Megaureter with Concurrent Kidney and Ureteral Stones: A Rare Case

Primary obstructive megaureter (POM) is a rare congenital anomaly in adults, typically involving functional obstruction at the distal ureteral segment without an identifiable mechanical cause. Adult presentation is uncommon, and bilateral involvement with concurrent renal and ureteral stones is exceedingly rare. We report the case of a 33-year-old male who presented with bilateral flank pain, recurrent graveluria, and intermittent fever. Imaging revealed bilateral hydronephrosis, dilated distal ureters, and multiple calculi in both kidneys and ureters. Exclusion of reflux or bladder outlet obstruction confirmed the diagnosis of bilateral POM. The patient underwent staged open surgical interventions, including ureterolithotomy, distal ureteral excision, tapering, extravesical ureteral reimplantation using the modified Lich-Gregoir technique, and pyelolithotomy. A total of 133 smooth calculi were retrieved from both ureters and kidneys. Histopathological examination confirmed POM by demonstrating absence of smooth muscle in the resected ureteral segments. This case highlights the diagnostic and therapeutic challenges in managing adult bilateral POM with extensive stone burden. It underscores the importance of considering congenital anomalies in adults with unexplained ureteral dilation and recurrent urolithiasis, and emphasizes the need for timely surgical intervention to preserve renal function and prevent recurrence.