成人期發現的罕見先天性泌尿道異常：併發急性腎盂腎炎之雙側完全性輸尿管重複併輸尿管膨出

黃揚軒¹、王世鋒¹

¹國泰綜合醫院 外科部 泌尿科

An Unusual Congenital Anomaly Presenting in Adulthood: Bilateral Complete Ureteral Duplication with Ureteroceles Complicated by Acute Pyelonephritis

 Yang-Hsuan Huang¹, Shih-Feng Wang¹

¹Division of Urology, Department of Surgery, Cathay General Hospital, Taipei City, Taiwan

Ureteroceles are a relatively rare congenital anomaly, affecting females 4 to 6 times more frequently than males, partly because completely duplicated systems are also more common in women. About 10% of all ureteroceles are bilateral, potentially affecting both renal units. In adults, the condition may be asymptomatic or cause flank pain, urolithiasis, or recurrent UTIs. Ureteroceles in adults are often found incidentally when imaging studies are performed for unrelated reasons. Adult ureteroceles are typically unduplicated, intravesical, and involve a single system.

A 62-year-old woman with no known systemic diseases presented to the emergency department with intermittent fever, abdominal pain, right flank soreness, nausea, and vomiting. Laboratory investigations revealed leukocytosis, along with hematuria, pyuria, and bacteriuria on urinalysis. Contrast-enhanced computed tomography (CT) of the abdomen and pelvis demonstrated bilateral duplicated ureters, mild dilatation of the upper moieties of both kidneys, perinephric fat stranding around the right kidney, and two large cystic lesions within the urinary bladder. With a clinical impression of bilateral ureteral duplication complicated by acute pyelonephritis, the patient was admitted for intravenous antibiotic therapy and further diagnostic evaluation.

Intravenous pyelography demonstrated a bilateral duplicated collecting system with dilatation of both upper moieties. However, both previous imaging studies were unable to clearly distinguish between complete and partial ureteral duplication. Subsequently, urological magnetic resonance imaging (MRI) was performed, which revealed bilateral complete ureteral duplication accompanied by large bilateral ureteroceles, consistent with the Weigert–Meyer law.

Following infection control, transurethral resection of the ureteroceles was performed two weeks after the patient’s discharge. Intraoperatively, both ureteroceles were identified and incised. After locating the lower ureteral orifices, the residual ureteroceles were excised. Double-J ureteral stents were then placed through the exposed lower ureteral orifices bilaterally. The right-sided stent dislodged spontaneously, while the left stent was removed two weeks postoperatively.

Preserving the function of the upper moiety in cases involving a duplex urinary system by early endoscopic incision is paramount. When used in appropriate cases, studies have indicated a definitive cure in over 90%. Studies show that patients with poorly functioning upper moieties after endoscopic treatment rarely require secondary heminephrectomy unless complications arise or the moiety becomes nonfunctional.