新生兒雙側完全性雙套輸尿管系統合併右側輸尿管膨出：一個案例分析

黃韋鈞、黃志平、陳國樑

中國醫藥大學附設醫院 泌尿部

Bilateral complete duplication of urinary collecting system with ureterocele of right upper moiety in a newborn: a case report.

Wei-Chun Huang, Chi-Ping Huang, Kuo-Liang Chen

Department of Urology, China Medical University Hospital, Taichung, Taiwan

Introduction:

The duplication of the urinary collecting system represents a common anatomical anomaly, which may present asymptomatically or be associated with ureterocele, vesicoureteral reflux, incontinence, obstructive uropathy, or renal parenchymal scarring. The incidence of duplicated urinary systems ranges from 0.7% to 4% of the population, with a higher prevalence in females and typically diagnosed during childhood. Currently, the optimal treatment strategies for patients with ectopic ureteroceles and duplex collecting systems remain contentious. Herein, we aim to present a case treated with transurethral incision in ureterocele with a duplicated urinary collecting system.

Case:

An 8-month-old girl with medical history including preterm labor at gestational age 30＋1 weeks, with prenatal hydronephrosis, bilateral duplication of the collecting system, right VUR[1]  grade 2, recurrent urinary tract infections, necrotizing enterocolitis status post loop ileostomy, and A-T-D colon fibrosis status post subtotal colectomy was admitted to our ward due to fever for one day.

Tracing back her history, prenatal imaging at 24 weeks' gestation revealed congenital bilateral hydronephrosis[2] , bilateral duplicated collecting system and suspect right ureterocele. After birth, renal imaging revealed a bilateral duplicated collecting system with bilateral upper moiety severe hydronephrosis, hydroureter and right ureterocele while bilateral lower moiety showed mild hydronephrosis. At that time, emergent Foley decompression was performed due to significant right ureterocele causing bladder outlet obstruction. The patient received regular renal echo follow up, nutritional support and underwent cystourethroscopy at 2 months of age, which confirmed the diagnosis of bilateral complete duplication of urinary collecting systems without definitive ureteroceles. However, recurrent urinary tract infection persisted. Followed imaging including VCUG demonstrated 1. Right grade II VU reflux. 2. A diverticula in the right UB wall. 3. ureterocele is more favored at the right side. After antibiotics treatment for infection control, the fever resolved, and no further fever flare-up was observed. Despite this, intermittent fever and foul-smelling urine prompted readmission, where recurrent urinary tract infection was diagnosed. Renal sonography turned out left upper moiety acute pyelonephritis and left lower moiety mild hydronephrosis while right small kidney, right upper moiety and lower moiety pelviectasis, right upper moiety with cystic change. Ureterocele of right upper moiety was also detected. DMSA renal scintigraphy revealed cortical defects in bilateral kidneys and global shrinkage of the right kidney. Therefore, we decided to redo cystourethroscopy again after antibiotic treatment. Bilateral complete duplication of collecting systems with the ureteral orifice of the right upper moiety located at the bladder neck, forming the ureterocele was confirmed intraoperatively. The transurethral incision was performed smoothly, leading to the resolution of subsequent urinary tract infections ever since. Follow up renal echo also showed hydroureter resolved but no ureterocele detected.

Conclusion:

Symptoms associated with duplicated collecting systems can vary from asymptomatic to symptomatic. Imaging modalities such as ultrasonography, voiding cystourethrography (VCUG), intravenous pyelography (IVP), computed tomography (CT), dimercaptosuccinic acid (DMSA) renal cortical scintigraphy, 99mtechnetium-mercaptoacetyltriglycine (99mTc-MAG-3) renography, and magnetic resonance urography (MRU) are valuable tools for diagnosing urinary tract anomalies, particularly duplicated urinary systems with ureterocele. In summary, we present this case of early intervention with transurethral incision resulting in an excellent outcome.