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Background <p>Bilateral single-system ectopic ureters (BSEU) with a hypoplastic bladder are exceptionally rare, and long-term renal outcomes after bladder‑preserving strategies are scarcely documented. We aimed to describe 20‑year trajectories of renal and lower urinary tract outcomes under a staged, augmentation‑sparing reconstruction.</p> Case presentation <p>A female infant with BSEU and a hypoplastic bladder underwent early refluxing ureterovesicostomy to initiate urine cycling, followed by bilateral ureteral reimplantation. During childhood, hydronephrosis fluctuated and postoperative vesicoureteral reflux (VUR) required endoscopic injections. Bladder capacity and compliance improved gradually, with daytime continence at 13.3 years and full nocturnal continence at 19.9 years. In adulthood (21.8 years), renal cortical integrity remained preserved on serial DMSA scans; serum creatinine was 0.77&#xa0;mg/dL and eGFR 79.6 mL/min/1.73&#xa0;m² (CKD G2). Periodic worsening of hydronephrosis correlated with voiding postponement and fluid restriction rather than fixed anatomic obstruction. Blood pressure was intermittently borderline.</p> Conclusions <p>This case demonstrates that early urine cycling, timely endoscopic control of recurrent VUR, and vigilant, life‑course nephro‑urological surveillance can preserve renal function without augmentation in severe congenital anomalies of the kidney and urinary tract (CAKUT) phenotypes. Modifiable behavioral factors may critically influence upper tract status even after anatomical reconstruction.</p>

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Twenty-year Renal Functional Outcome After Bladder-Preserving Staged Reconstruction in Bilateral Single-System Ectopic Ureters with a Hypoplastic Bladder: A Case Report

  • 期刊论文

  • 发表日期：

    2026年7月29日

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  • Kazuyoshi Johnin,
  • Toshihiro Sawai,
  • Kohei Mukai,
  • Yotaro Hirao,
  • Daichi Johnin,
  • Ryosuke Kikui,
  • Yuta Okumura,
  • Kentaro Inoue,
  • Yuri Mori,
  • Shota Nakagawa,
  • Masanari Nishida,
  • Masayuki Nagasawa,
  • Shigehisa Kubota,
  • Akinori Wada,
  • Kenichi Kobayashi,
  • Kazuaki Yamanaka,
  • Tetsuya Yoshida,
  • Susumu Kageyama

摘要

Background

Bilateral single-system ectopic ureters (BSEU) with a hypoplastic bladder are exceptionally rare, and long-term renal outcomes after bladder‑preserving strategies are scarcely documented. We aimed to describe 20‑year trajectories of renal and lower urinary tract outcomes under a staged, augmentation‑sparing reconstruction.

Case presentation

A female infant with BSEU and a hypoplastic bladder underwent early refluxing ureterovesicostomy to initiate urine cycling, followed by bilateral ureteral reimplantation. During childhood, hydronephrosis fluctuated and postoperative vesicoureteral reflux (VUR) required endoscopic injections. Bladder capacity and compliance improved gradually, with daytime continence at 13.3 years and full nocturnal continence at 19.9 years. In adulthood (21.8 years), renal cortical integrity remained preserved on serial DMSA scans; serum creatinine was 0.77 mg/dL and eGFR 79.6 mL/min/1.73 m² (CKD G2). Periodic worsening of hydronephrosis correlated with voiding postponement and fluid restriction rather than fixed anatomic obstruction. Blood pressure was intermittently borderline.

Conclusions

This case demonstrates that early urine cycling, timely endoscopic control of recurrent VUR, and vigilant, life‑course nephro‑urological surveillance can preserve renal function without augmentation in severe congenital anomalies of the kidney and urinary tract (CAKUT) phenotypes. Modifiable behavioral factors may critically influence upper tract status even after anatomical reconstruction.