Perioperative Anuria Resulting in the Diagnosis of Infrasphincteric Ectopic Ureter in an Adult Female.

Ectopic ureter, particularly in females, often presents at a young age as incontinence. Otherwise healthy girls who have continuous urinary wetting during day and night despite adequate toilet training should be held with a high level of suspicion for an extravesical infrasphincteric ectopic ureteral orifice. If such a diagnosis goes undiscovered, frequent reflux or obstruction may cause permanent damage to the renal unit, as in the patient discussed here.

Introduction

Ectopic ureter is an anatomic variant, where the ureteral orifice is located at a location other than the posterolateral aspect of the trigone of the bladder. This is more common in females than males, at a ratio of approximately 6:1. Male ectopic ureters most commonly insert into the posterior urethra. The most common sites of this anomaly in a female are the urethra, vestibule, and vagina. Females most often present with incontinence because the opening is often distal to the external sphincter. Other presentations include infection, hydronephrosis, or reflux. A duplex collecting system is present in 80% cases of ectopic ureter.

Case presentation

A 39-year-old woman presented with frequent urinary tract infections for approximately 3 years. She had a previous diagnosis of right-sided grade-IV vesiculoureteral reflux with right hydronephrosis resulting in a nonfunctioning right kidney. A computed tomography scan showed right cortical atrophy with dilatation of the right ureter, with minimal contrast entering the intrarenal collecting system. She then underwent a right laparoscopic radical nephroureterectomy via a transperitoneal approach to remove the chronically infected kidney. Midway through the case and in the postoperative area, the patient became anuric despite adjustment and replacement of the Foley catheter. After a short time, the patient was taken to the cystoscopy suite. Immediately after the cystoscope was introduced into the urethra, 2 openings were noted. The left opening, as expected, led directly into the bladder. However, the right opening lead directly into the ureteral stump, demonstrating the insertion of an ectopic ureter. No right ureteral orifice was found opening into the bladder, making it a single ectopic system (Fig. 1). A Foley was placed in the bladder using a guidewire under cystoscopic visualization and urine was evacuated. The patient has had no complications since the procedure.

Figure 1

Anatomy of ectopic ureter

Discussion

The patient presented in this case is unique for several reasons. Initially, the patient was diagnosed with right-sided grade-IV vesiculoureteral reflux, which by definition is incorrect because of the absent direct connection of the bladder to the ectopic ureter. Despite multiple cystoscopies and contrast computed tomography scans during the workup, the abnormality was difficult to identify. Also, considering this patient's anatomy, incontinence much earlier in life would have been expected to be the presenting and most severe symptom. The patient had complaints of incontinence in her teenage years, which had since resolved; however, it was significantly overshadowed by her frequent urinary tract infections. The incidental method of finding the correct diagnosis was also distinctive. During the placement of the perioperative Foley, the catheter tip went into the right-sided opening, which ended in the single ectopic ureteral stump. The remaining Foley tubing then inadvertently obstructed the urethra, and therefore stopped all outflow of urine from the functioning left kidney.

Conclusion

The case described here demonstrates a serendipitous method of diagnosis of ectopic ureter in an adult female. A high level of suspicion for young girls with incontinence should raise thoughts of ectopic ureter and prompt the proper workup to prevent permanent renal damage.