Laparoscopic Nephroureterectomy for Nonfunctioning Ectopic Pelvic Kidney with Ectopic Ureter and Lower Ureteral Stricture Opening in the Vestibule of the Vagina But No Incontinence: Challenges in Diagnosis and Surgical Dissection

Abstract

Failure of mature kidney to reach its natural location in renal fossa is termed as renal ectopia. Ectopic kidney can be found in pelvic, iliac, abdominal, and thoracic location. Pelvic ectopia has been estimated to occur in 1 of 2100 to 3000 autopsies. In contrast, ectopic ureters are commonly associated with complete renal duplication. Commonest presentation in females in continuous urinary incontinence with normal voiding habits as ectopic ureter open below the bladder neck in urethra or vagina. An ectopic kidney with ectopic ureter is extremely rare congenital anomaly. We report a 36-year-old woman presenting with left lower abdomen pain with no history of fever, dysuria, or urinary incontinence. On evaluation, she was found to have left nonfunctioning ectopic pelvic kidney with ectopic ureter opening in the vestibule of the vagina, which was managed with laparoscopic nephroureterectomy. One should suspect an ectopic ureter in a female presenting with continuous urinary incontinence since birth. However, diagnosis is challenging when clinical presentation is unusual with no urinary incontinence as seen in the index case. Detailed local examination in correlation with imaging is key for diagnosis and rule out other congenital anomalies. Laparoscopic approach in such clinical scenario is a safe and feasible option.

Introduction

Ectopic pelvic kidney is a rare congenital anomaly that results from an abnormal halt in migration of the kidney from the pelvis to its normal location during embryogenesis. Ectopic ureter is usually seen with duplex renal system and in women commonly present with continuous urinary incontinence. Rarely, it is reported with ectopic pelvic kidney. We present a unique amalgamation of ectopic pelvic kidney with an ectopic strictured ureter opening in the vestibule of the vagina without any history of urinary incontinence. Computed tomography imaging and local examination are essential for diagnosis and rule out other genitourinary anomalies. Laparoscopic nephroureterectomy was performed and patient had uneventful recovery.

Case Presentation

A 32-year-old woman presented with left lower abdomen pain of 6 months duration. She had no history of nausea, vomiting, fever, urinary, or bowel complaints. In the background history, patient was evaluated in a private hospital 15 days back and found to have grossly hydronephrotic left ectopic pelvic kidney on imaging. Percutaneous nephrostomy (PCN) was placed under ultrasonography guidance and she was referred to our institute. Initially, the PCN output was 300 mL; however, the output was minimal for the past 7 days. Her past medical and family history was insignificant. She is a mother to two children, both delivered normally. On examination, patient was conscious, afebrile, and her vitals were stable. Local examination showed PCN in situ. Systemic examination was grossly normal. Routine investigation reported hemoglobin was 12.3 g/dL, serum urea was 24 mg/dL, serum creatinine was 0.7 g/dL, International Normalized Ratio was 0.9 and urine analysis showed 2–4 pus cells and culture was sterile. Contrast-enhanced CT abdomen with CT urography revealed nonexcretory left ectopic pelvic kidney with gross hydronephrosis and dilated tortuous ureter traced behind and distal to the urinary bladder (Fig. 1). The right kidney, urinary bladder, and rest of the solid organs were normal. Percutaneous nephrostogram showed gross hydronephrosis of ectopic left kidney with dilated tortuous ureter tapering at level of pubic symphysis suggestive of stricture (Fig. 2). Renal functional scan in the form of ethylenedicysteine scan confirmed left nonfunctioning and right normal kidney. Patient was counseled and initially planned for laparoscopic nephrectomy. There was strong suspicion of ectopic left ureter based on imaging findings. Under anesthesia, local genital examination revealed an ectopic ureteral opening just below the external urethral meatus. Cystoscopic examination confirmed the site of left ectopic ureter distal to urethral meatus and no other ureteral opening was observed for left ureter, but right ureteral orifice was noted at normal position. Over 0.018F guidewire, 5F ureteral catheter was positioned with difficulty in the ectopic pelvic kidney (Fig. 3). Patient was placed in left lateral decubitus position and laparoscopic ports were placed as shown in Figure 4. The camera port was placed in the midline above the umbilicus and two working ports were placed on either side. The PCN was lying outside and removed. The hydronephrotic pelvic kidney was mobilized all around from the peritoneal attachments. The renal hilum was observed superomedially arising from the common iliac vessels. Single renal artery and vein were individually clipped and cut. The ectopic kidney was decompressed with 18-gauge spinal needle placed from the anterior abdominal wall for the ease of dissection. Operative duration was 130 minutes and pelvic ureteral dissection was challenging given the narrow pelvis and periureteral adhesions secondary to ureteral stricture. The dissection was done meticulously safeguarding the common iliac vessels, sigmoid colon, and the urinary bladder. The dilated tortuous ureter was identified, dissected as low as possible in the pelvis and transected (Fig. 5A–F). The cut end was not clipped to prevent closed loop obstruction. The specimen was retrieved through a small incision from the lower working port. Hemostasis was achieved, 18F drain was placed and port site were closed with vicryl 2-0 sutures. Postoperative hospital stay was uneventful. Patient was discharged on day 3 of surgery. Histopathology report showed nonfunctioning kidney with glomerular sclerosis and tubular atrophy. At 6 months follow-up, patient is asymptomatic and doing well.

FIG. 1.

(A–D) CT urography images showing normal uptake and excretion of right kidney with contrast draining in urinary bladder and left nonenhancing ectopic pelvic kidney (white arrow) with PCN in situ and ectopic dilated ureter (white arrow) observed behind and below the urinary bladder. PCN, percutaneous nephrostomy.

FIG. 2.

(A, B) Percutaneous nephrostogram showing grossly hydronephrotic left ectopic pelvic kidney with dilated tortuous ureter narrowing below the pubic symphysis suggestive of ureteral stricture with contrast splaying at its distal end.

FIG. 3.

Local examination under anesthesia showed ectopic left ureter was opening adjacent to external urethral meatus through which 5F ureteral catheter was passed.

FIG. 4.

Intraoperative positioning of the patient for laparoscopic exploration and ports placement.

FIG. 5.

Intraoperative sequential steps of laparoscopic nephroureterectomy. (A) Grossly hydronephrotic ectopic kidney lying in the pelvis with sigmoid colon pushed medially. (B) Renal hilum was observed in superomedial aspect arising from common iliac vessels. (C) Renal vein was clipped and cut, renal artery clips were applied on both sides. (D) 18-Gauge percutaneous needle was passed through anterior abdominal wall for decompression of ectopic pelvic kidney for ease of dissection. (E) Ectopic kidney is completely collapsed showing ureteral catheter in situ. (F) Ectopic kidney was mobilized all around and dilated tortuous ureter was transected till the distal most limit of dissection.

Discussion

Urinary tract anomalies include variation in number, size, location or rotation of kidneys, calices, ureter or urinary bladder. At times, it may be associated with certain syndromes and other organ anomalies. Renal ectopia is a common urinary tract anomaly with reported incidence of 1:500–1:1100 live births. It is defined as failure of mature kidney to reach its normal location in renal fossa. The hypothesis for renal ectopia include absence of orderly ascent and rotation such as ureteral bud maldevelopment, defective metanephric tissue, genetic abnormalities, maternal illnesses, or teratogenic causes.¹ The pelvic kidney located opposite the sacrum below the aortic bifurcation is the most common site of renal ectopia. The association of renal ectopia with ectopic ureter is extremely rare. Most of the ectopic ureters are seen with complete duplex renal system. The upper moiety commonly is drained by the ectopic ureter opening in the bladder neck, posterior urethra, or mesonephric duct derivatives in males and urethra, vestibule, vagina, or uterus in females.² Common presentation in males include flank pain, epididymo-orchitis, prostatitis, or recurrent urinary tract infection. In >95% of females, ureter opens distal to the urethral sphincter or into the reproductive tract resulting in classical symptom of continuous urinary incontinence with normal voiding habits. In the index case, the ectopic ureter was draining single system ectopic pelvic kidney and opening in the vestibule just adjacent to the external urethral meatus. The distal end of the ectopic ureter showed abrupt tapering on percutaneous nephrostogram suggestive of stricture, probably the reason patient never had any incontinence at presentation. This case highlights the presence of ectopic ureteral stricture opening in the vestibule draining a single system ectopic pelvic kidney with uncommon presentation. Ectopic kidney with ectopic ureter may be associated with genital defects such as uterine and vaginal anomalies in females and undescended testis and hypospadias in males. Common urinary tract anomalies seen with ectopic kidneys are pelviureteric junction obstruction, vesicoureteric junction obstruction, and reflux. Even contralateral kidney is susceptible to increased risk of congenital defects.³ Local examination is essential to look for ectopic ureteral opening and one should carefully inspect for the associated anomalies. The radiologic evaluation should focus on location of the kidney and ureter and look for any other genitourinary anomalies. Intravenous pyelogram is of little use in poorly functioning kidneys. Cross-sectional contrast CT and magnetic resonance urography is recommended for its precise localization and appears to be more effective. CT angiography may be considered to look for aberrant vessels when contemplating surgery in ectopic kidneys. Renal dynamic scan (Tc99 DMSA) is essential at times to document the function and location of the kidney.⁴ Functioning renal units require ureteral reimplantation. Nonfunctioning kidney warrants nephrectomy. Laparoscopic approach is a feasible option as kidneys are small and amenable for surgical excision. Narrow pelvis anatomy may pose technical challenges during dissection. Meticulous sparing of neurovascular structures, rectum, and bladder is of utmost importance. Minimizing the range of instrument movements and judicious use of energy sources helps preventing collateral damage. Robotic assistance may facilitate greater freedom of movement, but comes with increased cost to the patient. Extension of trocar incision can be circumvented with the use of morcellator for specimen extraction. In our case, we decompressed the hydronephrotic kidney making it easily retrievable through small extension of trocar site incision. Early ambulation, reduced postoperative analgesics requirement, and improved cosmesis are benefits of laparoscopic nephroureterectomy. Laparoscopic approach is useful for diagnostic evaluation as well as therapeutic excision of nonfunctioning kidney. In the index case, laparoscopic nephroureterectomy was performed and specimen was retrieved from extended incision of the lower working port. In the follow-up, patient is asymptomatic and doing well.

Conclusion

Ectopic pelvic kidney with an ectopic ureter opening in vestibule of the vagina is rare. Although females most commonly present with continuous urinary incontinence, a concomitant distal ureteral stricture is still rarer and may facade the incontinence episodes. Imaging and local examination is essential for diagnosis and planning surgical excision. Laparoscopic approach can be a feasible option with early recovery and better cosmesis.

Abbreviations Used

CT

computed tomography

PCN

percutaneous nephrostomy

Disclosure Statement

No competing financial interests exist.

Funding Information

No funding was received for this article.

Cite this article as: Ranjan KR, Parmar K, Tyagi S, Mandal S, Singh SK (2020) Laparoscopic nephroureterectomy for nonfunctioning ectopic pelvic kidney with ectopic ureter and lower ureteral stricture opening in the vestibule of the vagina but no incontinence: Challenges in diagnosis and surgical dissection, Journal of Endourology Case Reports 6:4, 497–501, DOI: 10.1089/cren.2020.0164.