Laparoscopic Mainz II Approach for Single System Ectopic Ureter with Bladder Agenesis

Abstract

Solitary renal unit with ectopic ureter with bladder agenesis is a rare congenital anomaly. Urinary incontinence in such cases is dealt with various continent and noncontinent procedures. We report a case of 20-year-old female patient who presented with continuous leakage of urine. Examination and investigations revealed an absence of right kidney, bladder agenesis, ectopic ureter opening into the vestibule, and multiple bony abnormalities. She was managed effectively by laparoscopic Mainz II with serous lined extramural ureteral reimplantation technique.

Introduction

Bladder agenesis is a rare congenital anomaly with an incidence of 1 in 600,000 patients. It is commonly found in females (female:male ratio being 30:1) who present early in life with clinical manifestations of persistent leakage of urine. It is often associated with other anomalies such as bilateral or unilateral renal agenesis; developmental dysplasia of bones, usually pelvic bones, scoliosis; and neurologic deficits such as spina bifida.¹ In most of these cases, they are seldom compatible with life as they die early because of complications leading to renal failure. Among the patients who tend to survive, the management includes continent and noncontinent urinary diversion procedures. The clinical features and management of such a case are discussed in brief in this report.

Case Report

A 20-year-old female presented with continuous leakage of urine with no normal voiding since birth. She had associated developmental dysplasia of right hip with an abnormal gait. Genital examination showed labial adhesions, urethral, and small vaginal opening (Fig. 1). Focused neurologic examination was normal. Blood parameters and renal function tests were normal. Ultrasonography of the abdomen showed left kidney measuring 10.7 cm with mild hydroureteronephrosis and an absent right kidney. Contrast-enhanced CT urogram revealed left solitary kidney with hydroureteronephrosis with an ectopic ureter and an absent bladder (Fig. 2).

FIG. 1.

Labial adhesions with urethral and small vaginal opening.

FIG. 2.

3D Reconstructed CT showing left solitary kidney with ectopic ureter with bladder agenesis. 3D, three-dimensional

The patient initially underwent labial adhesiolysis, followed by urethroscopy, which showed an ectopic ureter opening into the vestibule with bladder agenesis. Subsequently a urinary diversion procedure was contemplated for correction of urinary incontinence. Considering the pros and cons of different urinary diversions, she opted for Mainz II procedure. Colonoscopy was done before carrying out a definitive procedure. The procedure was carried out using four ports, two 10 mm and two 5 mm (Fig. 3). The umbilical port was initially used as a camera for ureteral dissection and rectosigmoid serosa to serosa opposition for making ureteral tunnel bed. Mobilized ureter was then implanted by serous lined extramural technique (Fig. 4a).

FIG. 3.

Port placement.

FIG. 4.

(a) Arrow showing serosa lined extramural tunneling. (b) Rectosigmoid pouch closure.

Subsequently switching the camera to right 10 mm port, further detubularization of rectosigmoid colon for a length of 15 cm was done and pouch was constructed (Fig. 4b). Ureteral catheter was used for splint and a rectal tube along with abdominal drain was kept. Postoperative recovery was uneventful. Rectal tube and ureteral catheter were removed on 3rd and 7th days, respectively. Per rectal voiding with continence for 3 to 4 hours of the dry interval was achieved.

Discussion

Bladder agenesis is a rare congenital disorder with female preponderance. Bladder development occurs during 4 to 6 weeks of gestation from primitive urogenital sinus after subdivision of cloaca and through mesenchymal–epithelial interactions. In these patients, there is normal division of cloaca but there is developmental failure of urogenital sinus and failure to incorporate mesonephric duct and ureter in trigone. The defect is compatible with life only if ureters drain ectopically into Mullerian structure in females and rectum in males.

Similar findings of single system ectopic ureter with bladder agenesis in females have been reported earlier by Baheti et al.² Most affected individuals are found to have associated orthopedic and neurologic abnormalities.

Management options for this problem include continent and noncontinent urinary diversion. Continent diversions fare better over noncontinent diversions in terms of quality of life, cost, and cosmesis. However, continent diversion has risk of metabolic complications and failure of continent mechanism.

Mainz II approach has the advantages of patient comfort, no need for intermittent catheterization, and minimal risk of failure of continent mechanism. Mainz II also provides an additional advantage of significantly decreasing peak and mean bowel contraction pressures, creating a low pressure and increased capacity urinary reservoir. Ureterointestinal anastomosis done laparoscopically in this case using extramural serosal technique of ureteral implantation was more feasible and had the advantage to accommodate a dilated ureter for creating an antirefluxing anastomosis. Although there is a slight risk of development of malignancy at the ureteral implantation site in such patients in long-term follow-up in various series, the reports have been limited.³ Moreover, patient counseling for future follow-up for colon cancer screening and metabolic complications is essential. Annual colonoscopy examination of these patients starting at 10 years after the surgery is recommended.³

In young female patients, who do not wish to have a cutaneous continent stoma and may not be compliant for intermittent catheterization, anal diversion surgeries such as Mainz pouch II can be a better alternative option.⁴

Minimally invasive approach has the advantage of less blood loss, earlier postoperative recovery, and cosmesis. Laparoscopic Mainz II procedure provides a durable alternative to other diversion procedures for such cases.

Conclusion

Mainz II procedure by a minimally invasive technique is a suitable option for diversion in single system ectopic ureter with bladder agenesis.

Abbreviations Used

3D

three-dimensional

CT

computed tomography

Disclosure Statement

No competing financial interests exist.

Cite this article as: Khelge V, Kalra S, Dorairajan LN, Manikandan R, Dutt UK (2019) Laparoscopic Mainz II approach for single system ectopic ureter with bladder agenesis, Journal of Endourology Case Reports 5:4, 164–166, DOI: 10.1089/cren.2019.0062.