Single vaginal ectopic ureter via Gartner's duct cyst spontaneously perforating into the bladder

We report on a girl with a rare variant of single vaginal ectopic ureter via Gartner's duct cyst, which ruptured spontaneously into the bladder leading to aggravation of urinary incontinence. The patient also had a megalovagina, and a fistula between the distal urethra and vagina. She became continent after removal of a dysplastic kidney, unroofing of the cyst and closure of the fistulas.     

Clinicopathological and embryological considerations of single ectopic ureters opening into Gartner's duct cyst: a unique subtype of single vaginal ectopia

We report 6 cases of single ectopic ureters opening into a Gartner's duct cyst. All but 1 patient were children. The presenting symptoms were urinary incontinence, urinary tract infection, a vaginal mass or weight loss. All but 1 patient had a mass protruding into the vagina. In 5 cases nephroureterectomy was performed successfully. The cyst wall was left untouched to allow for its spontaneous collapse. Although 2 cysts were found to communicate with the vagina, 4 presented as a closed space. All resected kidneys were more dysplastic than those with vaginal ectopia without cyst formation. The etiology and pathogenesis of the anomaly are unknown but some speculations are made.     

Bladder dysfunction in children with bilateral single ectopic ureters

Background: Single ectopic ureters are a rare malformation in children. Therapy consists of ureteral reimplantation. However, in case of bilateral single ectopic ureters, subsequent malformation of the bladder trigone and bladder neck may result in additional voiding dysfunction, and ureteral reimplantation alone may not solve the urologic problems. Methods: The authors report their experience with 2 girls, in whom bilateral single ectopic ureters were treated by ureteral reimplantation in early childhood and who did not gain adequate bladder control during following years. Results: Videourodynamic evaluation was done in both girls. No bladder overactivity was found during the urodynamic studies. However, cystography showed a widely open bladder neck during filling with no sufficient bladder neck closure shown by urethral pressure profile studies. When blocking the bladder outlet by balloon catheters, adequate bladder filling volume was achieved. Incontinence was cured by implantation of an AMS 800 artificial sphincter system in a 10-year-old girl. A 7-year-old girl was regarded to be too young for sphincter implantation and is waiting for surgery within the next years. Conclusion: Insufficient development of trigone and bladder neck with subsequent urinary incontinence has to be kept in mind when deciding on surgical procedures in children with bilateral single ectopic ureters.     

The artificial urinary sphincter AMS 800 in the treatment of complex female urinary incontinence: indications, results, complications and risk factors

Summary Indications, results and complications of 144 women who have undergone implantation of the current model AMS 800 since 1983 and remained in continuous follow-up in our department are presented. Included are 70 patients suffering from stress urinary incontinence (SUI) type III after 208 previously unsuccessful incontinence procedures, 54 patients with incontinence due to neurogenic bladder dysfunction (NBD) and 20 patients with congenital or acquired internal sphincter weakness of other causes. In the NBD group, apart from sphincter implantation a total of 113 additional operations were necessary due to complex malfunctions of the urinary tract: augmentation ileocystoplasty in 51 patients, ureterocystoneostomy in 23 patients and 43 operations at the bladder neck. One hundred and twenty-six patients (86 %) achieved total continence and 5 patients (3 %) were significantly improved. In 9 females (6 %) incontinence persisted, and 4 patients ultimately underwent urinary diversion into a Kock pouch. With the implanted artificial sphincter 116 patients (81 %) are able to empty their bladder spontaneously, without residual urine and without the necessity of intermittent self catheterisation. Under the aforementioned conditions implantation of the artificial urinary sphincter AMS 800 is the most effective therapy for complex female incontinence.      

Two ectopic openings of an unduplicated ureter into the bladder neck and the vagina: a case report

A case of two ectopic ureteral openings into the bladder neck and the vagina is reported. A 6-year-old-girl was admitted with gross hematuria and incontinence. The left kidney could not be visualized by excretory pyelography. Voiding cystogram revealed left vesicoureteral reflux. Left ureteral orifice could not be confirmed by cystoscopic examination. In January 1982, left nephroureterectomy was carried out. Contrast material injected into the left ureter during the operation was found to be drained into the bladder and the vagina. Thus, left ureter was resected close to the end of the ureter to avoid injury of the urethra and its sphincter. After the operation, incontinence disappeared. This case is the second case of two ectopic openings of unduplicated ureter.     

Success of the artificial urinary sphincter after failed surgery for incontinence

We implanted the artificial urinary sphincter in 15 incontinent patients for whom multiple urethral and bladder neck operations, including sphincter placement, had been unsuccessful. The 5 male and 10 female patients ranged from 3 to 26 years old (mean age 11 years). The etiology of incontinence was neurogenic bladder in 10 patients, epispadias in 2, exstrophy in 1, ectopic ureters in 1 and traumatic urethral disruption in 1. Of the 15 patients 13 required augmentation enterocystoplasty and clean intermittent catheterization. The initial anti-incontinence procedures were Young-Dees-Leadbetter bladder neck reconstruction in 10 patients, artificial urinary sphincter placement in 4 and bladder neck suspension in 1. Causes of failure of the primary treatment were erosion (artificial urinary sphincter), and incontinence and/or difficult catheterization (Young-Dees-Leadbetter). Followup from the last salvage operation averaged 21 months (range 6 to 37 months). A total of 58 operations was performed. Among the 4 patients in whom the artificial urinary sphincter eroded the bladder neck repeated attempts to place the cuff at the same site were unsuccessful and erosion occurred in all 4 within 1 year. Sphincter placement was more successful among the 11 patients who initially underwent Young-Dees-Leadbetter bladder neck reconstruction or bladder neck suspension; acceptable continence was attained in 8 patients (73%). We conclude that placement of the sphincter cuff around a previously eroded bladder neck probably will result in erosion. Sphincter implantation should be attempted in patients in whom bladder neck reconstruction has failed. Persistence in the treatment of these patients is essential because multiple operations often are necessary to achieve continence.     

Single ectopic ureter in the male

Three male patients with single eetopic ureters, opening into the ejaculatory duct, seminal vesicle, or prostatic urethra, are presented; in each the associated kidney was dysplastic. This diagnosis should be considered when a male patient presents with recurrent urinary tract infections, epididymitis, painful defecation, or vague, lower abdominal pain, associated with radiographic findings of a nonvisualized kidney and an extrinsic bladder defect. The surgical procedure of choice is nephroureterectomy, with excision of the structure receiving the ectopic ureter.     

Enterocystoplasty in the management and reconstruction of the pediatric neurogenic bladder

Eighteen children and young adults with neurogenic bladder underwent enterocystoplasty as part of urinary undiversion or for treatment of incontinence associated with reduced bladder compliance or detrusor sphincter dyssynergia. In 12, tubular sigmoid enterocystoplasty with transureteroureterostomy was performed with the smaller diameter ureter implanted into the bowel tenia. In two patients the ileocecal segment was used to augment the bladder, and the ureters were anastomosed to the ileum. In four patients the cecum or a patch of sigmoid colon was used to augment the bladder. Young-Dees bladder neck reconstruction was performed on eight patients at the time of surgery; one later required bladder neck reconstruction, and two later required an artificial sphincter. After a mean follow-up of 20 months, 16 of the 17 available for follow-up are continent with clean intermittent catheterization every 3 to 4 hours. Nine patients require anticholinergic or smooth muscle relaxing medication to increase functional bladder capacity. Most of the patients need chronic antimicrobial treatment to control bacteriuria.     

Ureter malformations and renal dysplasia--an embryological concept (author's transl)

A study was made of 32 cases of single ectopic ureters (without duplication) to assess the abnormalities observed in the corresponding renal parenchyma and to correlate renal status with location of the ectopic uretic opening. Specimens were obtained from 26 nephrectomies and renal dysplasia was found in 22 cases. There was a close relationship between renal anomalies and location of the ureteric opening. The more remote the ectopic orifice from its normal position, the more severe were the associated renal lesions. Dysplasia was always present when the ectopic ureter opened outside the urinary tract (vagina, Gartner's duct, vestibule, seminal tract). Am embryological concept is proposed to explain the association of renal dysplasia with abnormal location of the ureter opening. It is based on the abnormal--premature or late--appearance of the ureteral bud on the Wolffian duct resulting in the abnormal positioning of the bud on the Wolffian duct with the ureter growing into nephrogenic tissue lacking the potential for normal renal development and leading to renal dysplasia.     

Single system ureteral ectopia in boys associated with bladder outlet obstruction

PURPOSE: Ureteral ectopia is frequently associated with dysplasia of the associated renal segment in girls with ureteral duplication. However, single system ureteral ectopia is an uncommon anomaly more frequently noted in boys. We report on 6 boys with single system ureteral ectopia into the prostatic urethra above the verumontanum, who presented with radiological and clinical findings of bladder outlet obstruction. MATERIALS AND METHODS: Antenatal ultrasound in 3 boys demonstrated renal abnormalities and postnatal studies suggested the diagnosis of posterior urethral valve obstruction. Older boys presented with symptoms suggestive of bladder outlet obstruction. RESULTS: An ectopic ureter inserting into the prostatic urethra was noted in all 6 boys. The distal ureter was dilated elevating the bladder neck causing outlet obstruction. Surgical management consisted of nephrectomy and transurethral endoscopic incision of the distal ureter or nephroureterectomy with reconstruction of the prostatic urethra. In 2 younger boys voiding dysfunction with inability to empty developed. CONCLUSIONS: Single system ectopic ureters in boys may present with symptomatic and radiological findings resembling posterior urethral valves. Surgical treatment should include nephroureterectomy with reconstruction of the hypoplastic prostate. Some patients may have later voiding dysfunction.     

Crossed fused kidney with ectopic ureter: a case report

A 5-year-old girl with a rare anomaly of a single system ectopic ureter draining into the mesonephric duct cyst and ipsilateral crossed fused kidney is presented. Both ureters were catheterized and three dimensional (3-D) images of the urinary tract were reconstructed with helical computed tomographic (CT) data. A mesonephric duct cyst, the right ureter draining into the cyst and an ectopic orifice were well depicted with these images, especially with inferior projection images. 3-D display of helical CT data with a retrograde contrast study is a promising method of obtaining anatomical detail of an ectopic ureter.     

Single unilateral vaginal ectopic ureter: is it really a rarity?

Although previously regarded as a rarity, 13 of the 20 published cases of single unilateral vaginal ectopic ureter have been reported within the last year. We have seen 4 cases of a single unilateral vaginal ectopic ureter within a 4-month interval. The relationship of vaginal ureteral ectopia to müllerian and mesonephric duct anomalies is discussed. If the ureteral bud originates laterally from the mesonephric duct then the ureter may retain its connection with the mesonephric duct while failing to connect to the bladder. This condition combined with persistence of the distal mesonephric (Gartner's) duct, which ruptures vaginally, accounts for vaginal ureteral ectopia and incontinence. The diagnostic studies for vaginal ureteral ectopia include excretory urography, radionuclide scanning, ultrasonography and administration of oral phenazopyridine in conjunction with careful vaginoscopic examination. The combination of lifelong wetting and solitary kidney on excretory urography should alert the urologist to this syndrome. The single unilateral vaginal ectopic ureter may be more common than has been suggested previously.     

Single-system ectopic ureters. A review of 19 cases

Single system ectopic ureters are usually associated with multiple congenital abnormalities and the corresponding renal units is frequently abnormal. 19 cases of single system ectopic ureters were diagnosed and treated in our hospital during the last 20 years. The patients were less than 11 years old. The abnormality was bilateral in two cases and the 63% of all had associated malformations, being the anorectal abnormalities the most frequent. The incidence of reflux into the ectopic ureter was 57%, and the association to unilateral or contralateral renal agenesis, 26.3%. Thirteen ectopic ureters were reimplanted successfully into the bladder, and the cases of nonfunctioning kidneys were removed (nephroureterectomy). The follow-up period was 10 months to 14 years, developing the 68% of the cases without any complication and a good renal function. Two patients were transplanted due to a kidney failure. Diagnosis is often extremely difficult and requires a high degree of suspicion. This diagnosis may be incidental, and it may be made during investigation of patients with other malformations. An early diagnosis and treatment must be made in order to avoid a disturbance of renal function. We wish to point up the high incidence of reflux into the ectopic ureter and the high association to renal agenesis in the single system ectopic ureters in comparison with ectopic ureter of duplex system.     

Ectopic ureter opening to vestibule without urinary incontinence: a case report]

A case of a 2-year-old female with right ectopic ureter opening in vestibule vaginae and without urinary incontinence is reported. Excretory urogram showed mild dilatation of the upper right segment with bilateral complete duplication. Right ectopic ureter some functioning upper segment of the kidney was reimplanted into the bladder to avoid surgical intervention for heminephrectomy. According to the retrograde ureterogram of ectopic ureter the running courses and shapes of the dilated distal portions of ureters were compared between two groups, ectopic ureter with incontinence and that without incontinence. We suppose the continence mechanism of ectopic ureter is kept when the running course of the ureter through some portion of the urethral sphincter musculature.     

Two cases of male ectopic ureter with hydroureter

Male ectopic ureters are relatively rare. We herein report two cases of male ectopic ureter. In case 1 a 34-year-old man had left renal hypertrophy incidentally found by ultrasonography. In case 2 a 58-year-old man had the chief complaint of urinary retention. These two patients had ectopic ureters opening into the posterior urethra with hydroureter and renal hypoplasia (Thom I), and nephroureterectomy were performed. There are 105 cases of male ectopic ureter in the Japanese literature. The type of the disease (according to Thom's classification), age distribution, opening site, associated abnormalities and treatment of male ectopic ureter were discussed.     

Experiences in kidney transplantation with duplicated ureters

INTRODUCTION: The purpose of this study was to evaluate the complications of duplicated ureters in renal transplant recipients. METHODS: Between 1983 and 2004, 12 patients (median age 34 years) received renal transplants from donors with duplicated ureters. In four patients the ureter to bladder anastomoses were performed separately according to the method described by MacKinnon, including two cases transplanted with ureteral catheters because of narrow widths. In the following cases of eight duplicated ureters an anastomosis was performed between the distal part of each ureter to form a common ureteral ostium, which was connected to the urinary bladder. A ureteral catheter was used to the splint ureterovesical anastomosis. RESULTS: No graft loss to ureteral complications was observed. There was no ureteral necrosis in the postoperative period. No clinical symptoms of ureteral junction obstruction were revealed after removing the ureteral catheter. By ultrasound examination four patients showed a slight temporary pyelocaliectasis was observed and four patients developed temporary urinary fistulas. CONCLUSION: Our ureterocystoneostomy procedures with duplicated ureters were safe and useful in kidney transplantation.     

Vaginal wall sling: four years later.

Since December 1985, we have treated 65 patients with urinary stress incontinence due to intrinsic sphincter dysfunction with the vaginal wall sling procedure. Of the 54 patients who were available for follow-up, intrinsic sphincter dysfunction was related to multiple prior bladder neck suspension procedures in 48 patients. In the remaining 6 patients, 2 had pelvic trauma, 2 had neurogenic urethral dysfunction, 1 had urethral diverticulectomy, and 1 had pelvic radiation. The success rate of the vaginal wall sling procedure for correcting stress incontinence was 94.4 percent at a mean follow-up of 23.9 months. Postoperative complications were minimal. Although 83 percent were temporarily in urinary retention, in the absence of neurogenic bladder and augmentation cystoplasty, only 5.5 percent needed intermittent self-catheterization on a long-term basis. De novo detrusor instability developed postoperatively in 14.8 percent of the cases. In no patient did a vaginal inclusion cyst develop. The vaginal wall sling is a simple procedure with excellent success rate and minimal morbidity. We recommend it for patients with stress urinary incontinence due to intrinsic sphincter dysfunction.     

Surgical management of urinary incontinence in children with anatomical bladder-outlet anomalies

BACKGROUND: Pediatric urinary incontinence secondary to anatomical anomalies of the bladder outlet is relatively rare, and there is no consensus on its surgical treatment. We reviewed the clinical courses and surgical management of children with this pathology. METHODS: From 1991 to 2001, we performed bladder neck reconstruction on seven children (three boys and four girls). The mean age at the first intervention was 7 years (range 5-12 years). The underlying diagnosis was bladder exstrophy in two children, pseudoureterocele-type ectopic ureter in three, bladder neck incompetence from unknown etiology in one boy with hypospadias, and bilateral single ectopic ureter associated with cloacal malformations in one child. Lower urinary tract was evaluated through various imagings, endoscopy, and urodynamic studies. RESULTS: The methods used to reconstruct the bladder neck was the Young-Dees-Leadbetter procedure (performed on four children); the Kropp procedure (two children); and the Pippi-Salle procedure (one child). Bladder augmentation with intestinal segment was performed on four children; Mitrofanoff continent stoma was created in all patients. Urethral catheterization could not be routinely used in all patients, because of pain, stricture, or complicated urethral anatomy. All patients achieved urinary continence. CONCLUSION: The treatment of pediatric urinary incontinence from anatomical anomalies is technically challenging. Depending on the underlying disorders and anatomy, one needs to decide which method of reconstruction to perform. It would be safer to employ simultaneous augmentation and to create catheterizable continent stoma.     

Skene's duct cyst

Skene's duct cysts are rare. A review of the medical literature revealed Skene's duct cysts in 2 newborns and 1 adult in whom the cyst caused superficial external dyspareunia. A case of Skene's duct cyst is reported, which was associated with spraying of the urinary stream during voiding. Differential diagnosis includes urethral diverticulum, ectopic ureterocele, cystocele and Gartner's duct cyst.     

Two-stage transperineal management of posterior urethral strictures or bladder neck contractures associated with urinary incontinence after prostate surgery and endoscopic treatment failures

OBJECTIVES: The treatment of posterior urethral strictures or bladder neck contracture associated with severe urinary incontinence after prostate surgery and failure of endoscopic treatments is controversial. We report our experience with a transperineal approach in two steps: end-to-end urethroplasty/anastomosis and subsequent artificial urinary sphincter implantation. METHODS: Between September 2001 and January 2005, we observed six patients (58-68 yr old), with a combination of severe urinary incontinence and posterior urethral stricture with anastomotic bladder neck contracture after prostate surgery. In all cases, repeated endoscopic treatments of the strictures failed. The patients underwent transperineal end-to-end urethroplasty or anastomosis followed by transperineal artificial urinary sphincter placement after 6 mo. RESULTS: After the first surgical step, all patients were completely incontinent with absence of urethral strictures and complete anastomotic healing in all cases. Therefore, all patients underwent artificial urinary sphincter insertion. After a mean follow-up of 38 mo (range: 18-57 mo), five patients are continent with no postvoid residual urine and a perfectly functioning device. One artificial urinary sphincter was removed due to urethral erosion. CONCLUSIONS: In patients with posterior urethral strictures or bladder neck contractures associated with severe urinary incontinence, an artificial urinary sphincter implantation as a second step allows verification of the outcome of a previous end-to-end urethroplasty or anastomosis and utilizes a dedicated operative field to reduce the risks of prosthesis implants.