Obstructed hemivagina with ipsilateral renal anomaly with ureteric remnant in a prepubertal female / Philippine Journal of Obstetrics and Gynecology

@#Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), or Herlyn–Werner–Wunderlich syndrome, is a rare Müllerian duct anomaly that is characterized by a triad of uterus didelphys, unilateral obstructed vagina, and ipsilateral renal agenesis. Patients with this anomaly usually present after menarche with abdominal pain or a pelvic mass which worsens over some time. Herein, presented is a case of a 12‑year‑old nulligravid with cyclic pelvic pain and palpable pelvic mass after her menarche. A series of diagnostic tests were done which were compatible with the diagnosis of OHVIRA with an associated finding of ectopic ureteral insertion into the obstructed hemivagina and nonfunctional urinary bladder. The patient underwent diagnostic hysteroscopy, cystoscopy, and full resection of the longitudinal vaginal septum as well as drainage of hematocolpos, which are the main treatment of the patient with OHVIRA.

A variant of Herlyn Werner Wunderlich Syndrome

@#Herlyn Werner Wunderlich Syndrome is a rare congenital Mullerian anomaly that commonly presents with uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis. Presented here is a rare variant with contralateral dysplastic kidney with ectopic ureteral insertion instead of renal agenesis. The patient initially presented as a case of recurrent pelvic inflammatory disease who eventually underwent excision of vaginal septum with drainage of pyocolpos. Postoperatively, the patient had urinary incontinence and was eventually referred to Urology for further surgical intervention. The wide range of symptomatology and the uncommon anatomic presentation of this case led to the delay in the diagnosis and the consequent gynecologic complications.

Ureter ectópico extramural em um cão Labrador Retriever: relato de caso / Ectopic ureter extramural in a Labrador Retriever dog: a case report

A ectopia ureteral é uma enfermidade congênita que se caracteriza quando um ou ambos os ureteres apresentam-se inseridos fora do seu local anatômico, com a inserção ocorrendo no útero, no colo da bexiga, na uretra ou na vagina, devido a uma diferenciação anormal dos ductos mesonéfricos e metanéfricos. Nos machos, a inserção pode ocorrer também nos ductos deferentes e na próstata. A incontinência urinária é o sinal clínico mais comumente associado ao ureter ectópico. O presente relato descreve um caso de ureter ectópico extramural unilateral, em um cão da raça Labrador Retriever de sete anos de idade, corrigido cirurgicamente, e mostra a importância dos exames radiográfico contrastado e ultrassonografia.(AU)

Ureteral ectopia is a congenital disease that is characterized when one or both ureters are shown inserted outside their anatomic site, with the possible insertion in the uterus, bladder neck, the urethra or vagina due to abnormal differentiation of ducts mesonephrics and metanephrics. In males the insertion can also occur in the vas deferens and prostate. Urinary incontinence is the most common clinical sign associated with ectopic ureter. This report describes a case of unilateral extramural ectopic ureter in a dog of the Labrador Retriever breed at seven years old surgically corrected, and shows the importance of contrast radiographic examination and ultrasound.(AU)

Vaginal ectopic ureter diagnosed by magnetic resonance imaging in a child with complete transverse vaginal septum and hydrocolpos

Ectopic ureter draining into the vagina is a rare cause of urinary incontinence. Some cases have been reported in association with malformations of the genitourinary tract, but very few published cases are associated with vaginal septum. Our article describes the case of a girl who presented with hydrocolpos shortly after birth and was found to have a complete transverse vaginal septum. Despite successful correction of this genital anomaly, the patient developed persistent dribbling of clear fluid from the genital area. Extensive testing revealed unilateral kidney dysplasia and probable genital ectopia of the corresponding ureter. Magnetic resonance imaging (MRI) showed insertion of the ureter into the upper third of the vagina. The patient underwent nephroureterectomy of the dysplastic kidney with subsequent improvement in urinary incontinence. Ureteral ectopia is difficult to diagnose with conventional imaging methods (plain radiography, ultrasound, and CT). MRI has proved to be an excellent method for assessment of genitourinary tract conditions, particularly when other diagnostic modalities have failed or are limited, due to the high resolution of MRI scans and the possibility of native image acquisition on all three orthogonal planes. MRI is the best diagnostic modality for visualization of the course and insertion of ectopic ureters, and enables precise surgical correction.

Ectopia ureteral unilateral congênita em uma cadela Teckel Dachshund com pelagem arlequim: relato de caso / Single congenital ureteral ectopy in a female dog Teckel Dachshund with merle colour pattern: case report

O presente relato apresenta a primeira descrição de ectopia ureteral congênita na raça Teckel Dachshund, diagnosticada em uma cadela com pelagem arlequim. O animal, aos dois meses de idade, apresentava sinais de incontinência urinária e cistite bacteriana, sendo submetido a um plano diagnóstico para confirmação de ureter ectópico. A urografia excretora revelou hidroureter direito com desembocadura caudal ao trígono da bexiga. O exame físico do animal evidenciou ainda hérnia inguinal bilateral, o que reforçou a caracterização da origem congênita das alterações. Aspectos de bem-estar animal são também discutidos.

The present work reports the first description of congenital ureteral ectopy in the canine breed Teckel Dachshund, diagnosed in a female dog with merle colour pattern. The two month old animal, presented with continuous dribbling of urine and bacterial cystitis, was subjected to a diagnostic plan for ectopic ureter. The excretory urography showed a right hidroureter, which had an orifice located caudally to the trigone of the bladder. The animal also presented a bilateral inguinal hernia, which confirmed the case description as a multiple congenital anomaly condition. Animal welfare aspects are also discussed.

First-line Treatment Modality in Children with Complete Duplicating System Complicated with Ureterocele or Ectopic Ureter: Ureteropyelostomy / 대한비뇨기과학회지

PURPOSE: A complete duplicated collecting system complicated with an ectopic ureter or ureterocele results in renal damage due to a ureteral obstruction or reflux. The clinical outcome of ureteropyelostomy in complicated complete duplicated collecting system was evaluated. MATERIALS AND METHODS: 20 children received a ureteropyelostomy for either an ectopic ureter or ureterocele with a complete duplicated collecting system anomaly. The median age was 3.2 months. Of the children, 11 had ureteroceles including 7 cecoureteroceles, and 9 had ectopic ureters. The associated anomalies were 3 UPJ obstructions of the lower pole and 9 VUR. All received a ureteropyelostomy. The lower pole ureter was used as the common ureter except in 2 cases with a lower ureter in poor condition. A lower pole pyeloplasty was combined in 3 cases with UPJ obstructions of the lower pole. A distal ureteral stump was left open except in the cecoureterocele in order to prevent VUR. Their clinical courses were followed up with an ultrasonogram and 99mTc-DMSA scan. The median follow-up after surgery was 17.8 months. RESULTS: A reduced upper pole hydronephrosis was observed in 19 patients and an improvement in the differential renal function was noted in 17 patients on the follow-up studies. All ureteroceles had either disappeared or were reduced in size. 7 patients showed an intermittent asymptomatic bacteriuria and 1 patient showed symptomatic UTI who required an ureteroneocystostomy during the follow-up. CONCLUSIONS: A ureteropyelostomy is recommended as a first-line treatment modality for patients with a complicated complete duplicated collecting system owing to the lower chance of secondary surgery, the prevention of deterioration in the bladder function and the possibility of early correction.

First-line Treatment Modality in Children with Complete Duplicating System Complicated with Ureterocele or Ectopic Ureter: Ureteropyelostomy / 대한비뇨기과학회지

PURPOSE: A complete duplicated collecting system complicated with an ectopic ureter or ureterocele results in renal damage due to a ureteral obstruction or reflux. The clinical outcome of ureteropyelostomy in complicated complete duplicated collecting system was evaluated. MATERIALS AND METHODS: 20 children received a ureteropyelostomy for either an ectopic ureter or ureterocele with a complete duplicated collecting system anomaly. The median age was 3.2 months. Of the children, 11 had ureteroceles including 7 cecoureteroceles, and 9 had ectopic ureters. The associated anomalies were 3 UPJ obstructions of the lower pole and 9 VUR. All received a ureteropyelostomy. The lower pole ureter was used as the common ureter except in 2 cases with a lower ureter in poor condition. A lower pole pyeloplasty was combined in 3 cases with UPJ obstructions of the lower pole. A distal ureteral stump was left open except in the cecoureterocele in order to prevent VUR. Their clinical courses were followed up with an ultrasonogram and 99mTc-DMSA scan. The median follow-up after surgery was 17.8 months. RESULTS: A reduced upper pole hydronephrosis was observed in 19 patients and an improvement in the differential renal function was noted in 17 patients on the follow-up studies. All ureteroceles had either disappeared or were reduced in size. 7 patients showed an intermittent asymptomatic bacteriuria and 1 patient showed symptomatic UTI who required an ureteroneocystostomy during the follow-up. CONCLUSIONS: A ureteropyelostomy is recommended as a first-line treatment modality for patients with a complicated complete duplicated collecting system owing to the lower chance of secondary surgery, the prevention of deterioration in the bladder function and the possibility of early correction.

Diagnosis and treatment of ectopic ureter in children (report of 22 cases) / 中华泌尿外科杂志

Objective To summarize the experience in diagnosis and treatment of ectopic ureter in children. Methods A total of 22 cases (21 females and 1 male;mean age of 4 years,range from 1 month to 12 years) of ectopic ureter were analyzed retrospectively.Of them,17 cases had unilateral ectopic ureters and 5,bilateral.Normal,divided urination accompanied by abnormal leakage of urine occurred in 18 cases;completely continous leakage of urine in 3;and obstruction of upper urinary tract with infection of urinary system in 1.Fourteen cases had opening of ectopic ureters in vaginas,4 in urethras,3 in vestibula and 1 in bladder necks. Results Of the 22 cases 20 underwent surgical procedures.Of the 20 cases,11 had total nephrectomy and 7,partial nephrectomy.After the operation,leakage of urine disappeared,but 3 cases had syndrome of ureteral stump remnant.One case who underwent bilateral ureter-bladder reimplantation had mild urinary incontinence after the operation.One case who had vesica replaced with rectum was followed up for 5 years,the post-operative result was excellent. Conclusions It is of critical importance to establish definite diagnosis and to select appropriate mode of operation for the successful treatment of ectopic ureter in children.

A pelvic kidney and a contralateral incomplete double collecting system with associated bilateral ectopic obstructive megaureters

A two-month old female child presenting with recurrent febrile urinary tract infection (UTI) was diagnosed to have a single-system pelvic kidney and a contralateral incomplete ureteral duplication with associated bilateral ectopic obstructive megaureters draining into the proximal urethra. Bilateral cutaneous loop ureterostomy was done at that time. Subsequently, she underwent undiversion, transuretero-pyelostomy (double left ureters to right pelvis), right ureteroneocystostomy, and augmentation ureterocystoplasty. Biopsy of the right distal ureter was consistent with megaureter with acute and chronic ureteritis. Repeat IVP post-op showed well-opacified kidneys with no signs of obstruction and a well-distended urinary bladder with moderate post-void residual urine. A high index of suspicion, coupled with meticulous physical examination and rationally combined diagnostic procedures are important to make a diagnosis of a unique combination of rare congenital urologic anomalies. (Author)

A Case of Seminal Vesicle Cyst associated with Ipsilateral Ectopic Ureter and Renal Agenesis / 대한비뇨기과학회지

Seminal vesicle cysts combined with ipsilateral renal agenesis represent a rare urologic anomaly. This urogenital malformation is explained as a developmental failure in the same mesodermal region. We report a case of seminal vesicle cyst, ipsilateral dilated ureter and renal agenesis which was treated by surgical excision. And we searched the literature to review the clinical presentation, diagnosis and treatment options of this anomaly.

Four Cases of Unilateral Single Ectopic Ureter with Ipsilateral Dysplastic or Hypoplastic Kidney in Children: the Diagnostic Value of MR Urography / 대한비뇨기과학회지

We report on 4 cases with a unilateral single ectopic ureter associated with ipsilateral dysplastic or hypoplastic kidney and the usefulness of preoperative magnetic resonance(MR) urography in revealing the ureteral course and detecting the insertion site of ectopic ureter. Among two cases of unilateral single ectopic ureter associated with dysplastic kidney, one drains into the seminal vesicle and the other drains into the urethra. The other two cases have unilateral single ectopic ureter associated with hypoplastic kidney that drain into the vagina. In all cases, MR urography shows the ureteral course and insertion site of ectopic ureter regardless of the renal function. We believe that MR urograhy is a useful technique for the diagnosis of unilateral single ectopic ureter associated with hypofunctioning kidney.

Unilateral Single Vaginal Ectopic Ureter with Ipsilateral Hypoplastic Kidney: A Case Report / 대한비뇨기과학회지

To date, the unilateral single vaginal ectopic ureter has been regarded as the rarest form of ureteral ectopia because 80 per cent of all ectopic ureters are associated with a duplicated system and most of the ectopic ureters occur in male patients. This malformation may take the form of but is not limited to any ombination of abnormal development of the mesonephric and paramesonephric ducts. We report a case of unilateral single vaginal ectopic ureter with ipsilateral hypoplastic kidney in a 9 year-old female patient who has been suffered from diaper dermatitis due to persistent urinary incontinence since birth.

Two Cases of Single Ectopic Ureter Associated with Ectopic Renal Dysplasia / 대한비뇨기과학회지

A single ectopic ureter associated with ectopic renal dysplasia is extraordinary rare. We report 2 cases, of which one was a girl with ectopic ureter entering the vagina and the other was a girl with ectopic ureter entering the urethra.

A case of single vaginal ectopic ureter with preserved function of ectopic kidney / 대한비뇨기과학회지

We report a case of single vaginal ectopic ureter with ipsilateral small, malrotated ectopic right kidney in 5 year-old girl. She presented with persistent urinary leakage while normal voiding since birth. Excretory urogram, vaginogram and vaginoscopy confirmed the presence of vaginal ectopic ureter. The right terminal ureter was reimplantated into the bladder extravesically. Postoperatively, she has been well for 5 months with grade II-III/V vesicoureteral reflux on the right side.

The Ectopic Ureteral Orifice / 대한비뇨기과학회지

We describe 6 case of ectopic ureteral orifice was on vaginal wall and the most frequent symptom of ectopic ureteral orifice was incontinence. Vagino-gram and indigo carmine test was very important in diagnosis of ectopic ureteral orifice and identification of ectopic ureteral orifice and then nephrectomy, partial nephrectomy with ureterectomy was rational in treating ectopic ureteral orifice combining loss renal function.

Management of Duplicated Ureters with the Ectopic Ureter Opening into the Vaginal Vestibule by Ureter Opening into the Vaginal Vestibule by Pyeloureterostomy: A Case Report / 대한비뇨기과학회지

We experienced a case of duplicated ureters with the ectopic ureter opening into the vaginal vestibule in a 8-year-old girl. She presented with symptoms of almost continuous dripping of urine from the vagina which started from her infancy. She did have her voluntary control of voiding through her normal urethra. The management of ectopic ureter in the duplicated system depends on the renal function, presence of vesicoureteral reflux, and renal dysplasia. We were able to preserve the function of the entire renal parenchyma in this patient by pyeloureterostomy.

A Case of Ectopic Ureter Entering Seminal Vesicle Associated with Ectopic Renal Dysplasia / 대한비뇨기과학회지

We presented one case of 43 years old man with ectopic ureter entering the seminal vesicle associated with ectopic renal dysplasia. He had complained of perineal and lower abdominal discomfort, hemospermia. The definite diagnosis was made by seminal vesiculogram through vas deferens. We report the case with brief review of literatures.

A Case of Unilateral Ectopie Ureter Draining to the Urethra and the Other Ureter Draining to the Bladder / 대한비뇨기과학회지

We presented one case of 21 years old girl with a unilateral ectopic ureter draining to the distal urethra. She had complained of the urine leakage since her infancy without other urinary symptom which she was treated with left heminephrectomy with ureter. We reported the case with literature review.

Renal Dysplasia with Single Vaginal Ectopic ureter: Report of 1 Case / 대한비뇨기과학회지

We report one case in which delayed enhanced computerized tomography (1.5 hr. later) contributed in detecting a dysplastic kidney with a single vaginal ectopic ureter that had been nonfunctioning on excretory urography and had not been detected by other diagnostic methods.

Ectopic Ureter: Report of II Cases / 대한비뇨기과학회지

An ectopic ureter inserts at a point other than superolateral portion of the trigone of the bladder. The diagnosis maybe suspected the girl who wets constantly with normal voiding and in the young boy with epididymitis. Based on our experiences with II ectopic ureters operated on during the last 14 years, the diagnostic findings and the surgical approaches were discussed. The results were as follows. 1. There were 10 single ectopic ureters and one ureter with completed duplex system. 2. All cases were female, of them nine were between 2 and 10 years old at the time of operation. 3. Urinary incontinence was the most common presenting symptom. 4. IVP showed ipsilateral non-visualized kidney in 6 cases, moderate hydronephrosis in 3 and normal kidney in 2. 5. Operations were performed with ureteroneocystostomy in 7 cases, nephrectomy and partial ureterectomy in 3 and nephrectomy and total ureterectomy in one. 6. Histopathologic findings in 4 nephrectomized cases demonstrated renal dysplasia in 3 and severe chronic pyelonephritis in one.