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.
Ureterocele ; Humans ; Femalec ; Uterine Anomalies ; Vagina ; Uterus

PURPOSE: Ureteral duplication is a relatively common congenital urinary tract abnormality that can be associated with various clinical problems such as vesicoureteral reflux (VUR), hydronephrosis, and ectopic ureters. The purpose of this study was to analyze the clinical characteristics of pediatric patients with recently diagnosed ureteral duplication and to identify any differences from those described in previous reports.METHODS: We retrospectively reviewed the clinical characteristics and course of pediatric patients who were diagnosed with ureteral duplication between January 2008 and June 2017.RESULTS: A total of 32 pediatric patients were diagnosed with ureteral duplication during the study period. The male to female ratio was 1:2.2. Twenty-seven patients (84.4%) were first diagnosed with ureteral duplication at less than 3 months of age, and 26 (81.3%) were first diagnosed by prenatal ultrasonography. Four of the 32 patients were diagnosed with bilateral ureteral duplication, for a total of 36 occurrences of ureteral duplication. In 17 occurrences of complete ureteral duplication (47.2%), other urinary tract anomalies were also found; namely, ureterocele (7), VUR (11), and ectopic ureter (5). However, none of the patients with incomplete ureteral duplication had ureterocele or VUR.CONCLUSION: With the advent of routine prenatal ultrasound, ureteral duplication is being diagnosed earlier than was previously possible, enabling timely treatment of the various accompanying urinary tract anomalies. Multicenter studies are needed to establish guidelines for standardized evaluation and treatment of ureteral duplication.
Child ; Female ; Humans ; Hydronephrosis ; Male ; Retrospective Studies ; Ultrasonography ; Ultrasonography, Prenatal ; Ureter ; Ureterocele ; Urinary Tract ; Vesico-Ureteral Reflux

Hydronephrosis is the most common urogenital anomaly with the potential for obstructive process. And ultrasonography, as a first-line imaging modality, is a useful tool for evaluation of prenatal and postnatal hydronephrosis, even though evaluation of renal function is still needed. In this article, we review the common causes of congenital hydronephrosis and the sonographic findings of those diseases.
Hydronephrosis ; Ureterocele ; Vesico-Ureteral Reflux

PURPOSE: This study assessed the incidence and outcome of congenital anomalies of the kidney and urinary tract (CAKUT) detected by prenatal ultrasonography METHODS: There were 906 cases of CAKUT detected by prenatal ultrasonography and postnatally confirmed at the Asan Medical Center from October 1989 to October 2007. We investigated the incidence and outcome of these cases by reviewing medical records retrospectively. RESULTS: The order of incidence was hydronephrosis, multicystic dysplastic kidney (MCDK), duplex kidney, vesico-ureteral reflux (VUR), single kidney, hydroureteronephrosis, ectopic kidney, polycystic kidney, ureterocele, and posterior urethral valve (PUV). There were 520 cases (57.4%) of hydronephrosis, and 20% of these needed an operation due to significant obstruction. MCDK was associated with other CAKUT in 25.4% of all cases. Approximately 57.9% of duplex kidney cases needed surgical treatment due to ureterocele and VUR. VUR had a male: female ratio of 10:1. Two out of seven cases of autosomal recessive polycystic kidney had progressed to chronic renal failure. Patients with PUV were relatively uncommon, and one out of nine cases progressed to end-stage renal disease. CONCLUSION: CAKUTs detected by prenatal ultrasonography were composed of various anomalies, and almost all of them had a good outcome without any intervention. However, in some cases, recurrent urinary tract infection or renal failure occurred, especially in bilateral cases. For further management, a long-term multicenter study is needed to investigate the precise incidence and outcome of each anomaly in the general population.
Female ; Humans ; Hydronephrosis ; Incidence ; Kidney ; Kidney Failure, Chronic ; Medical Records ; Multicystic Dysplastic Kidney ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Recessive ; Renal Insufficiency ; Retrospective Studies ; Ultrasonography, Prenatal ; Ureterocele ; Urinary Tract ; Urinary Tract Infections ; Vesico-Ureteral Reflux

A paraurethral cyst is a rare congenital or acquired abnormality in females that is characterized by a round, yellow or orange-colored cystic mass on either side of the urethral meatus. We report a patient who presented with frequent, postvoiding dribbling. The important differential diagnostic considerations in infants and adults are an ectopic ureterocele, urethral diverticulum, cystocele and paraurethral tumors. Intravenous pyelography, voiding cystourethrogram, cystourethroscopy are essential for differentiating these lesions. This case of a paraurethral cyst was treated with complete excision.
Adult ; Cystocele ; Diverticulum ; Female ; Humans ; Infant ; Ureterocele ; Urethra ; Urography

PURPOSE: Non conservative treatment such as heminephrectomy is considered the treatment of choice when the upper pole kidney in children with a complete ureteral duplication complicated with ureterocele or ectopic ureter is nonfunctional. The postoperative outcome of the upper pole kidney with a complete ureteral duplication after pyeloureterostomy was evaluated, and we focused on those children with undetected functions on the upper pole kidney before surgery. MATERIALS AND METHODS: Between May 2002 and March 2006, we evaluated 28 children, 10 boys and 18 girls, who had undergone pyeloureterostomy for a complete ureteral duplication that was complicated with ureterocele or ectoplic ureter. Their mean age was 12.3 months. Ultrasound, 99mTc-dimercaptosuccinic acid(DMSA) scanning and voiding cystourethrography(VCUG) were performed preoperatively. The clinical courses were followed up with ultrasound at 1, 3, 6 and 12 months, and DMSA scanning was done between 6 and 12 months after surgery. The median follow-up period was 21.5 months. RESULTS: Among the 12 children with cortical thinning seen on ultrasound, 10 showed thickening of the renal cortex within 12 months. All 16 children with Grade III or less hydronephrosis on the upper pole kidney were downgraded, and 10 out of them showed complete resolution or Grade I hydronephrosis within 6 months. 4 cases with no visible photon uptake at the upper pole kidney preoperatively showed hot uptakes at the defective spot between 6 to 12 months after pyeloureterostomy. 5 out of 28 children had urinary tract infection(UTI) within 3 months after surgery; however, this was easily controlled with oral antibiotics. CONCLUSIONS: The function of the upper pole kidney is recovered within 1 year after pyeloureterostomy in most of the cases with a complete ureteral duplication, regardless of the severity of hydronephrosis at the upper pole kidney. Pyeloureterostomy can be an alternative treatment modality for treating a complete ureteral duplication that's complicated with ureterocele or ectopic ureter due to pyeloureterostomy's lower morbidity compared to non-conservative surgery such as heminephrecotmy.
Anti-Bacterial Agents ; Child ; Female ; Follow-Up Studies ; Humans ; Hydronephrosis ; Kidney* ; Radioisotope Renography ; Succimer ; Ultrasonography ; Ureter* ; Ureterocele* ; Urinary Tract

PURPOSE: To review the clinical manifestations, indications and the management outcomes of adult patients with ureteroceles. MATERIALS AND METHODS: Between 1995 and 2006, 20 adult patients (9 females, 3 males) with ureteroceles were investigated for their clinical symptoms, type of ureterocele and renal function. The outcomes of surgical or conservative management, according to the patients' symptoms were also individually analyzed. The median follow-up was 38 months (12-50 months). RESULTS: The ages at diagnosis of the ureteroceles ranged from 19 to 70 years (mean 37.9 years). The ureterocele-related symptoms were flank pain (3), hematuria (1) and lower urinary tract symptoms (4). Two cases were incidentally detected with ultrasound (1) or computed tomography (1), and another 2 patients presented with non-specific flank pain or a hematuria. Eight patients exhibited an intravesical single system and 4 were associated with upper pole of a duplex system. Only one patient had an ectopic ureterocele, in which the orifice was located in the mid-urethra. The ureterocele-related symptoms were managed using a transurethral incision (5) or resection (1) of the ureterocele, with ureteroscopic stone retrieval (2). The symptoms were resolved after surgery, and there were no recurrence of symptoms or any deterioration of the renal function during follow-up. CONCLUSIONS: To diagnose an ureterocele in adult patients requires a high index of suspicion, as not all patients present with the typical clinical manifestations associated in children. Our results suggested that ureterocele-related symptoms are the main indication for surgery in adult patients. While methods with lower morbidity may be a useful, expectant treatment, they may also be an appropriate option for the management of incidentally detected ureteroceles.
Adult* ; Child ; Diagnosis ; Female ; Flank Pain ; Follow-Up Studies ; Hematuria ; Humans ; Lower Urinary Tract Symptoms ; Recurrence ; Ultrasonography ; Ureterocele*

Purpose: We reviewed our experience in the management of ureteroceles in children as we selected the surgical method according to the patients' age and the differential function of the affected renal unit. Materials and Methods: Thirty-six children (10 boys, 26 girls) with ureteroceles who received surgical management between 1991 and 2003 and were followed up for at least 12 months postoperatively were the subjects of this study. Age at operation ranged from 1 month to 10 years (median 6.5 months) and patients were followed up for an average of 43 months postoperatively (12-103 months). Results: Twenty patients had functioning renal units, 16 including 4 infants, of which received transurethral incision (TUI), 3 ureteroureterostomy and 1 ureteroneocystostomy after ureterocelectomy. Eight of the 16 patients (50%) who received TUI developed de novo vesicoureteral reflux (VUR), 5 of whom requiring secondary open procedure. Of the 16 patients with nonfunctioning renal units who received either heminephrectomy or nephrectomy, 6 required secondary open procedures for persistent ureteral obstruction or bladder outlet obstruction. On follow-up, of the 20 patients with initially functioning renal units, 19 maintained preoperative level of renal function and all remained free of urinary tract infection (UTI). Conclusions: In surgical approach to the ureteroceles, individualization with respect to the function of the affected unit and the age of the patient appears to be effective in controlling UTI and preserving the renal function. TUI, despite the high rate of de novo VUR, may be a useful option in immediate decompression of the obstructed urinary tract in the face of acute UTI especially in younger children.
Child* ; Decompression ; Follow-Up Studies ; Humans ; Infant ; Nephrectomy ; Ureteral Obstruction ; Ureterocele* ; Urinary Bladder Neck Obstruction ; Urinary Tract ; Urinary Tract Infections ; Vesico-Ureteral Reflux

PURPOSE: Our experience of upper moiety in the complete duplex system was retrospectively analyzed to determine its optimum management. MATERIALS AND METHODS: Between 1988 and 2003, 27 patients were treated with the complete duplex system. Fifteen patients had ureterocele (9 intravesical, 6 ectopic) and the other 12 had an ectopic ureter. In all cases, excretory urography, ultrasonography, voiding cysto-urethrogram (VCUG) and dimercaptosuccinic acid (DMSA) renal scan were performed. The initial treatment was performed using salvage (transurethral incision or ureteropyelostomy) or non-salvage procedures (upper pole nephrectomy or nephrectomy). The median follow-up was 30 (13-48) months. RESULTS: The 27 patients were divided into three groups based on the function of the upper moiety from the DMSA renal scan - <10%; 13 patients (group A), 10-15%; 3 patients (group B), > or=15%; 11 patients (group C). In group A, upper pole nephrectomy was performed in 9 patients, a total reconstruction in 3 and a nephrectomy in 1. In group B, an upper pole nephrectomy was initially performed, with an ureteropyelostomy and transurethral incision (TUI). In group C, all patients received a transurethral incision as the initial treatment. The ipsilateral renal function was well conserved in the cases of upper pole nephrectomy, with no complications. Patients initially receiving salvage procedures showed a significant improvement and conservation of the ipsilateral renal function, but 4 patients required additional operative management due to moderate to severe vesicoureteral reflux (VUR), recurrent urinary tract infection and decreased renal function of the upper moiety. CONCLUSIONS: Salvage procedures are a preferable adequate therapeutic modality for the complete duplex system with a well conserved renal function.
Follow-Up Studies ; Gene Duplication ; Humans ; Nephrectomy ; Retrospective Studies ; Succimer ; Ultrasonography ; Ureter ; Ureterocele ; Urinary Tract Infections ; Urography ; Vesico-Ureteral Reflux

PURPOSE: The most important management of congenital hydronephrosis consists of the early diagnosis and evaluation of the pathologic abnormalities of congenital hydronephrosis. This study was conducted to investigate the different causes of hydronephrosis and its clinical outcome. METHODS: 54 live neonates who were hospitalized and diagnosed with congenital hydronephrosis at Chungnam National University Hospital from Aug. 1998 to Aug. 2003 were retrospectively analyzed. RESULTS: Hydronephrosis(renal pelvic AP diameter >5 mm) was postnatally detected in 54 cases(2.1%) among 2,539 neonates who were hospitalized from Aug. 1998 to Aug. 2003. There were three times more males than females. Additional imaging studies revealed that ureteropelvic junction obstruction was the most common postnatal diagnosis(48.7%), followed by multicystic dysplastic kidney, vesicoureteral reflux and duplication kidney with ureterocele. Spontaneous regression of hydronephrosis was revealed in 25 renal units(75.8%) of mild hydronephrosis, 14 renal units of moderate hydronephrosis and 1 renal unit of severe hydronephrosis. Operative correction were carried out in 14 renal units(70%) of severe hydronephrosis. CONCLUSION: The most common established cause of congenital hydronephrosis in this study was ureteropelvic junction obstruction. There are many cases of spontaneous regression in mild to moderate congenital hydronephrosis. Urinary tract infections occur in many neonates with hydronephrosis. Therefore, early detection and evaluation of congenital hydronephrosis and continuous follow-up at regular intervals are necessary for conservation of renal function.
Chungcheongnam-do ; Early Diagnosis ; Female ; Follow-Up Studies ; Humans ; Hydronephrosis* ; Infant, Newborn ; Kidney ; Male ; Multicystic Dysplastic Kidney ; Retrospective Studies ; Ureterocele ; Urinary Tract Infections ; Vesico-Ureteral Reflux