Urethral Duplication Research Articles

Epispadic accessory urethra in an 8 months old female with urethral duplication: a case report

Introduction and Importance: Urethral duplication is an uncommon urogenital congenital disability that presents as an accessory urethra in an atypical location. This condition is often coupled with other congenital anomalies like bladder exstrophy, pseudohermaphroditism, and renal disorders. Case Presentation: We report a case of a female infant aged eight months with urethral duplication, showing an unusual urinary opening in the prepubic area and urinary discharge. A comprehensive diagnostic assessment, consisting of a clinical exam, imaging tests, and cystoscopy, verified the existence of an accessory urethra and the normal one in epispadias. The patient had a successful surgery to remove the accessory tract with primary closure. Clinical Discussion: Urethral duplication is associated with diverse clinical signs and symptoms. Accurate diagnosis needs thorough imaging studies, and treatment should be tailored according to the type and anatomical location of the accessory tract. Conclusion: The histological confirmation of urethral duplication was obtained following the surgical excision of the additional tract. This case underscores the significance of accurate diagnosis and treatment approaches to manage this case.

A new spectrum of neonatal urethral pathologies in the era of early vesicoamniotic shunting?

Intrauterine vesicoamniotic shunting (VAS) was shown to affect survival of male fetuses with megacystis in suspected lower urinary tract obstruction (LUTO). Data on postnatal management are largely lacking. We aim to describe the pathologies diagnosed in children born after vesicoamniotic shunt placement in early pregnancy for megacystis. All newborns with previous intrauterine VAS treated in our institution were analyzed retrospectively. We evaluated the clinical spectrum of urethral pathologies. We also compared patients who received a shunt before the 17th gestational week with those who received it later. Between 2014 and 2023, 26 patients (all male) with a history of VAS for suspected LUTO were treated in our institution postnatally. Five fetuses with dislocated shunts underwent re-implantation in utero. Overall, premature birth before the 38th week of gestation was observed in 14 patients. Seven patients received a Harrison® shunt whereas 19 received a Somatex® shunt. Twelve patients required surgical shunt removal under general anesthesia due to shunt migration/embedding. Posterior urethral valves were found in 10/26 patients, 10/26 patients showed a urethral hypoplasia [Fig.1] and two patients had urethral duplications. In two patients, we identified a prune belly syndrome. One patient had posterior and anterior urethral valves. One patient had a high grade bilateral vesicoureteral reflux without LUTO. The 11 patients shunted early (before 17GW) showed a trend towards a higher proportion of urethral hypoplasia in the early shunt group (54% vs. 26%) without statistical significance. In our observation, patients treated with VAS had a noticeable high proportion of complex urethral pathologies such as urethral hypoplasia. These data should be taken into consideration for prenatal counselling of parents and planning of postnatal management.

Bladder duplication in the male cat: the first case report in China

BackgroundBladder duplication is a rare congenital lower urinary tract anomaly disease characterized by the presence of two bladders, possibly with duplication of the urethra. This disease is rarely reported in cats. The clinical symptoms are commonly occult, with increased difficulty in making a definitive diagnosis, especially if there is no obvious urethral duplication. The diagnosis is typically based on radiographs and ultrasound, with computer tomography serving as a more advanced imaging diagnostic modality. Cases of duplicated bladders with accessory tubular tissues are even scarcer in both human and veterinary medicine.Case presentationA 6-year-old male neutered cat was brought to the hospital because of vomiting and constipation. Cystography revealed increased soft tissue density of a fusiform structure in the lower middle abdomen. The purulent-filled cavitary structure and the accessory tubular structure were removed via surgery, and histopathological examination confirmed a double bladder with attached accessory tubular tissue. After antibiotic treatment, the cat recovered uneventfully.ConclusionThis is the first case of bladder duplication in China and the first case of feline bladder duplication with tubular structure attachment in the world. This information will provide a reference for the diagnosis and treatment of similar cases in the future.

Urethral duplication with a large cystic dorsal penile mass in a newborn: a case report

BackgroundUrethral duplication is a rare urogenital anomaly with varying anatomical orientations leading to diverse clinical presentations. We present a case of urethral duplication in a neonate featuring a large dorsal cystic mass on the penis, an unusual presentation.Case presentationA term male neonate with a prenatally diagnosed 10 × 10 cm genitourinary cystic mass was delivered via caesarean section. Examination revealed a large cystic mass extending dorsally from the pubic symphysis over a flattened, elongated penile shaft with a single urethral opening in the glans and undescended testes. A size 6-French feeding tube inserted into the urethra drained the bladder.Urethral communication with the cystic mass was confirmed via voiding cystourethrogram and cyst enlargement noted during voiding. Cyst fluid analysis indicated a urinary origin. Computed tomography and ultrasound were not diagnostic. Initial imaging revealed a dorsal cystic mass projecting from the pubic symphysis without bladder connection.Surgical intervention at 3 weeks revealed a Type IIA-2 urethral duplication, with a dorsal hypoplastic urethra communicating with the posterior urethra. Correction included resection of the dorsal urethra, cyst excision, and reconstruction of the penis with the orthotopic ventral urethra and bilateral orchidopexies.Satisfactory functional and cosmetic outcomes were observed at 2 and 8 months after surgery.ConclusionThis case highlights the significance of identifying unique urethral duplication presentations. The novel occurrence of Type IIA-2 urethral duplication terminating in a dorsal cystic mass underscores diagnostic complexity, surgical intricacies, and aesthetic considerations associated with such cases.

A novel alpha-numeric classification of urethral duplication and triplication.

A novel alpha-numeric classification of urethral duplication and triplication.

Role of Cysto-urethroscopy in the management of congenital urethral duplication in pediatrics. A case Report

Role of Cysto-urethroscopy in the management of congenital urethral duplication in pediatrics. A case Report

Urethral multiplicity in boys: systematic review of case reports and case series from the last 15 years.

Urethral multiplicity is a rare congenital anomaly characterized by the presence of two or more urethral channels. It is more common in males and can cause double urinary stream, incontinence, obstruction, and recurrent urinary infections. Diagnosis is difficult due to diverse clinical manifestations. Implementing an evidence-based treatment plan is challenging due to the need for more concise and informative summary publications. Our paper provides a comprehensive review of the management of this pathology and might serve as a valuable resource for pediatric urologists and specialists in the field. A comprehensive search in four electronic databases, PubMed®, PubMed Central® (PMC), Scopus, and Clarivate Analytics's Web of Science (WoS), was conducted to identify case reports and series published between 2008 and 2023 on urethral multiplicities. The quality of the articles was assessed using qualified instruments. Covidence® tool-guided synthesis was followed by individual patient data extraction. Further classifications and analysis were made using Microsoft Excel®. Out of the 90 papers included in the review, 62 were case presentations, and 28 were case series. We found 250 boys with urethral multiplicity. Based on Effman's classification, there were 38 cases of type I (15.3%), 21 type IIA1 (8.4%), 55 type IIA2 (22.1%), 91 type IIA2Y (36.5%), 4 type IIB (1.6%), and 6 type III (2.4%) urethral duplications. There were 19 cases of prepubic sinuses (7.6%), 9 triplications (3.6%), and 6 unknown forms (2.4%). We have provided data for each type, including clinical presentation, investigations, surgical management, and outcomes. Urethral multiplicities are a rare and varied group of malformations that require high-quality imaging examination for successful management. Treatment is specific to each patient and may depend on the surgeon's preference or skill. https://www.crd.york.ac.uk/prospero/display_record.php?ID = CRD42023471685, identifier (CRD42023471685).

Anterior Sagittal Epispadias Urethral Duplicity: A Case Report

Urethral duplication is a rare congenital urological anomaly that can be detected incidentally or due to urinary symptoms in young patients. Symptoms may include recurrent urinary tract infections, dysuria, bifurcated urinary stream, urinary incontinence, and purulent discharge from the accessory meatus. The pathophysiology remains poorly understood, with various embryological theories proposed. Treatment typically involves surgical excision of the accessory urethra, especially in symptomatic cases. Urethral duplication is often associated with other malformations, and Effman's classification system categorizes it into three types based on the location of the accessory urethra. We present the case of a 4 years old boy with anterior sagittal epispadias urethral duplicity.

Bladder Neck Surgery is not Routinely Needed to Achieve Urinary Continence in Patients with Primary Epispadias

BackgroundThis study aims at investigating the continence outcome in primary epispadias patients treated at a tertiary center. The authors hypothesized that additional continence procedures following primary epispadias repair is not routinely needed. MethodsPatients treated for primary epispadias at the authors’ institution between 2007 and 2019 and toilet trained, were identified from a prospective maintained database. Males underwent chordee correction, urethroplasty and glanuloplasty. Females underwent genitoplasty with reduction urethroplasty. If continence was not achieved by 4–5 years of age, pelvic floor muscle (PFM) biofeedback therapy was performed. Other continent procedures were discussed with family/patient if still incontinent. Primary outcome: urinary continence. Secondary outcomes: PFM biofeedback therapy, continence surgery, hydronephrosis. Type of epispadias, age at repair and follow-up presented as median was also reported. ResultsThirty-three patients (29 males) were included. Twelve had penopubic epispadias, 13 glanular/penile, 4 duplicated urethra, 4 females. Median age at repair: 2 years (IQR 1–3), at follow-up: 8 years (IQR 6–10). Daytime continence: 100 % in penile/glanular; 33 % in penopubic and 75 % in duplicated urethra. Nighttime continence: respectively 92 %, 50 % and 100 %. 24 % of males were intermittently incontinent. All patients except one voided urethrally. One patient underwent bladder neck closure, ileocystoplasty and Mitrofanoff. One girl achieved daytime continence, 2 were intermittently incontinent, one continuously incontinent. All were enuretic. 38 % of boys and 100 % of girls had biofeedback therapy. None had hydronephrosis/renal impairment. ConclusionsMost children with primary epispadias can achieve social urinary continence spontaneously or with the support of PFM biofeedback therapy. Other continence procedures should be reserved for patients who do not attain satisfactory continence. Level of EvidenceTreatment study – level IV.

Urethro-urethrostomy for urethral duplication (Type IIA1) in a 3-years-old boy: Surgical approach

To report a novel maneuver of end-to-side urethro-urethrostomy for managing Type IIA1 urethral duplication (UD). A 3-years-old boy was referred to our institute for abnormal appearance of genitalia. Physical examination revealed an epispadiac meatus on the dorsum of the penile shaft, in addition to the orthotopic meatus at the tip of glans. He can void through both urethrae with continence (grade I). Voiding cystourethrography and the cystoscopy confirmed the Type IIA1 UD with two urethrae arising independently from the bladder neck. A novel maneuver of end-to-side urethro-urethrostomy transferring the dorsal urethra through the corpus cavernosa and anastomosing it to the posterior wall of the ventral urethra was successfully performed. The urethral catheter was removed 2 weeks postoperatively. Neither urethral stricture nor fistula was noticed. After 1 year of followed-up, the boy can void fluently with continence (grade I). The Qmax was 10.4ml/s. Our maneuver of end-to-side urethro-urethrostomy for managing Type IIA1 UD was safe and effective, especially for the continent cases with the ectopic meatus on the penile shaft.

Postnatal surgical treatment and complications following intrauterine vesicoamniotic shunting with the SOMATEX® intrauterine shunt. A single center experience

Intrauterine vesicoamniotic shunting (VAS) using a Somatex® shunt was shown to significantly affect survival of male fetuses with megacystis in suspected lower urinary tract obstruction (LUTO) [Figure1]. Data on postnatal surgical management and complications are largely lacking. To describe the postnatal management of patients with prenatal VAS for megacystitis in suspected severe LUTO. All male newborns with previous intrauterine VAS using a Somatex® shunt treated in our institution were retrospectively analyzed. We evaluated the spectrum of urethral pathologies and postnatal surgical management, especially focusing on shunt removal. Between 2016 and 2022, 17 patients (all male) were treated postnatally in our institution after VAS for suspected severe LUTO. Five fetuses with dislocated shunts underwent re-implantation in utero. Overall, premature birth before the 38th week of gestation was observed in eight patients (8/17). Seven shunts could be removed without further anesthesia as a bedside procedure. Ten patients required surgical shunt removal under general anesthesia due to migration (59%). Laparoscopic shunt extraction was performed in 8/10 cases. Most frequently, dislocated shunts were located incorporated in the detrusor in eight cases and the removal required a bladder suture in 2/8 patients. In one case, the shunt was removed from the abdominal wall and in one case from the intestine wall [Figure2]. Posterior urethral valves were found in 8/17 patients, 6/17 patients showed a urethral atresia and one patient had urethral duplication. In two patients, we identified a high grade bilateral vesicoureteral reflux without LUTO. In our observation, more than half of the newborns with megacystis in suspected LUTO require a shunt removal surgery after early VAS using a Somatex® shunt. Urethral atresia may be found more frequently in these patients. These data should be taken into consideration for prenatal counselling of parents and planning of postnatal management.

The so-called Y-type urethral duplication: anatomical insights through controversial terminology

BackgroundY-type urethral duplication is a term frequently used to describe a rare condition in the male associated with double urinary stream: a weak interrupted urinary stream through a hypoplastic penile urethra, while the main urine flow is through the anus/perineum via accessory posterior channel. Fortunately, the advent of MRI has provided a powerful tool to study these anomalies in depth and on multiple planes.The study included boys presenting with abnormal micturition through the anus beside weak interrupted urine stream through the penis. Investigations included pelviabdominal ultrasound to screen for possible associated upper urinary tract anomalies, conventional contrast X-ray studies, and pelvic MRI.ResultsDuring the period between 2016 through 2021, the study included two newly diagnosed cases with abnormal communication between the lower urinary tract and the anus (the so-called Y-type urethral duplication), in addition to one previously reported case whose preoperative imaging studies (including pelvic MRI) were available for analysis. Different surgical solutions have been applied for each case. Hypoplasia was not only restricted to the penile urethra but also affected the corporeal bodies of the penis with variable degrees. In two cases, only a single corporeal body could be identified. In another case, the three corporeal bodies were present but appeared distorted (irregularities and interruption of corporeal bodies).ConclusionIn our present study, pelvic MRI examination clearly unveiled significant degrees of corporeal dysgenesis among cases diagnosed as Y-type urethral duplication that would strongly suggest these cases to belong to the same disease spectrum of penile dysgenesis/agenesis.

Cystoscopic Guided Laser Cauterization in a Dog with Complete Y-Type Urethral Duplication

Duplication of the urethra is a rare congenital malformation. A 14-month-old, sexually intact, male, Lagotto dog with complete Y-type urethral duplication was subjected to accessory urethra treatment for cystoscopic guided laser cauterization, with a 10-Watt diode laser with 550-micron fiber and 2.3 Fr outer diameter. The laser cauterization, that was repeated every 14 days for a total of three times, was performed by inserting the instrument from the accessory urethra outlet in the perianal area until it could be seen by the urethroscope, inside the ischial urethra. Nevertheless, this technique that proved to be non-invasive, less expensive than surgery, of short duration, and safe, did not allow the complete closure of the abnormal urethral tract. Therefore, the subsequent surgical removal of the accessory urethra was carried out. To the authors' knowledge this is the first report on the use of cystoscopic guided diode laser cauterization for accessory urethra treatment.

Pseudodiphallia with Effman type IIA2 urethral duplication: A case report and literature review

Pseudodiphallia with Effman type IIA2 urethral duplication: A case report and literature review

Urinary Lithiasis Secondary to Urethral Duplication: A Case Report

Urinary Lithiasis Secondary to Urethral Duplication: A Case Report

Technical details and long-term outcomes of P.A.D.U.A. for congenital urethral narrowing; a case series and review of the literature

The P.A.D.U.A. technique is a method of addressing congenital urethral narrowing. It involves passive dilation with a series of progressively larger indwelling catheters. Utilization is limited by scant literature, particularly regarding technical details and long-term durability. Tools for achieving safe and reliable urinary drainage are critical in these patients, who require careful stewardship of their kidney and bladder function. To describe long-term urethral patency and urinary function following P.A.D.U.A., and to provide sufficient technical detail to reproduce the technique. Patients with congenital urethral narrowing managed with P.A.D.U.A. were identified and chart review was performed. Details of catheter exchange sequences were compiled and described. The primary outcome was the attainment of adequate urethral caliber by successful completion of P.A.D.U.A., and the secondary outcome was voiding per urethra at most recent follow-up. P.A.D.U.A. achieved adequate urethral caliber in 9/11 (82%) of patients. This included seven patients with Prune Belly Syndrome, one with isolated urethral atresia, and one with a cloacal anomaly. P.A.D.U.A. failed to achieve urethral patency in one patient with urethral duplication, who was unable to progress through the catheter sequence, and one patient with Prune Belly Syndrome, who completed P.A.D.U.A. but developed recurrent narrowing one week later. There were no delayed failures of urethral patency. Patients who achieved patency underwent a median of seven catheter placements over 92 days. Median (range) initial and final catheter sizes were 3.5F (1.9-8F) and 14F (8-16F). While 82% achieved patency, only 3/11 (27%) were voiding spontaneously per native urethra at most recent follow-up. This series of patients undergoing P.A.D.U.A. for primary treatment of congenital urethral narrowing is the largest to date and provides granular technical details. It aligns with prior reports suggesting that P.A.D.U.A. achieves urethral patency in most patients with Prune Belly Syndrome or isolated urethral atresia, but has limited application in the hypoplastic duplicated urethra. Despite high rates of urethral patency in this select population, many patients will not achieve and maintain spontaneous voiding without catheterization. This is likely due to ongoing deterioration of bladder function caused by the prenatal developmental insult, paralleling the phenomenon seen in posterior urethral valves. P.A.D.U.A. is an effective and durable technique for achieving urethral patency. However, due to complicating factors such as the underlying bladder pathology present in many patients, urethral patency achieved with P.A.D.U.A. does not guarantee long-term safe and reliable spontaneous emptying per urethra.

A Rare Case of Complete Urethral Duplication and Short Review of the Literature

Abstract Duplication of the urethra is a rare congenital anomaly, predominantly seen in males. Due to the rarity of the condition, there is no fixed consensus regarding the management of these patients. We report the case of a 4-year-old male child who presented with phimosis and balanoposthitis, with the occasional double stream of urine and was incidentally found to have duplication of the urethra. Cystoscopy revealed the duplication to be Effman Type II A-1. No surgical intervention was done for the duplication as the patient was asymptomatic. Our aim is to report this rare case and to enhance the knowledge by reviewing the already existing scanty literature of this rare condition.

Missed urethral duplication associated with hypospadias, post-hypospadias repair urethral stenosis, and recurrent urinary tract infections in an adult: a case report

BackgroundDuplication of urethra is a very rare congenital disorder. Several types of this anomaly have been reported around the world, and are also discussed in this report. However, the mechanism of this anomaly is still unclear.CaseA 45-year-old Persian man with a complaint of recurrent urinary tract infection was referred to our clinic. He had a history of repairing penoscrotal hypospadias in childhood along with obstructive and irritating symptoms in adolescence. On his last voiding cystourethrogram and retrograde urethrogram, stenosis was observed in the proximal bulbar urethra along with a double urethra in the dorsal region of the main urethra. The double urethra was removed with operation, and he was followed for 1 month after surgery. He had no complaints of recurrence or urinary incontinence.ConclusionsThis report shows the different classification systems, types of double urethra, and approach and management, which mainly involves surgery; however, surgical management should be done according to the anatomical findings of the abnormality.

Congenital anterior urethral valves and diverticulum: A case series and association with posterior urethral valves

There is limited data regarding long-term results and associated complications in patients with anterior urethral valve (AUV) and diverticulum (AUD). We retrospectively reviewed AUV/AUD cases managed by us between the year 2002-2020. Presentation, investigations, management, concomitant posterior urethral valves (PUV) and pre-operative characteristics predisposing to long-term poor renal outcome were assessed. There were 27 patients [AUV (n=11); AUD (n=16)] with 5 having concomitant PUV. All presented with poor urinary stream and dribbling at a median age of 1-year (5 days-12 years). More patients with AUD (9 of 16, 56.3%) especially with concomitant PUV presented at ≤1-year-age as compared to those with AUV (4 of 11, 36.4%). Concomitant PUV and AUD cases (n=3) had characteristic micturating cystourethrography (MCUG) features. (Fig. 1A) Retrograde urethrography delineated the valve and distal urethra dimensions better (Fig.1B, C). Urethral hypoplasia distal to the valve (n=4) urethral duplication (n=1) were associated (Fig.1D). Syringocele was ruled out in proximal AUD by absence of filling defect on MCUG and appearance of urethral walls on urethrocystoscopy. Serum creatinine > 1mg/dL (n=5), trabeculated bladder (n=12), and vesicoureteral reflux (VUR) (n=12) was noted at presentation. Diverticulum/valve excision (n=13, 48.2%), fulguration alone (n=12, 44.4%) and primary urinary diversion (n=2, 7.4%) were performed. Follow up (range:3 mo-19 years), showed deranged renal function tests (n=6), VUR (n=4), impaired renal function on scans (n=8), and lower urinary tract dysfunction (n=7). Outcome with and without associated PUV was similar. Long term results were better in AUV compared to AUD. Pre-operative raised serum creatinine (>1mg/dL), trabeculated bladder, non-dilated posterior urethra on MCUG and bilateral impaired renal function on scans had significant association with follow up eGFR less than 60ml/kg/min. Co-existing PUV and AUD present earlier and have specific imaging findings. In proximal AUD, possibility of syringocele should be kept in mind, as they have similar presentation and imaging. Concomitant PUV did not alter prognosis. Secondary effects on bladder and renal function were more with AUD. Follow up eGFR less than 60ml/kg/min was associated with pre-operative elevated serum creatinine, trabeculated bladder, non-dilated posterior urethra, and bilateral impaired renal function on scans.

Urethral duplication associated with complex chordee: a narrative review of literature and report of a case

BackgroundUrethral duplication (UD) is reportedly rare. It is rarer in females. Knowledge on this anomaly comes from isolated report of cases. The aim of this review is to summarize information available on this anomaly thereby revealing gaps in knowledge, and to appropriately situate a recently managed case.MethodsPublications on UD in English language from 2001 to 2021 were searched for in the literature. Of importance were age at presentation, class of UD, nature of associated penile deformity and other structural anomalies. Available information was used to synthesize opinions after descriptive analyses using SPSS® version 21 (IBM Co., Armonk, NY, USA). In addition, a recently managed case of UD was reported and appropriately situated in the discourse.ResultIn all, 115 articles met the inclusion criteria. Majority (75.7%) were individual case reports, while the rest were retrospective review of case series. These provided a total of 269 reported cases. Of this lot, 38 cases were excluded because they either had no Effmann’s class or were not described well enough for an Effmann’s class to be assigned. Ultimately, 231 cases formed the basis for this review. Male to female ratio was 12:1. Types III and IIB had more females. Types IIA 2 (26.0%), IIA 2 “Y” (26.4%) and IA (22.5%) were frequently reported. About 61.8% males and 68.4% females had no associated defects. Isolated dorsal chordee was prevalent (7.1%), especially among type IA (16.7%) UD. Reported in 10.4%, vesicoureteric reflux may not be attributable always to high pressure voiding.A boy who presented at the age of 18 years with Effmann type IA UD and an associated complex chordee of the penile shaft was reported. The complex nature of the chordee adds to the challenge of explaining associated penile defects in UD.ConclusionUD is rare, but reported from all parts of the globe. There are a number of associated defects involving the external genitalia and other organs reported in UD in the male. The mechanisms of these defects are yet to be fully understood.