Urethral Duplication Research Articles

Pre- and postnatal imaging of a girl with a cloacal variant

We describe the prenatal MR findings in a 29-week fetus with a cloacal variant (urogenital sinus and anterior placed anus) in combination with an enlarged clitoris and urethral duplication and correlate them with postnatal imaging. Fetal MR imaging permits the diagnosis and characterization of cloacal and urogenital sinus malformations in utero. This information may guide pre-, peri- and postnatal management.

Urethral Duplication in Association with Anorectal Malformation

Urethral duplication is an uncommon congenital anomaly, not often reported, which may be partial or complete. Anorectal malformations are not as uncommon and they may be associated with a host of associated anomalies. However, the association of urethral duplication with anorectal malformation is rare; this report describes two such cases. In one case the intervening septum could be incised endoscopically and in the other case the duplicated urethra was excised.

Congenital Patent Urachus Associated with Incomplete Urethral Duplication: A Rare Association

Congenital patent urachus (CPU) is an uncommon anomaly. Blind incomplete urethral duplication is encountered more frequently than other types of urethral duplication (UD) in clinical practice. We describe a child with CPU in association with distal blind incomplete UD. A possible pathogenesis of this extremely rare coexistence is discussed.

Embryologic preputial sinus remnant mimicking urethral duplication

Urethral duplications are rare congenital anomalies. Approximately 200 cases of urethral duplications have been reported in literature. We present a seven-year-old-boy with an embryologic preputial sinus remnant which was mimicking urethral duplication. The clinical presentations and embryology of the urethral duplication and preputial sinus are discussed.

Y‐type urethral duplication in the male

To present a single series of Y-type duplication with an analysis of the presenting features, the management and outcome, as complete Y-type urethral duplication in the male is a rare congenital anomaly that presents many challenges. We retrospectively reviewed patient charts, identifying those with urethral duplication, and then those with complete Y-type duplication. The age at presentation, investigations, management and outcomes were reviewed. Of 49 patients with a diagnosis of urethral duplication, 13 had the complete Y-type. Three were lost to follow-up and were therefore excluded from the study. The median (range) age at presentation was 2 months (birth to 10 years). All patients had other comorbidities, with anorectal malformations in seven, renal agenesis in four, sacral agenesis/partial agenesis in two, and unilateral/bilateral cryptorchidism in four. Presenting features included passage of urine perineally or rectally in seven, and dribbling from the orthotopic urethral meatus in one. The diagnosis was confirmed by micturating cysto-urethrography. A mean of 14 procedures was required to achieve a final complete repair, and included urethral reconstruction, repair of fistulae, urethral dilatation, and evaluative cystoscopy. Excluding cystoscopy and urethral dilatation, a mean (range) of 3 (1-5) reconstructive procedures was required in each patient. The median follow-up was 8 years. Five boys developed urethral strictures and one developed multiple fistulae. The best outcomes were in boys who had a staged urethral reconstruction. Due to the strong association with other congenital anomalies we recommend that all patients should be evaluated thoroughly, including a detailed physical examination, renal tract ultrasonography and spinal radiography. Surgical management remains a significant challenge, requiring many procedures with unpredictable outcomes, the goals of which should be to maintain continence and reconstruct the urethra with good cosmesis. The optimum management scheme must be individualized in this rare condition.

A complex case of megalourethra and its repair

Megalourethra is a rare malformation of the urethra caused by a lack of corpus sponigosum and in some cases corpora cavernosa in the region of the distal urethra. The absence of these structures causes a ballooning of the urethra despite there being no mechanical obstruction. A male child presented with so-called fusiform megalourethra, with absence of the corpora cavernosa and urethral duplication. A voiding cystourethrogram was used to diagnose a fusiform megalourethra with pronounced meatal stenosis and extreme stenosis of pendulous urethra. In addition, there was urethral duplication in the form of an accessory urethra stretching from the urethral colliculus to the perineum. Absence of the corpora cavernosa was also suspected in the distal urethra. The surgical procedure involved pendulous urethroplasty with an onlay technique using urethral duplication and penile reduction. This method of treating megalourethra has not been previously reported. The operative technique for fusiform megalourethra with genital malformation has to be tailored to each individual case, depending on the intraoperative and endoscopic findings.

Laparoscopically Assisted Anorectal Pull-Through for High Imperforate Anus: Three Years' Experience

We describe our three-year experience with the laparoscopically assisted anorectal pullthrough for high imperforate anus using laparoscopic muscle electrostimulation. From March 2001 to January 2004, 7 patients with a diagnosis of high anorectal malformation underwent laparoscopically assisted anorectal pull-through. The patients, all males aged from 4 to 9 months (mean age, 5.8 months), presented with a rectourethral fistula. The associated malformations noted were sacral malformation, laryngeal stenosis, urethral duplication, multicystic kidney, nonpalpable testis, and esophageal atresia. All patients were treated with a colostomy in the newborn period followed by a delayed laparoscopically assisted anorectal pullthrough. Laparoscopy included stimulation of the puborectal muscle, using a modified Peña electrostimulator introduced through a trocar. All patients underwent a postoperative period of anal dilatation. In 6 cases the laparoscopically assisted anorectal pull-through was successful; there was 1 conversion to the open technique, due to strong tension from the colostomy. Although longer follow-up to evaluate continence is to come, laparoscopically assisted anorectal pull-through should be considered for the correction of the high imperforate anus and, according to our experience, it represents the gold standard. It offers the advantage of good visualization of the fistula and the surrounding structures and minimally invasive abdominal and perineal wounds. With the laparoscopic Peña stimulator the direct observation of the contraction of the puborectalis sling allows an evaluation of the functional contractility and an accurate colonic pullthrough in the center of the muscle complex.

A new case of male Y-type urethral duplication and review of literature

Urethral duplication is a rare congenital anomaly, affecting mainly boys. Generally, the duplication develops on the sagittal plane; the accessory urethra may run dorsally or ventrally to the orthotopic one. The embryology of this pathology is still controversial. In the English medical literature, approximately 175 cases have been reported. We report here a new case of urethral duplication type II A 2 “Y” in a 14-year-old boy and review the current literature of this rare entity.

An exceptional case of urogenital sinus associated with a scrotal pouch and duplicate urethra in a female child

We report the case of an unusual genitourinary anomaly in a 5-year-old patient. The malformation consisted of a para-anal persistent urogenital sinus in a 46XX patient associated with a scrotum-like structure in the presumed clitoral location. A duplicate urethra arising from the anterior aspect of the bladder neck drained into the scrotal pouch. Two hemivaginas drained into the urogenital sinus. The patient was totally continent through the urogenital sinus but the duplicate urethra was incontinent. No hormonal problems were encountered. The girl underwent total urogenital sinus mobilization, scrotal pouch ablation, and duplicate urethra resection.

Intermittent bladder urinary retention in a young woman: an unusual presentation of partial urethral duplication

A singular case of female accessory urethra associated to a mobile bladder stone with an unusual clinical presentation is reported. The role of perineal ultrasound is emphasized as a useful diagnostic tool to study female urethra in a static and dynamic setting, including cases of partial or complete urethral duplication.

A rare presentation of abortive epispadiac urethral duplication and vanishing midurethra

We report a 4-year-old boy with a hitherto undescribed case of abortive epispadiac duplication of the urethra presenting as a blind-ending penopubic sinus proximally and a mucosa-lined groove overlying the glanular corona distally but with absent midurethra. The patient was treated for cosmetic reasons with good results. A plausible embryological genesis of this defect is discussed.

Tubularized incised plate urethroplasty for abortive hypospadiac urethral duplication

Urethral duplication is among the rarest congenital anomalies and is characterized by two urethral canals. The urethral canals are most commonly located in the sagittal plane. We present a case of abortive hypospadiac urethral duplication and its surgical management with tubularized incised plate urethroplasty.

An exceptional combined malformation: duplication of the urinary and intestinal tracts and the vulva (04-80CR)

The authors reported the surgical treatment of a 2-year-old girl with complete duplication of the kidney, ureter, bladder, urethra, and the vulva, associated with intestinal duplication and complete duplication of the appendix and colon. Congenital intestinal malrotation also concurred. After a series of preoperative examinations, exploratory operation including reduction of intestinal malrotation, fusion of bladder and colon, obliteration of the duplicated urethra, resection of the intestinal and appendiceal duplications, and cosmetic repair of the vulva was performed. Incontinence of urine and stool disappeared, and she had been followed up for 10 months until this report. Repeat voiding cystourethrography performed recently demonstrated a fused bladder and the disappearance of the duplicated urethra.

Congenital prepubic sinus: A variant of dorsal urethral duplication (Stephens type 3)

Abstract A 3-year-old boy was admitted for yellowish discharge from a tiny opening in the midline prepubic area on the dorsal base of the penis. No other symptom related to the genitourinary tract was noted. Sinuousgraphy showed a non-communicating sinus tract with the urinary tract. At surgical exploration, through a diamond-shaped incision, a 4.5 cm-long sinus was found that ended blindly as a fibrous cord at the anterior surface of the pubic symphisis. Histologically, it was lined by stratified squamous epithelium and surrounded by bundles of smooth muscle and collagen. According to Stephens' classification, the sinus appears to be a variant of dorsal urethral duplication of Stephens type 3.

Congenital urethral anomalies in boys. Part II.

In the second part of this article, congenital urethral anomalies other than posterior urethral valve were reviewed. The anomalies considered in the current review were anterior urethral valve, lacuna magna, syringocele, Cobb's collar, duplication of urethra, megalourethra, and prostatic urethral polyps. The literature was extensively reviewed concerning the presentations, diagnosis, different types of treatment modalities, morbidity, mortality, and new concepts for the above disorders. Anterior urethral valves or diverticula are the most prevalent congenital anomalies of anterior urethra. The lacuna magna is the largest depression in the dorsal aspect of the fossa navicularis. It is demonstrable on a well-performed voiding cystourethrography of the distal urethra. The dilated Cowper's gland duct is the other missed diagnosed anomaly of the urethra in boys. The congenital narrowing of the bulbar urethra with a variable clinical presentation and obstruction grade and different types of anterior urethral obstruction are the most common presentation of these anomalies. However, other symptoms or signs including, hematuria, bloody spotting on underwear, discomfort or sever pain in the vicinity of the glance, interrupted voiding, infection, bulging of anterior urethra, enuresis, and postvoiding dribbling are the only nonspecific manifestations of these disorders. All of these disorders are demonstrable on a well-performed voiding cystourethrography of the distal urethra. The urologist must be aware about these uncommon congenital anomalies and the anterior urethra should be carefully evaluated for such anomalies. Diagnosis of these entities is elusive unless the physician is looking for them. Nonspecific symptoms mentioned here besides radiographic findings can be a valuable clue for diagnosis.

Duplicidad uretral incompleta en un varón. Aportación de un nuevo caso

Urethral duplication is a rare congenital anomaly affecting mainly males and being usually diagnosed during paedriatric age. We report a 20 year old male complaining of double urethral meatus with double urinary stream. Physical examination confirmed and additional hypospadic meatus below a normally placed urethral meatus. Retrograde urethro-cystography and voiding cysto-urethrograms showed two distinct urethras originating from a common bladder neck and the diagnosis of Effmann type IIA2 incomplete urethral duplication was made. No treatment was felt to be applied after associated anomalies were ruled out.

Voiding Cystourethrography in Boys. Does the Presence of the Catheter During Voiding Alter the Evaluation of the Urethra?

We determined whether the presence of the catheter during the voiding phase of voiding cystourethrography alters the evaluation of the urethra concerning the normal structures as well as pathological findings, especially posterior urethral valves. A total of 123 males 3 days to 16 years old (median age 2.6 months) underwent voiding cystourethrography. Urethral catheterization was performed in all cases. Four views were taken during the voiding phase with and without the catheter in place. Only 80 patients had available results. These examinations were studied with special attention to the normal structures and pathological findings. A total of 36 examinations (45%) were normal. Pathological findings were observed in 44 patients (55%), with abnormal vesical findings and/or vesicoureteral reflux in 33 (41.25%). In 11 patients (13.75%) 12 urethral abnormalities were found (posterior urethral valves 3, hypospadias 4, prostatic utricle 1, verumontanum polyp 1, prune belly syndrome with urethral dilatation 1, imperforate anus with urethral fistula 1 and urethral duplication 1). In all cases excluding those involving hypospadias there was no difference between the views with and without the catheter. However, concerning the normal structures, the verumontanum and fossa navicularis were better delineated without the catheter in 27% and 33% of cases, respectively. Our study shows that a urethral catheter does not alter the diagnosis of abnormalities of the posterior urethra but may hamper the observation of normal structures or abnormalities of the anterior urethra.

Radiological Findings of Male Urethral Duplication Associated with Bladder Duplication: Case Report

Urethral duplication or accessory urethra is a rare congenital anomaly. Even rarer, is its association with bladder duplication. We report a case of urethral duplication associated with bladder duplication in a seven-year-old boy who underwent retrograde urethrography, sonography and magnetic resonance (MR) imaging. While retrograde urethrography can demonstrate the extent of the duplicated urethra, MR imaging and sonography can provide detailed information on the anatomy of the adjacent tissues as well as urethral duplication.

Radiological Findings of Male Urethral Duplication Associated with Bladder Duplication: Case Report

Radiological Findings of Male Urethral Duplication Associated with Bladder Duplication: Case Report

Adult presentation of complete female urethral duplication

Adult presentation of complete female urethral duplication