Urethral duplication in children: surgical treatment and results

PURPOSE: Urethral duplication is a rare congenital anomaly. We report the clinical presentation, imaging findings and surgical treatment in 7 boys with incomplete sagittal duplication of the urethra. MATERIALS AND METHODS: Duplication involved hypospadias in 5 cases (group 1) and a bifid urethra with an accessory preanal tract (Y duplication) in 2 (group 2). Group 1 was treated with 1-stage urethroplasty, including marsupialization of the dorsal orthotopic urethra, ventral-to-dorsal urethrourethrostomy and penile island flap onlay repair to cover the open dorsal urethra. In contrast, group 2 was treated with 2-stage urethral reconstruction with detachment and mobilization of the accessory preanal branch in association with a scrotal tubed neourethra followed by urethroplasty, as in group 1. In all cases the dorsal penile urethra was located between the corpora cavernosa and surrounded by the tunica albuginea. RESULTS: A urethrocutaneous fistula developed in 1 of the 5 group 1 patients. In group 2, 1 patient had recurrent penoscrotal meatal stenosis after the 1-stage procedure and 1 had a urethral diverticulum with calculi at the scrotal tubed neourethra 7 years after urethral reconstruction. Six of the 7 patients now void spontaneously through a meatus located normally at the tip of the glans. The remaining patient with a neurogenic bladder is on intermittent catheterization via appendicovesicostomy due to difficult catheterization of the irregular and sensitive neourethra. CONCLUSIONS: While the ideal surgical management of urethral duplication anomalies remains uncertain, we used a combination of surgical techniques to correct this severe malformation.     

Congenital megalourethra associated with urethral duplication and imperforate anus

A newborn with a prenatal diagnosis of right hydroureteronephrosis and enlarged penis is presented. At birth, the baby had an imperforate anus (IA) with a megalourethra; radiologic and ultrasonographic studies showed a left polycystic kidney and right hydroureteronephrosis, right vesicoureteral reflux, and an incomplete urethral duplication with dilatation of the posterior urethra. The IA was corrected on the 1st day of life and a vesicostomy was performed at 1 month. At 1 year of age the valve obstructing the ventral posterior urethra was resected and the vesicostomy was closed. At 14 months the baby underwent a urethroplasty with a vertical preputial tubularized island flap and excision of the penile urethral duplication. Exact knowledge of the malformation was essential in planning the appropriate surgical treatment.     

Surgical repair of urethral circumcision injuries

PURPOSE: The 2 types of urethral injury that can occur during circumcision are urethrocutaneous fistula and urethral distortion secondary to partial glans amputation. We report the surgical repair of these rare injuries. MATERIALS AND METHODS: In 8 patients urethrocutaneous fistulas located on the distal penile shaft or at the coronal margin were managed by splitting the glans and using a Mathieu style skin flap in 4 or vascularized penile skin flap in 4 to bridge the urethral defect. Three patients underwent repair of a hypospadiac deviated urethra secondary to partial glans amputation by 1 cm. of urethral mobilization and repositioning the meatus into a terminal position within the remaining glans tissue. RESULTS: The 8 patients with urethrocutaneous fistulas voided via a terminal meatus without fistula recurrence at a mean followup of 3.2 years (range 1 to 6). The 3 patients with partial glans amputation and urethral deviation repaired by short urethral advancement had functionally acceptable results, defined as a normal urinary stream, although 1 required meatal dilation postoperatively. CONCLUSIONS: The 2 types of urethral injuries that can occur during circumcision are a subcoronal urethrocutaneous fistula and scarred abnormal urethra from partial glans amputation. The urethrocutaneous fistula can be successfully repaired by splitting the glans and forming a neourethra from a vascularized pedicle flap of penile skin. The abnormal urethra after partial glans amputation is more difficult to repair but repositioning the urethra in a more cosmetic location has restored function.     

The penile disassembly technique in hypospadias repair

OBJECTIVE: To report experience and results with penile disassembly in hypospadias repair. PATIENTS AND METHODS: From November 1995 to May 1997 penile disassembly was used in 92 patients aged from 9 months to 32 years. The indications for operation were hypospadias with severe penile curvature (especially with curvature in the distal third of the corpora cavernosa), chordee without hypospadias, and small penis with hypospadias. The technique involves separating the penis into its component parts, i.e. the glans cap with neurovascular bundle dorsally, together with the undivided or divided urethra and urethral plate ventrally, and the corpora cavernosa. The manoeuvre allows any curvature to be corrected, especially when in the distal third of the corporal bodies, glans tilt to be rectified, and the penis to be enlarged, particularly elongated, which is a significant gain in small penises with hypospadias. RESULTS: The patients were followed for 3-20 months (mean 14); the penis was straightened in all cases, with no recurrence of curvature. In 37 patients (40%) penile disassembly combined with extensive urethral mobilization resolved the hypospadiac meatus with no need to form a neourethra; the penis was larger after surgery. Complications were related to urethroplasty and included four urethral stenoses, two fistulae and three diverticula. There was no injury to the neurovascular bundle and urethra; sensitivity and erection were preserved in all patients. CONCLUSION: The penile disassembly technique is most effective for hypospadias with severe curvature, especially for glans tilt and curvature located distally. Penile augmentation is possible using this technique.     

Incomplete urethral duplication with cyst formation in a dog

Incomplete urethral duplication with cyst formation was diagnosed in a dog that had soft, fluctuant, subcutaneous swellings in the ventral perineal and penile areas and a history of nocturia and incontinence during recumbency that were unresponsive to treatment with antibiotics. Retrograde urethrocystography, voiding urethrography, double-contrast cystography, radiography after direct administration of contrast medium into cystic structures, and excretory urography were performed to evaluate the urinary tract. Communication between the cysts and the urethra was demonstrated radiographically only after intralesional injection of contrast medium. Nocturia and incontinence resolved after surgical removal of the urethral duplication and cysts. The dog was clinically normal 1 year after surgery.     

Congenital H-type urethroanal fistula

A case of congenital urethroanal fistula with a normal anterior urethra in a male child is reported. The fistula was demonstrated between the prostatic urethra and anorectum. This anomaly is usually associated with an atretic anterior urethra and has been variously described as a variant of a urethral duplication by some authors, and of an anorectal malformation (ARM) by others. We conclude that its rightful classification is as a variant of ARM in which the fistula is a result of persistence of the cloacal duct and corresponds to the anorecto-vestibular fistula with a normal anus (perineal canal) in a female.     

Incomplete hypospadiac urethral duplication with posterior urethral valves

Urethral duplication (UD) is an uncommon malformation. Obstruction rarely occurs in hypospadiac UD. We describe two children with incomplete hypospadiac UD in association with posterior urethral valves, a combination not previously recognised. The embryonic significance of this anomaly is discussed. Keywords Urethral duplication. Hypospadias. Posterior urethral valve. Megalourethra     

Duplication of the urethra

Ten patients with patent urethral duplication are presented. Nine patients had two independent patient channels originating from the bladder or the posterior urethra. Both voiding cystourethro and retrograde urethrography are necessary to completely evaluate this anomaly. In each instance the ventral channel, regardless of the position of its meatus, proved to be the more functional urethra. In those patients whose dorsal channel was in an epispadiac position, the symphysis pubis was abnormally wide. A functionally and anatomically accurate classification is proposed, and the embryology of these anomalies is discussed.     

Multicoil MR imaging of symptomatic female urethral and periurethral disease

High-resolution magnetic resonance (MR) imaging with phased-array pelvic and endorectal coils has dramatically enhanced the ability to visualize abnormalities of the female urethra and periurethral tissues. These include developmental abnormalities (eg, urethral duplication, ectopic ureterocele), benign processes (eg, urethral diverticulum, caruncle, leiomyoma, trauma, and fistula; stress incontinence; paravaginal cyst), and malignant processes (eg, primary urethral carcinoma, secondary urethral malignancies). High-resolution MR imaging can be used to assess complications such as fistula formation and periurethral abscess, localize various entities, exclude pathologic processes whose presence could lead to an incorrect diagnosis, differentiate processes that might be confused at physical examination, and contribute to surgical planning and facilitate surgical correction.     

Water balance of Dermatophagoides pteronyssinus (Acari: Pyroglyphidae) maintained by brief daily spells of elevated air humidity

OBJECTIVES: To review the urological abnormalities in conjoined twins, a rare congenital abnormality arising from an incomplete separation of a pair of monozygotic twins, and who can be joined from the head to the pelvis with varying degrees of union. PATIENTS AND METHODS: The records and urological anomalies of conjoined twins presenting from 1985 to 1995 were reviewed retrospectively and the management and surgical reconstruction described. RESULTS: Seven of 11 sets of conjoined twins underwent surgical exploration. Complex urological problems occurred in all twins with omphalo-ischiopagus. The urological anomalies were: Set 1; both twins had one lateral crossed fused renal ectopia with uncrossed ureters draining into a single bladder with one urethra. They had one set of genitalia. Each twin received one crossed fused ectopic kidney, half the bladder and genitalia, with one retaining the urethra and one a vesicostomy. Set 2; these twins had four kidneys (two lateral and two central that were fused), four ureters (two of which crossed) and these drained into two sagittal bladders that emptied by one urethra associated with a single set of external genitalia containing three corpora cavernosa. At separation, the crossed ureters were re-routed, each received a bladder and the genitalia were split. Set 3; there were four kidneys with two crossed ureters draining into two collateral bladders, there was a partial duplication of the urethra and a single hypospadiac penis. After separation, each had kidneys draining into a bladder, one received the urethra and external genitalia, and one a urostomy. CONCLUSION: Complex urological problems occur in conjoined twins, although restricted to those with pelvic fusion (ischiopagus). Assessment and treatment needs to be individualised and overseen by a surgeon with experience in complex urology.     

A case of primary giant calculus in female urethra

Urinary calculus is rarely seen in the urethra and is usually encountered in men with urethral stricture or diverticulum. Primary urethral calculi are extremely rare in females. We describe a case of a giant urethral stone impacted in the urethra of a 103-year-old female.     

Conservative surgical therapy for penile and urethral carcinoma

OBJECTIVES: Invasive penile and urethral tumors are traditionally treated with aggressive excision that requires involved organ and adjacent organ sacrifice. An alternative approach seeks to completely excise the tumor with adequate margins while preserving form and function of the organ. We present 6 patients who underwent such organ-sparing surgery. METHODS: Six selected cases (4 penile and 2 urethral) are presented with operative photographs and pertinent data. RESULTS: Three distal tumors of the penis were treated with excision limited to the glans with histopathologic findings of verrucous carcinoma, melanoma, and angiosarcoma. One patient with squamous cell carcinoma of the distal shaft refused partial penectomy and underwent a local wedge resection. A patient with locally advanced bulbourethral transitional cell carcinoma (TCC) refused cystourethrectomy and underwent an anterior urethrectomy and perineal urethrostomy. A 48-year-old woman with an adenocarcinoma contained in a very distal urethral diverticulum underwent simple diverticulectomy and excision of distal urethra. Postoperative voiding and sexual function were well preserved. Follow-up was 12 to 48 months. The patient with angiosarcoma died of lung metastases at 48 months with no local disease, and the patient with bulbourethral TCC developed pelvic disease at 12 months with no local recurrence and died of metastases at 25 months. CONCLUSIONS: Organ-sparing surgery is appropriate in selected patients on the basis of stage and location, high risk of distant failure, and patient disposition. Close follow-up is necessary. Comanagement with reconstructive and oncologic specialists optimizes results and outcomes.     

Splenorenal shunt blood flow by transit-time ultrasound as an index of collateral circulation in portal hypertensive rats

OBJECTIVE: The purpose of this study was to explore the role of sonography for women with urethral symptoms and a suspected urethral diverticulum. SUBJECTS AND METHODS: Nineteen women with urethral symptoms underwent voiding cystourethrography (VCUG) and transvaginal, transperineal, and urethral sonography (using a catheter-based transducer). VCUGs and sonograms were evaluated for diverticula, defined on sonography by direct visualization of the neck connecting the periurethral sac with the urethral lumen. The diverticular neck, size, location, and shape were noted. Lesions revealed by sonography as not connected to the urethra were also noted. RESULTS: Of 19 women, 14 had urethral diverticula and one had two diverticula, for a total of 15 diverticula. On sonography the diverticula ranged in diameter from 2 mm to 5 cm. Both sonography and VCUG showed 13 of the 15 diverticula. In addition, sonography revealed two infected periurethral cysts, a periurethral leiomyoma, a diffuse urethritis, and scarring or deformity of one patient's urethra from a prior diverticulectomy. On sonography, eight of the 13 diverticula wrapped around more than 50% of the urethral circumference. The neck was precisely seen (by definition) in 13 of 15 diverticula on sonography and in two of 13 diverticula on VCUG. CONCLUSION: Sonography is useful in this group of women with urethral symptoms and suspected urethral diverticula. It provides information on the extent and location of the diverticular neck, both of which are important in surgical excision. Also, sonography provides information on lesions not connected to the urethra. Sonography may prove useful in a broader group of women with urethral symptomatology.     

Urodynamic assessment of women with urethral syndrome (author's transl)

Thirty women presenting with urethral syndrome were investigated. A full urologic, radiologic, gynecologic and urodynamic assessment was carried out. In most patients the cause of their troubles was found. In 19 of them an obstruction was demonstrated. Two types of obstruction were seen: The first occurred mainly in postmenopausal women. Here, a narrow distal segment of the urethra was demonstrated. The second type of obstruction was seen mainly in younger patients. The cause was an incomplete relaxation or a spasm of the external striated sphincter. Other causes of symptoms were instability and atony of the detrusor. In some women no cause for their symptoms and recurrent urinary infection was found.     

Onlay island flap urethroplasty in the treatment of urethral strictures

A skin island flap was used to create a neourethra in 65 patients aged 2 to 66 years with prolonged or multiple stricture of the anterior urethra. Before, all patients were treated repeatedly and unsuccessfully by open or endoscopic surgery. The principle of the used onlay technique was to supplement the missing lumen of the urethra by an island, usually preputial flap. In case of as missing segment of the urethra the original inlay-onlay technique was applied. After a mean follow-up period of 27 months 12 complications (18.4%) were recorded, six strictures of the proximal anastomosis, three fistulas and three dehiscences of the glans. CONCLUSION: The onlay island flap urethroplasty makes it possible to cure prolonged and multiple strictures of the urethra in one stage and with a relatively low complication rate.     

Forensic medicine in China

PURPOSE: A variant form of anterior hypospadias, called a megameatus and intact prepuce (MIP), is thought to be less amenable to conventional distal hypospadias repair. The feasibility of using the standard technique with a parameatal-based foreskin flap is described herein. MATERIALS AND METHODS: Nine children with the MIP variant underwent repair. A foreskin flap for urethroplasty was harvested from either the ventral (Mathiew) or unilateral site. The glans was split along with the cleft glanular groove to create the glans wings. The flap was laid on the urethral plate to form a neourethra, and glanulomeatoplasty was completed by approximation of the glans wings. Sleeve reapproximation of the penile foreskin was performed for uncircumcised skin closure. RESULTS: The functional and cosmetic results of the procedure were excellent in 8 cases including 1 with temporary postoperative edema of redundant foreskin. The last case underwent excision of the ventral excess foreskin for cosmetic reasons. CONCLUSIONS: Although the etiology of the MIP variant remains obscure, the urethral plate distal to the meatus is uniformly pliable and healthy in this variant. Furthermore, the ventral portion just proximal to the meatus is well developed and not atretic so that the parameatal ventral foreskin is safely harvested for onlay urethroplasty.     

Arguments against the involvement of Borrelia burgdorferi sensu lato in Alzheimer''s disease

The posterior prostatomembranous urethral stricture or distraction defect has historically been the most formidable challenge of stricture surgery. This uncommon lesion occurs most often as the sequelae of pelvic fracture injuries, or straddle trauma, and is associated with serious urethral disruption and separation--an injury that is often complicated by inappropriate initial management using substitution skin flap techniques with the development of recurrent stenosis, irreversible impotence, and occasional incontinence. Management by endoscopic techniques may be possible in patients with short strictures or in those after prostatectomy, but they rarely play a role in resolving the complex obliterated urethra with a significant defect [1]. Resolution of post-traumatic posterior urethral distraction defects and other posterior urethral pathologic conditions has dramatically improved over the past two decades despite an inaccessible subpublic location involving exposed sphincter-active and erectile neurovascular anatomy. The contemporary, perineal, one-stage bulboprostatic anastomotic operation as popularized by Turner-Warwick [20] with selective scar excision is a versatile procedure with a high patent lumen success. Patients undergoing anastomotic urethroplasty have a substained patent urethral lumen success rate approaching 100% versus those who have undergone urethral skin flap or patch repair, where the restricture rate in 5 and 10 years increases twofold to threefold [1, 20]. A patent urethra after an anastomotic urethroplasty at 6 months is free from further recurrent stricture and gives credence to Mr. Turner-Warwick's admonition that "urethra is the best substitute for urethra".     

The natural history of condyloma in children

OBJECTIVES: To prospectively evaluate our previously established pathologic risk factors in women undergoing cystectomy for bladder cancer and to determine if these criteria identify appropriate female candidates for orthotopic diversion. METHODS: Prospective pathologic evaluation was performed on 71 consecutive female cystectomy specimens removed for primary transitional cell carcinoma of the bladder. The histologic grade, pathologic stage, presence of carcinoma in situ, number, and location of tumors were determined. In addition, final pathologic analysis of the bladder neck and proximal urethra was performed and compared with the intraoperative frozen-section analysis of the distal margin (proximal urethra). RESULTS: Tumor at the bladder neck and proximal urethra was seen in 14 (19%) and 5 (7%) cystectomy specimens, respectively. Bladder neck tumor involvement was found to be the most significant risk factor for tumor involving the urethra (P <0.001). All patients with urethral tumors demonstrated concomitant bladder neck tumors. However, more than 60% of patients with bladder neck tumors had a normal (tumor-free) proximal urethra. Furthermore, no patient with a normal bladder neck demonstrated tumor involvement of the urethra. Intraoperative frozen-section analysis of the distal surgical margin was performed on 47 patients: 45 without evidence of tumor and 2 patients with urethral tumor involvement. In all cases, the intraoperative frozen-section analysis was correctly confirmed by final permanent section. CONCLUSIONS: We prospectively demonstrate that bladder neck tumor involvement is a significant risk factor for urethral tumor involvement in women. However, despite bladder neck tumor involvement, a number of women undergoing cystectomy for bladder cancer have a normal urethra and may be candidates for orthotopic diversion. Furthermore, our data demonstrate that intraoperative frozen-section analysis of the distal surgical margin accurately and reliably evaluates the proximal urethra and currently determines which patients undergo orthotopic diversion at our institution.     

Hypospadias repair in the 1990s

Hypospadias is a congenital anomaly in which the urethral meatus is abnormally located anywhere from the glans to the perineum. Refinements in pediatric anesthesia, surgical technique and instrumentation, and a greater understanding of the psychologic and emotional issues related to hypospadias repair have led to this procedure being performed at an earlier age. The goal of reconstruction is to bring the urethral meatus to the tip of the penis, to correct associated penile curvature if present, to create a conical-shaped glans, and to achieve cosmetically acceptable penile shaft skin coverage. The surgical technique employed varies with the penile anatomy and surgeon's preference. Complications may occur both immediately and long after the surgical procedure, and thus follow-up is necessary.     

Prenatal sonographic diagnosis of posterior urethral valves: identification of valves and thickening of the posterior urethral wall

This report describes 2 heretofore unreported sonographic findings that may aid in the antenatal diagnosis of posterior urethral valves. Each of 2 fetuses showed a prominent or thickened posterior urethra and one or more bright echogenic lines representing valve tissue within a dilated and/or thickened posterior urethra. These findings helped solidify the antenatally suspected diagnosis of posterior urethral valves. The diagnosis was confirmed clinically and surgically during neonatal life. These 2 imaging findings may help in providing a more definitive antenatal diagnosis of posterior urethral valves and allow more expeditious therapy.