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Urethral Duplication

University of Louisville Presentation

Uzoma Anele

on 12 September 2012

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Transcript of Urethral Duplication

Duplication Uzoma Anele, MS4
August 12, 2012 Most patients with incomplete urethral duplications are asymptomatic 1980 2000 1990 1986 Only about 150 cases reports in the literature 2012 Approximately 300 case reports available in the literature Epidemiology Epispadiac duplications are associated with dorsal curvature of penis Patients with complete duplications can present with urinary obstruction, incontinence, infection, or multiple streams Urethral Duplication in the Male: Review of 16 Cases Rare congenital malformation defined by the juxtaposition of two or more muscular channels lined by urinary tract mucosal epithelium Associated with upper urinary and gastrointestinal tract anomalies Most duplications occur in the same sagittal plane 1. Maitama H., Mbibu H., Tella U. Urethral Duplication: Case Report and Literature Review. Annals of African Medicine Vol. 11, No. 3; 2012. pp 186-189

2. Erdil H., Mavi A., Erdil S., Gumusburun E. Case Report:
Urethral Duplication. Acta Medica Okayama Vol 57, No. 2; 2003. pp 91-93

3. Ramareddy R., Alladi A., Siddappa O. Urethral Duplication: Experience of Four Cases. Journal of Indian Association of Pediatric Surgeons Vol 17, No 3; 2012 pp 111-114

4. Fernandez-Pineda I., Fernandez-Hurtado M.A., Candau R., Merino F. Urethral Duplication: Case Report and Review of Literature. Actas Urologicas Espanolas Vol 34, No 5; 2010. pp 477-488

5. Etnsel B., Yazici M., Gursoy H., Ozkisacik S. Tubularized Incised Plate Urethroplasty for Abortive Hypospadiac Duplication. Pediatric Surgery Internantional Vol 21; 2005. pp 494-496

6. Podesto M., Medel R., Castera R., Ruarte A. Urethral Duplication in Children: Surgical Treatment and Results. The Journal of Urology Vol 160; 1998. pp 1830-1833

7. Berrocal T., Novak S., Arjonilla A., Guitierrez J., Prieto C., Urrutia M. Complete Duplication of Bladder and Urethra in the Coronal Plane in a Girl: Case Report and Review of the Literature. Pediatric Radiology Vol 29; 1999. pp 171-173

8. Kumar A., Goyal N., Trivedi S., Dwivedi U., Singh P. Y-Duplication of the Urethra: A Rare Case Report. The Internet Journal of Surgery Vol 11, No 2; 2007

9. Lin H., Shen X., Geng H., Xu M. Treatment and Medical Follow-up of a Boy with Urethral Triplication. Urology Vol 80, No 1; 2012 pp 214-215

10. Yilmaz O., Gunsar C. Tubularized Incised Plate Urethroplasty is an Applicable Method for the Treatment of the Hypospadiac Type of Urethral Duplication. Balkan Medical Journal Vol 28; 2011. pp 316-318

11. Chu A., Cho J., Kim S., Jun J. Prenatal Ultrasound Findings of Fetal Urethral Duplication. Utrasound Obstetric Gynecology Vol 36; 2010. pp 652-653

12. Salle J., Sibai H., Rosenstein D., Brzezinski A., Corcos J. Urethral Duplication in the Male: Review of 16 Cases. The Journal of Urology Vol 163; 2000. pp 1936-1940

13. Wein A., Kavoussi L., Novick A., Partin A., Peters C. Campbell-Walsh Urology 10e. Chapter 126 pp 3407-3408. Chapter 131 pp 3551-3552

14.Human Embryology Organogenesis http://www.embryology.ch/anglais/turinary/devebauche04.html#metanephros

15. Upadhyay J. Urethral Anomalies and Urethral Prolapse. http://emedicine.medscape.com/article/1015912-overview References Etiology & Pathophysiology Diagnosis Normal Urinary Tract Development JOA˜O LUIZ PIPPI SALLE, HICHAM SIBAI, DANIEL ROSENSTEIN, ALEX ELIAS BRZEZINSKI, AND JACQUES CORCOS To discuss the etiology and pathophysiology of urethral duplication To become familiar with the various classification systems To briefly review normal genitourinary system development To explore available diagnostic, treatment and management options To introduce the epidemiology of urethral duplication Objectives Objectives Retrospective review of records of 16 male patients treated for urethral duplication in the last 10 years

Age at presentation ranged from newborn to 8 years

Evaluation included ultrasound, voiding cystourethrography, retrograde urethrography and endoscopy Just the Tip Approximately 300 Case Reports Meta-Analysis Effmann
Classification Incomplete Duplication (Type I) Complete Duplication with Caudal Duplication (Type III) Complete Urethral Duplication (Type II) Casselman and Williams Das and Brosmas Rica et al Does not distinguish sagittal from coronal collateral duplication Distal Proximal A. 2 Meati B. 1 Meatus Partial or Complete Caudal Duplication Woodhouse and William Classification
Sagittal vs Coronal duplication
Das and Brosman Classification
Type I - complete accessory urethra arising from a separate or confluent opening within bladder and extending to an external orifice
Type II - accessory urethra arising from primary urethra and may or may not extend to a distal orifice
Type III - accessory urethra arising from bladder neck or prostatic urethra and opens onto perineum
Firlit Classification
Urethra that arises proximally from the bladder, bladder neck, or duplicated bladder Other Classification Systems Partial failure or an irregularity in growth of the lateral mesoderm layers of the cloacal membrane in the midline accounts for the form with a dorsal epispadiac channel Patten and Barry Abnormal termination of the Mullerian duct responsible for urethral duplication Suggested asymmetry in closure of urorectal septum results in a urethroperineal fistula Result of an abnormal relationship between lateral folds of genital tubercle and ventral end of the cloacal membrane Associated with severe congenital anomalies such as imperforate anus, cloacal atrophy, conjoined twins, early amnion rupture syndrome, prune belly syndrome and hand-foot genital syndrome (Maitama, et al) Difficult to determine abnormal embryology of a system when the normal has not yet been fully elucidated (Abrahamson J, 1961) Woodhouse and William Attributed to an ischemic process in embryogenesis History Imaging Physical Exam Treatment & Management Oligohydramnios None Multiple Urinary Streams
Obstruction Incontinence
Urinary Tract Infections Perineal/Anal Urinary Dribbling or Stream Imperforate Anus Bladder Extrophy Hypospadias/Epispadias Double Meatus Ultrasound VCUG MRI Y-type duplication with 2-stage repair involving displacement of urethra from anal canal to perineum in stage 1 and subsequent urethral reconstruction using a long tubularized preputial island flap in stage 2 (Salle, et al 2000) Mean follow-up 45 months
13 patients (81%) uncomplicated outcome
Y-type duplication developed urethral stenosis at proximal anastomosis which was successfully treated with dilation Coronal urethral duplication present in only 1 case (6%)
Sagittal urethral duplication present in 94% of cases
Double urethral meatus or urinary stream presenting sign in 11 cases (61%)
3 patients who presented with UTIs had associated vesicoureteral reflux
1 case presented with penile urinary dribbling and anal voiding
Vesicoureteral reflux most common associated abnormality found in 37.5% of cases
Bladder exstrophy in 3 cases (19%)
Hypospadias, cryptorchidism, and anal stenosis each 1 case Type I partial duplication present in 25% of cases
Type II complete duplication present in 62.5% of cases
Type III complete urethral and bladder duplication present in 12.5% of cases Only symptomatic patients or those with significant cosmetic problems were treated surgically
Three patients underwent meatoplasty
Eight patients (50%) underwent excision of accessory dorsal urethra
Observation elected in 2 patients (Type III but asymptomatic bladder and urethral duplication) Occasionally cystoscopy necessary to visualize the verumontanum and other urethral characteristics (Effmann, et al 1976)
IVP or CT urogram used to assess or evaluate for other abnormalities in upper tract Y-type duplication presents one of the greatest challenges
In reconstruction, the ideal urethra is that with the largest caliber, around the verumontanum with an intact sphincter
Surgical techniques include:
use of apical urethra even when hypoplastic
electrofulgaration or injection of sclerosing agents into the accessory tract
urethroplasty using scrotal flaps (Williams & Bloomberg)
use of buccal mucosa when no foreskin available for repair (Ortolano & Nasaralloh, 1986)
anastomosis of ventral urethra to dorsal urethra when latter has adequate caliber
perineal tract excision alone if confirmed normal orthotopic urethra and small perineal tract (Wagner et al, 1996) Voiding with single stream Normal and duplicated urethra draining urine Y-type duplication with VUR and dorsal accessory urethra Mobilization and excision of accessory urethra
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