Type IA Urethral Duplication

CASE REPORT Bali Medical Journal (Bali Med J) 2019, Volume 8, Number 3: 587-591 P-ISSN.2089-1180, E-ISSN.2302-2914 Type IA urethral duplication: A case report Case Report Miftah Adityagama,1 Yonas Immanuel Hutasoit2* Published by DiscoverSys CrossMark Doi: http://dx.doi.org/10.15562/bmj.v8i3.1602 ABSTRACT

Background: Urethral duplication is a rare congenital malformation stopped filling 2 cm from the meatus in the dorsal penoscrotal Volume No.: 8 mainly affecting men and boys. Although a number of theories have with a total dead-end impression as an accessory urethra and been proposed to describe this condition, the actual mechanism of there was no abnormalities in the principal urethra looked from this disorder is still not clear. The most frequent anomaly occurs in the the contrast filled the penile urethra, bulbous urethra, prostatic Issue: 3 sagittal plane, in which the duplicated urethra is in either dorsal or urethra, and bladder. That was the form of Effmann Type IA urethral ventral position in relation to the orthotopic urethra. The therapeutic duplication. The dorsal accessory urethra was excised entirely management of these conditions is complicated and depends on the without complication and he remains symptom-free eight months presence of symptoms as well as the type of anomaly. after surgery. First page No.: 587 Case Description: We present a case of urethral duplication in a Conclusion: In summary, urethral duplication is a rare clinical 2 years old male child. The malformation was characterised by the condition. It has many variants that were classified by Effman. presence of the meatus in the dorsal penoscrotal and accompanied Management depends on the anatomy of the duplication involved and P-ISSN.2089-1180 by the sign of infection in it. Urethrography showed contrast the patient’s clinical symptoms.

Keywords: Double urethra, urethral duplication, Effman’s classification, reconstructive surgery. E-ISSN.2302-2914 Cite This Article: Adityagama, M., Hutasoit, Y.I. 2019. Type IA urethral duplication: A case report. Bali Medical Journal 8(3): 587-591. DOI:10.15562/bmj.v8i3.1602

1Department of Urology, Faculty INTRODUCTION patients. The treatment aims to preserve renal of Medicine Universitas Indonesia, function, achieve continence, and achieve the Cipto The urethral duplication, also known as double 2Mangunkusumo National General best possible cosmetic outcome. The ventral part urethra, is a rare anomaly occurring in pediatric of the urethra is always the functioning urethra, Hospital, Jakarta, Indonesia cases, this deformity is most often found in male 2Department of Urology, complete with sphincters and the urinary bladder. Fatmawati General Hospital, children while for female children it is very rare, Several methods may be used in obliterating the Jakarta, Indonesia with a previous literature recording no more than accessory channels, such as injection of sclerosing 40 cases of double urethra found in female chil- 1,2 agent or other ablative interventions. The tech- dren. The clinical entity comprised of a wide niques, however, are slowly being abandoned due spectrum with various anatomical variations. Thus, to the risk of corporeal thrombosis and fibrosis. there are several classifications used in defining Reconstruction by combining the ventral and each case of double urethra or urethral duplication, 3 accessory urethra parts may be utilized, although such as the Effmann classification system. It can it carries a potential risk for meatal stenosis and be complete or incomplete, the majority of cases obliteration of dorsal meatus. The current golden occur in the sagittal plane, further divided into standard is surgical excision of dorsal accessory dorsal and ventral duplication according to the urethra. The procedure itself needs to be done position of the ectopic urethra. Collateral duplica- delicately in order to avoid damage to the external tion without bladder duplication may also be found sphincter and neurovascular bundle.1 without accompanying bladder duplication. The relative rarity of the case combined with the wide spectrum of manifestation creates some difficulties CASE REPORT in pinpointing the exact pathophysiological mech- *Correspondence to: dr. Yonas Immanuel Hutasoit; anism. Several literatures had suggested that there A 2-years-old male patient was referred to urology Department of Urology, Fatmawati were multiple mechanisms that may cause urethral polyclinic at Fatmawati General Hospital with the General Hospital, Jakarta, Indonesia; duplication, each possibly related with different chief complaint there was whitish discharge from [email protected] types of manifestation.1 a hole in the dorsal penoscrotal. That condition Individually tailored management is required has been realised by the parents since the last Received: 2019-09-11 due to the spectrum above of clinical manifesta- one month, but they have noticed the small hole 1,4 Accepted: 2019-10-10 tions in cases of urethral duplication. The general in the dorsal penoscrotal since the patient was Published: 2019-12-01 consensus is, presently, to treat only symptomatic 3-months-old. Previously they took the patients

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to a paediatrician because the patients were fussy when urinating and looked reddish around the hole then stated there was inflammation. There was no difficulty in urinating. Good emission of urine, not branched, and there was no urine comes out of a hole in the dorsal penoscrotal. There were no abnormalities in growth and development or other congenital abnormalities. From the physical examination we found a normal external urethral meatus on the tip of the glans penis (Figure 1a) and the second meatus with a diameter of 2 mm in the Figure 1 The normal external urethral meatus dorsal penoscrotal (Figure 1b). The two testicles (a) and the second meatus in the dorsal were found normal. penoscrotal (b) During urethrography and urethroscopy examination, it was obtained a meatus from the dorsal penoscrotal as long as 2 cm, a pinpoint hole and debris appeared, and the wire could not be inserted. Contrast stopped filling 2 cm from the meatus in the skin, with a total dead-end impression (Figure 2a). 7 Fr ureteroscopy sheath was inserted through the external urethral meatus on the tip of the glans penis; urethra, verumontanum, bladder neck and bladder were found normal. There were no holes along the urethra; the right and left uret- Figure 2 U rethrography showed contrast stopped eral opening were identified. Contrast filled the filling 2 cm from the meatus in the dor- penile urethra, bulbous urethra, prostatic urethra, sal penoscrotal, with a total dead-end and bladder. There was no stenosis and there were impression (a), and contrast filled the no tracts to another place (Figure 2b). penile urethra, bulbous urethra, pros- Patients had no other complaint besides a whit- tatic urethra, and bladder (b) ish discharge from a meatus in the dorsal penoscrotal which is then followed by the presence of pain and redness around it. The patient was then planned for exploration and excision of the tract from the meatus in the dorsal penoscrotal.

Surgical Technique Patient was positioned under general anaesthe- sia. The aseptic and antiseptic procedure was conducted at surgical site and the surrounding area. Evaluation of genitalia external was carried out; the Figure 3 P enile degloving and identification the external urethral meatus was located at the tip of dorsal tract (a), the dorsal tract appears glans penis, two testicles were found normal, and to the symphysis (b) there was a second meatus at the dorsal penoscrotal. Mattress stitch on glans penis using suturing thread prolene 4-0. Foley catheter 8 Fr was inserted at the opening of external urethral meatus at the tip of the glans penis. Circumglandular incision was made following marker and penile degloving was conducted, identification of the dorsal tract (Figure 3a). The dorsal tract appears to the symphysis (Figure 3b). Excision of the dorsal tract Figure 4 E xcision of the dorsal tract and released and released up to the symphysis, obliteration of the up to the symphysis (a), excised tissue tissue appeared (Figure 4). The tissue was sent to be of the dorsal tract as long as 2 cm (b)

588 Published by DiscoverSys | Bali Med J 2019; 8(3): 587-591 | doi: 10.15562/bmj.v8i3.1602 CASE REPORT

examined into the anatomical pathology laborato- bones.5 Arena et al.,6 found that in 60% of cases rium. Raw surface was the covered using the byars is accompanied by genitourinary malformation flap technique with PDS 5-0 interrupted (Figure 5). such as ureteropelvic junction obstruction, extra rotation of the penis, vesicoureteral reflux, renal RESULT ectopia, renal agenesis, or posterior urethral valves. There was no additional abnormality in our patient. The results of examination from anatomical pathol- Various theories have been proposed to explain ogy of the tissue shown a picture of the urethra the embryological development of urethral dupli- with a chronic inflammatory reaction and no signs cation, including disorders of mesoderm develop- of malignancy appeared. Ten days after surgery ment, abnormal Mullerian ducts, ischemia during (Figure 6), patient came back to our urology poly- embryogenesis, and defects in the development of clinic. Physical examination showed good surgical the urogenital sinus.7–10 Casselman and Williams10 wound healing, there was no discharge from the suggested that a partial failure or an irregularity surgical wound in the dorsal penoscrotal was found of the ingrowth of the lateral mesoderm between as before and there were no problems with voiding. the ectodermal and endodermal layers of the cloacal membrane in the midline accounts for the DISCUSSION forms with a dorsal epispadias channel. Das and Brosman8 stated that abnormal termination of the Double urethra or urethral duplication is a congen- Mullerian duct was responsible for the development ital disorder which rarely happens. Approximately of urethral duplication. Rica et al.,11 reported that 150 – 300 cases have been reported to date and is asymmetry in the closure of the urorectal septum more frequently found in male children, with a results in an urethra-perineal fistula. previous literature recording no more than 40 cases There were a few anatomical variations of of double urethra found in female children. This urethral duplication. Duplication may occur at disorder is often associated with other abnormal- bladder neck area or only affecting the distal part ities in the genitourinary tract, heart, bowel, and of the urethra.12,13 This disorder commonly occurs on the sagittal section, with urethra on the dorsal part and ventral of penis. Ventral urethra is usually more functional as it has sphincter and verumontanum mechanisms. Urethral duplication rarely occurs on the coronal section.12–14 There are many classifications used to categorise urethral duplication. Gross and Moore classified urethral duplication as a complete second passage originating from the bladder to the dorsum of the penis and or as an accessory pathway which is unfunctional that ends blindly on the dorsal or ventral surface. Williams and Kenawi15 categorised urethral duplication based on the ectopic location of the urethra compared to normal urethra. However, the most commonly used and accepted in this area recently is the Effmann classification. Effman categorised urethral duplication into three types. Type I is Figure 5 Raw surface was the covered using the composed of incomplete with blind-ending and/ byars flap technique or accessory urethra, if the accessory urethra opens onto the dorsal or ventral surface of the penis without communicating with principal urethra or bladder it is classified as type IA and classified as type IB when the accessory urethra emerges from principal urethra and terminates blindly within the periure- thral tissue. Type II duplication is complete patent urethral duplication with four subtypes, namely type IIA 1 when the second channel of the urethra arising independently from the bladder, type IIA 2 if the second channel arising from the first urethra Figure 6 Th e surgical wound ten days after and courses into a second meatus, when the second surgery meatus located in the perineum it is classified as

Published by DiscoverSys | Bali Med J 2019; 8(3): 587-591 | doi: 10.15562/bmj.v8i3.1602 589 CASE REPORT

type IIA “Y”, and type IIB with the second channel urethral duplication, some experts recommended arising independently from the bladder but joining that surgical management is unnecessary in asymp- the first and coursing into one meatus. Type III is tomatic patients. Surgical treatment is needed for when urethral duplication as a component of partial reconstructing deformities, such as dorsal meatus, or complete caudal duplication, usually associated to fix the emission sites of urine, incontinence, with bladder duplication.16 recurrent urinary tract infections, obstructive This classification is more functional, repre- symptoms as well as to mend urinary leakage on senting all clinical types of urethral duplication.17 the perineal area.23,27 Surgical management can be Eiffmann type IIA 2”Y” is the most common type conducted with various techniques, consisting of reported of urethral duplication18–20 and usually one step to two steps of surgery, with simple coagu- associated with severe congenital anomalies like lation or reconstructive surgery, depending the type imperforate anus, cloacal atrophy, conjoined of urethral duplication. If the two urethral orifices twins, early amnion rupture syndrome, prune are too close to each other on the glans, then the belly syndrome and hand-foot genital syndrome.21 septum between the two meatus can be excised to According to this classification, our patient give a single urinary meatus. Other than this, most belonged to type IA. Although this classification procedures involve excision of the accessory urethra is considered to be the most exhaustive, it does with reconstruction of the normal urethra.18,28 The not distinguish sagittal from coronal duplication simplified treatment scheme proposed by Salle at and does not recognise female form. Lima at al.22 al. based on the type of urethral duplication from suggested a new classification, the proposed clas- Effmann classification. In the cases of type IA sification scheme is based on the required surgi- urethral duplicationspectrum above they proposed cal approach, this classification is more complete meatoplasty or exeresis of the accessory urethra. and includes coronal and sagittal pieces and also Urethro-urethrostomy can be done with or without divided between men and women. urethroplasty for the type IIA 1 while for the type Diagnosis of urethral duplication is based on IIA 2 the treatment is urethroplasty. They proposed simple physical examination of the penis and is to exeresis the non-functional bladder for type III confirmed by a voiding cystourethrography or urethral duplication in sagittal plane and exeresis retrograde urethrogram to identification the pres- bladder septum or hemibladder plus reimplan- ence of urethral duplication and its anatomical tation of the urethra in the contralateral bladder type.23 Ultrasound may be helpful in the diagnosis of zone for the type III urethral duplication in coro- other associated anomalies.23,24 Upper urinary tract nal plane without diphallia and if accompanied by abnormalities are found in up to 80% of patients diphallia penoplasty and glans plasty is needed. No and are diagnosed with intravenous urography or surgical intervention is needed for cases of urethral ultrasonography.5 Clinical signs and symptoms duplication type IB and IIB.29 of urethral duplication are diverse. Patients may In our case the accessory urethra was excised complain of intermittent discharge from the acces- completely, we did that procedure in accordance sory urethra having two emission sites of urine, with the treatment scheme proposed by Salle at al. incontinence, recurrent urinary tract infections, because in this Type 1A urethral duplication the obstructive symptoms, or no symptoms at all.25,26 In accessory urethra was blind-ending in distal loca- our patient there were no complaints experienced tion and was not connected to the principal urethra before and the until there was sign of infection in or bladder. The outcome of this patient was very the accessory urethra with sign and symptoms in good. the form of whitish discharge from meatus of accessory urethra. CONCLUSION The first step in the management of urethral duplication is to recognise the normal-functioning Urethral duplication is generally an uncommon urethra; in fact, the true urethra is that with the congenital abnormality in urological practice largest calibre, a normal verumontanum, and an presenting in various types and some with associ- intact sphincter. Urethroscopy can be conducted ated other malformation, which the aetiology and to evaluate the diameter and the presence of veru- mechanism are unknown. Signs and clinical symp- montanum.23 Treatment of urethral duplication toms of these disorders vary; hence management of should be individualised based on the anatomic the patients must be individualised. Surgical treat- types and also clinical findings and severity of the ment is needed for reconstructing deformities. The accompanying anomaly. Many patients are asymp- patient presented with Type IA urethral duplica- tomatic and do not require any surgery. There is no tion. He remains symptom-free eight months after clear consensus yet regarding the management of complete excision of the accessory urethra.

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CONFLICT OF INTEREST 13. Palmer LS, Palmer JS. Management of Abnormalities of the External Genitalia in Boys. In: Campbell-Walsh Urology The authors affirm no conflict of interest in this Eleventh Edition. 2016. p. 3379–80. 14. Onofre LS, Gomes AL, Leão JQDS, Leão FG, Cruz TMA, study. Carnevale J. Urethral duplication - A wide spectrum of anomalies. J Pediatr Urol. 2013;9:1064–71. 15. Williams D, Kenawi M. Urethral duplication in the male. FUNDING Eur Urol. 1975;1:209–15. 16. Effmann E, Lebowitz R, Colodny A. Duplication of the There is no external funding done for this study. urethra. Radiology. 1976;119:179–85. All expenses were covered by the institution and 17. Maitama HY, Tella UM, Mbibu HN. Urethral duplication: Case report and literature review. Ann Afr Med. authors. 2012;11:186. 18. Podesta M, Medel R, Castera R, Ruarte A. Urethra duplication in children: Surgical treatment and results. J Urol. AUTHOR CONTRIBUTIONS 1998;160:1830–3. 19. Wagner J, Carr M, Bauer S, AH C, Retik A, Hendren W. Y.I.H made assessment of the case, performed the Congenital posterior urethral perineal fistulae: A unique operation, followed up the patient, gifted knowledge form of urethral duplication. Urology. 1996;48:277–80. 20. Slavov C, Donkov I, Popov E. Case of duplication of the and supervised the case; M.A collected the data, urethra in an adult male, presenting with symptoms of followed up the patient and wrote the manuscript. bladder outlet obstruction. Eur Urol. 2007;52:1249–51. 21. Stephens F. Abnormal Embryology-cloacal dysgenesis. Congenital Malformations of the Urinary Tract. Part 1, REFERENCES Chapter 2. Vol. 5, New York: Praeger Scientific. p. 1983. 15–52. 1. Coleman RA, Winkle DC, Borzi PA. Urethral duplica- 22. Lima M, Destro F, Maffi M, Persichetti Proietti D, tion: Cases of ventral and dorsal complete duplication and Ruggeri G. Practical and functional classification of review of the literature. J Pediatr Urol. 2010;6:188–91. the double urethra: A variable, complex and fascinat- 2. Ismail A, Al Rayes T, Alsalihi M, Abbas T. Female double ing malformation observed in 20 patients. J Pediatr Urol urethra: A case report. J Pediatr Surg. 2012;47:e29–31. [Internet]. 2017;13:42.e1-42.e7. 3. Abouzeid AA, Safoury HS, Mohammad SA, el-Naggar O, 23. Okur MH, Aydogdu B, Arslan S, Basuguy E, Zeytun H, Zaki AM, Hassan TA, et al. The double urethra: Revisiting Arslan MŞ. Type II A2 duplication of urethra in an the surgical classification. Ther Adv Urol. 2015;7:76–84. 8-month-old male child: A case report. Pediatr Urol Case 4. Lopes RI, Giron AM, Mello MF, Neto CMB, dos Santos J, Reports. 2015;2:17–22. Moscardi PRM, et al. Urethral duplication type influences 24. Baid M, Dutta A. Urethral duplication in a 15-year- on the complications rate and number of surgical proce- old: case report with review of the literature. Rev Urol. dures. Int Braz J Urol. 2017;43:1144–51. 2014;16:149–51. 5. Haleblian G, Kraklau D, Wilcox D, Duffy P, Ransley P, 25. Erdil H, Mavi A, Erdil S, Gumusburun E. Urethral Mushtaq I. Y-type urethral duplication in the male. BJU Duplication. Acta Med Okayama. 2003;57:91–3. Int. 2006;97:597–602. 26. Gozar H, Prișcă R, Derzsi Z. Two Cases of Dorsal Urethral 6. Arena S, Arena C, Scuderi MG, Sanges G, Arena F, Di Duplication. Acta Medica Marisiensis. 2013;59:52–4. Benedetto V. Urethral duplication in males: Our experi- 27. Cicek T, Kizilkan Y, Coban G, Kosan M. Urethral duplica- ence in ten cases. Pediatr Surg Int. 2007;23:789–94. tion: A rare cause of recurrent urinary infection. J Pak Med 7. Woodhouse C, Williams D. Duplications of the lower uri- Assoc. 2015;65:90–2. nary tract in children. Br J Urol. 1979;51:481–7. 28. Middleton AJ, Melzer R. Duplicated urethra: an anomaly 8. Das S, Brosman S. Duplication of the male urethra. J Urol. best repaired. Urology. 1992;39:538–42. 1977;117:452–4. 29. Salle J, Sibai H, Rosenstain D, Brzezinski A, Corcos J. 9. Sánchez M, Vellibre R, Castelo J, Arias M, Sarmiento R, Urethral duplication in the male: review of 16 cases. J Urol. Costa A. A new case of male Y-type urethral duplication 2000;163:1936–40. and review of literature. J Pediatr Surg. 2006;41:e69-71. 10. Casselman J, Williams D. Duplication of the urethra. Acta Urol Belg. 1966;34:535–41. 11. Rica P, Holder T, Ashcraft K. Congenital posterior urethral perineal fistula: a case report. J Urol. 1978;199:416–7. 12. Alanee S, Gupta P, Gleich P, Shukla AR. Complete urethral duplication: Description of surgical approach mim- This work is licensed under a Creative Commons Attribution icking distal epispadias repair. J Pediatr Urol [Internet]. 2012;8:343–7.

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