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Congenital anterior urethrocutaneous fistula: Two case reports and review of literature.
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MedLine Citation:
PMID:  25125898     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Congenital anterior urethrocutaneous fistula (CAUF) of the male urethra is a rare anomaly. CAUF can be defined as a urethral fistula in which the urethra and external urethral meatus are intact, typically with intact prepuce and no history of any penile trauma (including surgical trauma).We report 2 cases of CAUF to highlight this pathology with hints of the published similar cases in the literatures and discuss the surgical reconstruction ways for management.
Authors:
Hamdan H Alhazmi
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Urology annals     Volume:  6     ISSN:  0974-7796     ISO Abbreviation:  Urol Ann     Publication Date:  2014 Jul 
Date Detail:
Created Date:  2014-08-15     Completed Date:  2014-08-15     Revised Date:  2014-08-18    
Medline Journal Info:
Nlm Unique ID:  101510823     Medline TA:  Urol Ann     Country:  India    
Other Details:
Languages:  eng     Pagination:  239-41     Citation Subset:  -    
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Full Text
Journal Information
Journal ID (nlm-ta): Urol Ann
Journal ID (iso-abbrev): Urol Ann
Journal ID (publisher-id): UA
ISSN: 0974-7796
ISSN: 0974-7834
Publisher: Medknow Publications & Media Pvt Ltd, India
Article Information
Copyright: © Urology Annals
open-access:
Received Day: 26 Month: 3 Year: 2013
Accepted Day: 29 Month: 5 Year: 2013
Print publication date: Season: Jul-Sep Year: 2014
Volume: 6 Issue: 3
First Page: 239 Last Page: 241
PubMed Id: 25125898
ID: 4127862
Publisher Id: UA-6-239
DOI: 10.4103/0974-7796.134285

Congenital anterior urethrocutaneous fistula: Two case reports and review of literature
Hamdan H. Alhazmi, MDaff1
Department of Surgery, College of Medicine and King Khalid University Hospital, King Saud University, Saudi Arabia
Correspondence: Address for correspondence: Dr. Hamdan H. Alhazmi, Department of Surgery-Division of Urology, College of Medicine and King Khalid University Hospital, King Saud University, P.O.BOX.7805(37), RIYADH 11472. E-mail: drhamdan@ksu.edu.sa

INTRODUCTION

Congenital anterior urethrocutaneous fistula (CAUF) of the male urethra is a rare anomaly of unknown etiology, while urethrocutaneous fistula after hypospadias repair is a common complication. CAUF is usually positioned distally. It was first reported by Campbell in 1951.[1] Caldamone et al, 1999, reported the largest series (14 cases).[2] Forty-five observations have been reported in the literature to date.[3, 4, 5, 6, 7] We report 2 cases of CAUF with review of all previously published 45 cases.


CASE REPORT
Case 1

A 6-month-old boy presented with a fistula located on ventral penile urethra since birth. There was no history of trauma or surgical intervention. He was passing urine mostly through this fistula and also from the external urethral meatus. The preputial skin was intact, and there were no other anomalies. The fistula size was 8 × 5 mm, just below the coronal sulcus. The patient was operated on at the age of 1 year. A 10-F urethral catheter inserted in the external meatus and passed through the fistulous opening easily [Figure 1]. At the operation, cystoscopic evaluation was normal. The fistula was closed primarily by multi-layer closure of the urethral plate with vicryl 6/0 sutures over a 10 Fr feeding tube and an interpositioned dartos flap [Figure 2a and 2b]. The hypospadias silicon stent (8 Fr) was kept in place for 7 days and after removal, the patient voided normally. After 1-year follow up, the child was healthy without any complaint.

Case 2

A 2-month-old boy presented with a fistula located on his ventral penile urethra after circumcision, just below the coronal sulcus it measured 10 by 8 mm. Although the pathology of prepuce is not examined for urothelium to exclude iatrogenic cause of fistula, the margins of the fistula were completely mature just after circumcision. He was passing urine mostly through this fistula. Both testicles were in place and there were no other anomalies. The patient was operated on when he was 10 month old. At the operation, there was distal penile megalourethra proximal to the fistula. The fistula excision and reduction urethroplasty was done tell the normal-caliber urethra at penoscrotal junction. The 3-cm urethral defect was closed primarily by multi-layer closure with vicryl 6/0 sutures over a 10 Fr feeding tube and an interpositioned dartos flap. The hypospadias silicon stent (8 Fr) was kept in place for 7 days and after removal, the patient voided normally and was discharged. Postoperatively, he had tiny coronal urethrocutaneous fistula which was closed spontaneously within 3 months. After 6-month follow up, the child was healthy without any complaint.


DISCUSSION

CAUF has been named as criptospadias (an unusual type of hypospadias). Some patients have undergone circumcision before diagnosis, raising the possibility that the fistula may have been iatrogenic. But, the fact that most of the reported patients were uncircumcised (31/47) confirms the congenital nature of this lesion.[3, 4, 5, 6, 7]

Most of CAUF are coronal or subcoronal. The fistula was at the distal shaft in 24/47 patients, mid shaft in 13/47, penoscrotal junction in 3/47, and non-specified in 7/47.[3, 4, 5, 6, 7]

CAUF may be isolated (29/47; 62%) or associated with other pathology as shown in Table 1. Our second case is the first report of CAUF with megalourethra.

In male fetus, urethral development and sexual differentiation begin approximately in utero at 8 weeks and are completed in 15 weeks. The male urethra is formed by urethral folds fusion along the ventral surface of the penis, which extends to the coronal sulcus. The embryonic male urethra has three separate parts. The proximal portion was formed by the portion above the Wolffian duct opening including verumantanum utricle and urogenital sinus. The second segment extends from the verumantanum to the base of the glans. The glans segment is formed separately. Failure of fusion of urethral folds leads on to various levels of hypospadias or in extremely rare occasions may lead to congenital urethrocutaneous fistula. According to the level at which the lack of fusion occurs, such anomalies can be localized anywhere from perineum to glans.[4]

The etiology of congenital urethrocutaneous fistula is not clear yet, but several hypotheses have been proposed. Some authors believe that the etiology may be same as in hypospadias. Coronal type of fistula may be explained by misalignment of the glanular and penile urethra. It may represent the ventral functional limb of a Y-type urethral duplication. Other explanation could be the blowout phenomenon as stated by Campbell;[1] such theory can be supported by one case report with a small ventral penile cyst at birth, resembling an epithelial inclusion cyst, which ruptured at 1-2 weeks of age and became a small fistula.[7] For cases associated with ARM (anorectal malformation), the defect in the urethral plate, which is the ventral extension of the cloacal plate, may affect the cloacal membrane as well.[3]

Repair should be individualized to the defect. Initially, clinical examination, retrograde and voiding urethrocystogram, or cystourethroscopic examination may be required to exclude the associated anomalies as urethral duplication and ARM.[3, 4] Treatment usually consists of one of two techniques: (1) for isolated cases; the fistula can be circumscribed and then closed in simple multilayer fistula closure, or (2) for more complex cases, closure requires straightening of the penis, ventral glans can be opened through the distal urethra and reconstruction of the urethra all the way to the most distal point of the glans by the more complex reconstruction techniques employed in formal hypospadias surgery as Thiersch-Duplay tubularization or tubularization and incision of the urethral plate technique, pedicled island preputial tube or onlay urethroplasty, and buccal mucosa urethroplasty.[3, 5, 7] Endoscopic treatment of isolated congenital urethroperineal fistula had been reported.[6] Primary repair was successful in 42/47 of cases, 4 patients required two operations and 1 patient required three closures.[3, 7]


CONCLUSION

CAUF is a rare anomaly with almost 90% success rate after single surgical procedure. Associated anomalies should be excluded before deciding surgical correction.


Notes

Source of Support: Nil

Conflict of Interest: None.

ACKNOWLEDGMENT

Great thanks to Professor Khalid Fouda, Dr. Ahmed Abdulaziz and Dr. Mahmoud Salem, who have assisted me in completing this work.


REFERENCES
1. Campbell M. Clinical Pediatric UrologyYear: 1951PhiladelphiaW. B. Saunders Co5313
2. Caldamone AA,Chen SC,Elder JS,et al. Congenital anterior urethrocutaneous fistulaJ UrolYear: 19991621430210492230
3. Galinier P,Mouttalib S,Carfagna L,Vaysse P,Moscovici J. Congenital anterior urethrocutaneous fistula associated with a stenosis of the bulbar urethra in the context of high anorectal malformation without fistulaJ Plast Reconstr Aesthet SurgYear: 200962e11318694660
4. Rashid KA,Kureel SN,Tandon RK. Congenital anterior penile isolated urethrocutaneous fistula: A case reportAfr J Paediatr SurgYear: 2008552319858667
5. Ceylan K,Köseoðlu B,Tan O,Atik B. Urethrocutaneous fistula: A case reportInt Urol NephrolYear: 200638163516502075
6. Bedir S,Kilciler M,Ozgok Y. Case report: Endoscopic treatment of isolated congenital urethroperineal fistulaJ EndourolYear: 20062042416426132
7. Akman RY,Cam K,Akyuz O,Erol A. Isolated congenital urethrocutaneous fistulaInt J UrolYear: 200512417815948735

Figures

[Figure ID: F1]
Figure 1 

Congenital anterior urethrocutaneous fistula with catheter in the urethra



[Figure ID: F2]
Figure 2 

Intra-operative repair of congenital anterior urethrocutaneous fistula (a) dissection of the fistula, (b) good urine stream from external urethral meatus after completion of a Thiersch-Duplay repair



Tables
[TableWrap ID: T1] Table 1 

Congenital anomalies associated with congenital anterior urethrocutaneous fistula




Article Categories:
  • Case Report

Keywords: Anterior, congenital, fistula, urethrocutaneous.

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