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Congenital penile urethrocutaneous fistula: A rare anomaly and review of literature.
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MedLine Citation:
PMID:  21976932     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Congenital penile urethrocutaneous fistula is described as an unusual developmental anomaly in children who present with an abnormal opening on the ventral aspect of penis with a normal foreskin and an absence of chordee and hypospadias. The authors present a discussion on the etiology, embryology, and management of this entity along with a description of three cases. We emphasize meticulous clinical examination for the diagnosis and to rule out other associated anomalies.
Authors:
Ram Mohan Shukla; Biswanath Mukhopadhyay; Kartik Chandra Mandal; Shib Sankar Barman
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Urology annals     Volume:  3     ISSN:  0974-7834     ISO Abbreviation:  Urol Ann     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-10-06     Completed Date:  2011-11-10     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  101510823     Medline TA:  Urol Ann     Country:  India    
Other Details:
Languages:  eng     Pagination:  161-3     Citation Subset:  -    
Affiliation:
Department of Pediatric Surgery, Nil Ratan Sircar Medical College and Hospital, Kolkata, India.
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Journal Information
Journal ID (nlm-ta): Urol Ann
Journal ID (publisher-id): UA
ISSN: 0974-7796
ISSN: 0974-7834
Publisher: Medknow Publications, India
Article Information
© Urology Annals
open-access:
Received Day: 27 Month: 8 Year: 2010
Accepted Day: 03 Month: 11 Year: 2010
Print publication date: Season: Sep-Dec Year: 2011
Volume: 3 Issue: 3
First Page: 161 Last Page: 163
ID: 3183711
PubMed Id: 21976932
Publisher Id: UA-3-161
DOI: 10.4103/0974-7796.84953

Congenital penile urethrocutaneous fistula: A rare anomaly and review of literature
Ram Mohan Shuklaaff1
Biswanath Mukhopadhyayaff1
Kartik Chandra Mandalaff1
Shib Sankar Barmanaff1
Department of Pediatric Surgery, Nil Ratan Sircar Medical College and Hospital, Kolkata, India
Correspondence: Address for correspondence: Dr. Biswanath Mukhopadhyay, 7E, Dinobandhu Mukherjee Lane, Sibpur, Howrah - 711 102, West Bengal, India. E-mail: drbmukhopadhyay@yahoo.co.in

INTRODUCTION

Congenital urethral fistula and congenital chordee without hypospadias are best explained by the theory of deficient urethral plate.[1] This abnormality usually is an isolated deformity, but may be associated with imperforate anus, hypospadias, or ventral chordee.[25] The cause is unclear, but probably reflects a focal defect in the urethral plate that prevents fusion of the urethral folds.[5] Thirty-two cases of congenital urethral fistula were reported till 1999 that included cases with associated anomalies also.[6] Since then around half a dozen more such cases have been reported, most of them being isolated ones without any associated anomalies.[713] Here, we report three cases of congenital anterior penile urethrocutaneous fistula out of which two are isolated and one is associated with Anorectal malformation (ARM); management plans are discussed with review of literature.


CASE REPORTS
Case 1

A 1-year and 6-month-old male baby presented with complaints of absence of anal opening since birth with status divided sigmoid colostomy done outside and an abnormal opening on the undersurface of penis, which had been present since birth. The patient was referred for definitive surgery for ARM from a district hospital. The patient was passing urine from both the terminal glandular meatus and the fistulous opening. There was no history of any trauma, strangulation, stone impaction, or surgery. There were no signs of inflammation. On examination of perineum, gluteal folds were well developed with a midline groove and an ovoid shape urethrocutaneous fistula with clear margins measuring 2 cm by 1 cm was present on the ventral aspect of penile urethra approximately 3 cm distal to penoscrotal junction [Figure 1]. The external urethral meatus appeared normal with normal foreskin and without any chordee or hypospadias. Distal colostogram showed intermediate anomaly with rectourethral fistula. Ultrasonography of abdomen, micturating cystourethrogram, Hemogram, blood urea, and serum creatinine were normal. After anterior sagittal anorectoplasty tubularized incised plate urethroplasty was planned with the aim to reconstruct a near-normal caliber neourethra. After applying stay sutures, proposed circumferential and racket-shaped incisions incorporating the fistulous opening were marked. The penile shaft skin was degloved, fistulous margins were mobilized and urethral plate ‘relaxing incision’ was made in through fistulous opening. The margins of fistulous opening were tubularized on 6F Nelaton's catheter. The neourethral suture line was reinforced with dartos flap. The penile skin was rotated ventrally to provide skin coverage. The postoperative period remained uneventful, and presently patient is doing well for the last 2 years.

Case 2

A 3-month-old male presented with an abnormal opening on the ventral aspect of penile urethra, which had been present since birth. The patient was passing urine via normal meatus and the congenital fistula. There was no previous history of surgery, trauma, stone impaction, or strangulation. On examination, there was normal prepuce, glans, external urinary meatus, and penile shaft without chordee. A congenital urethrocutaneous fistula was present in the mid penile region measuring 1 cm × 0.5 cm in size [Figure 2] approximately 2.5 cms distal to penoscrotal junction with well-defined margins. There were no signs of inflammation and urethra distal to the fistula appeared to be normal on clinical examination. A catheter was passed through the fistula, which came out of the glandular meatus suggesting intact distal urethra. Routine blood tests, urine examination, micturating cystourethrogram, and abdominal ultrasonography were normal. At the age of 8 months, fistula was circumcised and then closed using local skin turn down flaps. An additional layer of local soft tissue was used to re-enforce the repair before final closure of fistula by local skin rotation flap. A 6F Nelaton's catheter was left in the bladder for drainage. The postoperative period remained uneventful. After 12 months of follow-up, the patient is having normal voiding and there has been no recurrence.

Case 3

An 8-month-old baby was referred to us with complaint of passing urine from 2 openings, which had been present since birth. There was no previous history of surgery, trauma, stone impaction or strangulation. The patient used to pass most of the urine from the urethral opening and only a small amount through the fistula opening. On examination, a fistula of size 0.5 cms × 0.5 cms with well-defined margins was present in the penoscrotal region [Figure 3]. There were no signs of inflammation present and urethra distal to the fistula appeared to be normal clinically. The external urethral meatus was normal, with an absence of chordee or hypospadias. All basic investigations were normal. Surgery was performed by circumcising the fistula and then closing using local skin turn down flaps. The patient recovered satisfactorily and is on regular follow-up for the last 7 months and is doing well.


DISCUSSION

Congenital anterior urethrocutaneous fistula is not commonly associated with imperforate anus; this association is more common in posterior urethrocutaneous fistula, which may represent Y type urethral duplication. Urethra develops from urogenital membrane and this urogenital membrane is the anterior portion of cloacal membrane after it has been divided by urorectal septum. Therefore, a primary defect in the urogenital membrane and cloacal membrane may explain associated imperforate anus and congenital urethrocutaneous fistula.[14]

The etiopathogenesis of congenital penile urethrocutaneous fistula still remains unclear.[6, 9, 15, 16] Although its etiopathogenesis has been explained by the theory of pressure necrosis of penile urethra by fetal parts, yet an absence of scarring in the present and a few reported cases supports the theory of focal developmental defect of urethral plate, which prevents the fusion of urethral folds, thus resulting in isolated congenital penile urethrocutaneous fistula.[4, 9, 15] Olbourne suggested that the fistula probably reflects a focal or temporary defect in the urethral plate function that results in a complete defect or a partial deficiency of urethral fold fusion.[5] Similarly, the testosterone or androgen receptors may be at fault leading to the development of fistula.[1] The distal glanular penile urethra is normal as it develops from a surface ectodermal invagination.[4] A deficiency of spongiosum with complete canalization of glanular urethra may represent an abnormality of the anlage of corpus spongiosum which is derived from the inner genital folds. Distal type of fistula may be explained by misalignment of the glanular and penile urethra,[6] as seen in one of our cases.

Surgical approach to repair congenital anterior urethrocutaneous fistula depends upon the type of fistula. In cases of an isolated fistula with intact spongiosum, repair with local flaps are sufficient but if they are associated with deficient distal urethra or spongiosum, associated chordee or hypospadias then formal hypospadias repair is recommended.[3] To conclude, congenital penile urethrocutaneous fistula is an extremely rare but easily manageable anomaly and a good clinical examination is needed for diagnosis and to rule out other associated anomalies.


Notes

Source of Support: Nil

Conflict of Interest: None.

REFERENCES
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2. Elder JS. Wein AJAbnormalities of the genitalia in boys and their surgical managementCampbell-Walsh UrologyYear: 20079th edPhiladelphiaSaunders, Elsevier37456
3. Welch KJ. Ravitch MM,Benson CD,Aberdeen E,Randolph JGHypospadiasPediatric surgeryYear: 19793rd edChicagoYear book Medical Publishers135376
4. Ritchey ML,Sinha A,Argueso L. Congenital fistula of the penile urethraJ UrolYear: 1994151106128126793
5. Olbourne NA. Congenital urethral fistula: Case reportsPlast Reconstr SurgYear: 19765723791250897
6. Caldamone AA,Chen SC,Elder JS,Ritchey ML,Diamond DA,Koyle MA. Congenital anterior urethrocutaneous fistulaJ UrolYear: 19991621430210492230
7. Harjai MM. Congenital urethrocutaneous fistulaPediatr Surg IntYear: 200016386710955569
8. Nakane A,Hayashi Y,Kojima Y,Mizuno K,Okada A,Sasaki S,et al. Congenital urethrocutaneous fistulaInt J UrolYear: 20007343411020060
9. Islam MK. Congenital penile urethrocutaneous fistulaIndian J PediatrYear: 200168785611563258
10. Agarwal P. Congenital anterior urethrocutaneous fistula revisitedIndian J Plast SurgYear: 200437646
11. Akman RY,Cam K,Akyuz O,Erol A. Isolated congenital urethrocutaneous fistulaInt J UrolYear: 200512417815948735
12. Ceylan K,Köseoğlu B,Tan O,Atik B. Urethrocutaneous fistula: A case reportInt Urol NephrolYear: 200638163516502075
13. Rashid KA,Kureel SN,Tandon RK. Congenital anterior penile isolated urethrocutaneous fistula: A case reportAfr J Paediatr SurgYear: 2008552319858667
14. Kluth D,Hillen M,Lambrecht W. The principles of normal and abnormal hindgut developmentJ Pediatr SurgYear: 199530114377472968
15. Betalli P,Carretto E,Midrio P,Zanon GF,Gamba PG. A new indication for buccal mucosal graft: Isolated congenital fistula of the penile urethraPediatr Surg IntYear: 200319586714556028
16. Maarafie A,Azmy AF. Congenital fistula of the penile urethraBr J UrolYear: 1997798149158532

Figures

[Figure ID: F1]
Figure 1 

Congenital urethral fistula in a patient of anorectal malformation



[Figure ID: F2]
Figure 2 

Isolated congenital urethral fistula



[Figure ID: F3]
Figure 3 

Fistula in penoscrotal junction



Article Categories:
  • Case Report

Keywords: Anorectal malformations, hypospadias, urethrocutaneous abnormalities, urethrocutaneous fistula.

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