|Year : 2014 | Volume
| Issue : 3 | Page : 186-187
Congenital urethrocutaneous fistula: A rare isolated lesion
Vijay C Pujar, Shirin S Joshi
Department of Pediatric Surgery, KLE University, Belgaum, Karnataka, India
|Date of Web Publication||19-Sep-2014|
Vijay C Pujar
Department of Pediatric Surgery, KLE University, Belgaum, Karnataka
Source of Support: None, Conflict of Interest: None
Urethrocutaneous fistula (UCF) are common after hypospadias repair, but occurrence of congenital UCF without hypospadias is extremely rare and only about 35 cases are reported in the literature. The etiology is not established yet. We report a rare case of isolated congenital UCF in 1 year child managed by Snodgrass technique and review of literature regarding the etiology and management.
Keywords: Congenital fistula, hypospadias, urethrocutaneous
|How to cite this article:|
Pujar VC, Joshi SS. Congenital urethrocutaneous fistula: A rare isolated lesion. J Sci Soc 2014;41:186-7
| Introduction|| |
Congenital anterior urethrocutaneous fistula (UCF) is a rare anomaly that may present in an isolated fashion or in association with hypospadias or chordee. This has been described as unusual type of hypospadias, cryptospadias and congenital UCF. , Congenital urethral fistula and congenital chordee without hypospadias are best explained by the theory of deficient urethral plate.  This abnormality, usually is an isolated deformity, but may be associated with imperforate anus, hypospadias, or ventral chordee.  The cause is unclear, but probably reflects a focal defect in the urethral plate that prevents fusion of the urethral folds. 
| Case report|| |
The case we present here is about 1 year-old male child who was brought with the complaints of passing urine from the abnormal opening over the ventral aspect of penis since birth. There was no history of any inflammatory lesion or trauma at that site. Child was passing entire urine through this abnormal opening.
On examination, child had wide UCF at mid penile level. Proximal ½ cm urethra appeared hypoplastic covered with thin skin [Figure 1]. Distal urethra, prepuce, and meatus were normal. Both testicles were descended and normally placed. Infant feeding tube passed through meatus would emerge out through fistula.
Routine blood investigations ultrasonography did not reveal any abnormality. Cystoscopic examination performed to rule out urethral duplication. At operation, the fistula was spatulated proximally until healthy urethra was seen. Fistula was closed over an infant feeding (7F) tube by Snodgrass technique. Dartos fascia and skin were closed separately [Figure 2]. Catheter was removed on 7 th postoperative day and child is voiding through meatus and there was no recurrence of the fistula.
| Discussion|| |
An isolated congenital anterior UCF without anorectal malformations, hypospadias or chordee is an extremely rare anomaly reported in literature. The etiology of congenital UCF is not clear yet, but several etiologic hypotheses have been proposed.
The association of imperforate anus with urethral abnormalities is known as urogenital membrane forming the urethra is the part of cloacal membrane, which in turn forms the anorectal region. 
Although popular etiopathogenesis has been explained by the theory of pressure necrosis of penile urethra by fetal parts, yet an absence of scarring in the present case 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 UCF. ,,
The theory of development of UCF due to failure of fusion of urethral plate ventrally as seen in hypospadias was proposed by Olbourne.  Similarly, the testosterone or androgen receptors may be at fault leading to the development of fistula.  The distal glandular penile urethra is normal as it develops from a surface ectodermal invagination.  A deficiency of spongiosum with complete canalization of glandular 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 glandular and penile urethra  as seen with fistula at coronal level.
Segmental deficiency of the corpus spongiosum or penile fascia, minor local trauma or infection caused fistula between the skin and a poorly covered urethral tube beneath it  is the alternative theory proposed.
Another hypothesis is that increasing urethral pressure proximally because of a meatal obstructive lesion causes urethra thinning and eventually a fistula. 
In our case, there was a local developmental deficiency of the corpus spongiosum around the fistula was the probable cause and no urethral obstructive lesions was noticed.
Type of repair of UCFs should be governed by the local findings like status of corpus spongiosum, skin and distal urethra. Local flap repair may be sufficient in children with intact corpus spongiosum. Deficient corpus or distal urethral plate abnormalities need formal hypospadias repair. 
Different surgical techniques including pedicle, prepuce based skin flap, direct fistula closure or modified Denis Browne urethroplasty have been proposed in the repair of congenital penile UCF. Recently, Snodgrass technique of tubularized incised plate is also used for this condition. The technique should be decided on the local findings in each case.
| Conclusion|| |
Congenital penile UCF is an extremely rare but easily manageable anomaly and needs to rule out associated anomalies.
| References|| |
|1.||Goldstein M. Congenital urethral fistula with chordee. J Urol 1975;113:138-40. |
|2.||Elder JS. Abnormalities of the genitalia in boys and their surgical management. In: Wein AJ, editor. Campbell-Walsh Urology. 9 th ed. Philadelphia: Saunders, Elsevier; 2007. p 3745-6. |
|3.||Olbourne NA. Congenital urethral fistula: Case reports. Plast Reconstr Surg 1976;57:237-9. |
|4.||Kluth D, Hillen M, Lambrecht W. The principles of normal and abnormal hindgut development. J Pediatr Surg 1995;30:1143-7. |
|5.||Ritchey ML, Sinha A, Argueso L. Congenital fistula of the penile urethra. J Urol 1994;151:1061-25. |
|6.||Islam MK. Congenital penile urethrocutaneous fistula. Indian J Pediatr 2001;68:785-6. |
|7.||Betalli P, Carretto E, Midrio P, Zanon GF, Gamba PG. A new indication for buccal mucosal graft: Isolated congenital fistula of the penile urethra. Pediatr Surg Int 2003;19:586-7. |
|8.||Caldamone AA, Chen SC, Elder JS, Ritchey ML, Diamond DA, Koyle MA. Congenital anterior urethrocutaneous fistula. J Urol 1999;162:1430-2. |
|9.||Welch KJ. Hypospadias. In: Ravitch MM, Benson CD, Aberdeen E, Randolph JG, editors. Pediatric surgery. 3 rd ed. Chicago: Year book Medical Publishers; 1979. p. 1353-76. |
|10.||Tennenbaum SY & Palmer LS. Congenital urethrocutaneous fistulas. Urology 1994;43:98-9. |
[Figure 1], [Figure 2]