in contrast to acquired urethrocutaneous fistula , congenital anterior urethrocutaneous fistula is a rare anomaly that may present in an isolated fashion or in association with hypospadias or chordee . in the literature , it has been described as unusual type of hypospadias , cryptospadias and congenital urethrocutaneous fistula . there is considerable controversy regarding existence of this entity and some claim that it is a result of injury following circumcision . a 3-year - old boy presented with a congenital , mid - penile ventral opening with normal external meatus . there was no previous history of circumcision , trauma or urinary retention . on examination prepuce , distal urethra was patent , with a thinned out ventral skin bridge between the fistula and frenulum . a fistula was present in the mid - penile region measuring less than 0.5  0.5 cm in size . infant feeding tube of 6 fr was passed from the normal urinary meatus to bladder easily . the whole of the urethra from fistula to the corona was laid open [ figure 2 ] and formal urethral closure was performed by using local tissue flaps in two layers . the glanular urethra and meatus were normal and hence left untouched [ figure 3 ] . after a 5-year follow - up , congenital fistula that is threaded lay opened urethra distal to the congenital fistula with normal corona , glans and external urinary meatus final look after formal closure of the fistula a 5-year - old male child was referred to us with diagnosis of distal penile hypospadias . the parents of the child stated that he used to pass urine simultaneously from the glans tip and ventral openings since birth . examination of local site revealed a small opening on the ventral side of the penis slightly proximal to corona along with a deep pit at the external urethral meatus . the glans and prepuce were normal with good quality skin bridge between the fistula and frenulum . on calibrating the pit at the site of the normal meatus under sedation , the urethra distal to the fistulous opening was patent and hence the diagnosis of congenital urethrocutaneous fistula was made [ figure 4 ] . the child was able to pass urine with thin stream from the external meatus following calibration . two weeks post - cannulation , there was complete re - obliteration of the distal urethra . in view of recurrent obliteration of the distal urethra , entire urethra including the glans was laid open and snodgrass repair was performed [ figure 5 ] . the patient is doing well without any complication after a follow - up of 2 years . the patent distal urethra after calibrating the external urethral meatus with repaired left inguinal hernia post - operative view of case 2 a 3-year - old boy presented with a congenital , mid - penile ventral opening with normal external meatus . there was no previous history of circumcision , trauma or urinary retention . on examination prepuce , distal urethra was patent , with a thinned out ventral skin bridge between the fistula and frenulum . a fistula was present in the mid - penile region measuring less than 0.5  0.5 cm in size . infant feeding tube of 6 fr was passed from the normal urinary meatus to bladder easily . the whole of the urethra from fistula to the corona was laid open [ figure 2 ] and formal urethral closure was performed by using local tissue flaps in two layers . the glanular urethra and meatus were normal and hence left untouched [ figure 3 ] . after a 5-year follow - up , congenital fistula that is threaded lay opened urethra distal to the congenital fistula with normal corona , glans and external urinary meatus final look after formal closure of the fistula a 5-year - old male child was referred to us with diagnosis of distal penile hypospadias . the parents of the child stated that he used to pass urine simultaneously from the glans tip and ventral openings since birth . examination of local site revealed a small opening on the ventral side of the penis slightly proximal to corona along with a deep pit at the external urethral meatus . the glans and prepuce were normal with good quality skin bridge between the fistula and frenulum . on calibrating the pit at the site of the normal meatus under sedation , the urethra distal to the fistulous opening was patent and hence the diagnosis of congenital urethrocutaneous fistula was made [ figure 4 ] . the child was able to pass urine with thin stream from the external meatus following calibration . two weeks post - cannulation , there was complete re - obliteration of the distal urethra . in view of recurrent obliteration of the distal urethra , entire urethra including the glans was laid open and snodgrass repair was performed [ figure 5 ] . the patient is doing well without any complication after a follow - up of 2 years . the patent distal urethra after calibrating the external urethral meatus with repaired left inguinal hernia post - operative view of case 2 the aetiology of congenital urethra - cutaneous fistula until now is not well established ; perhaps it is multi - factorial because of varied appearance of congenital fistulas with or without associated hypospadias . campbell stated that congenital anterior urethro - cutaneous fistula represents embryonal urethral blow - out behind a distal congenital obstruction . olbourne theorized that a focal defect in the urethral plate results in arrested distal migration of the urethral plate or localized deficiency of a portion of the plate . similarly , the testosterone or androgen receptors may be at fault leading to the development of fistula . coronal type of fistula may be explained by misalignment of the glanular and penile urethra . cook and stephens suggested an alternative mechanism , namely pressure atrophy from the heel of the babies foot , leading to the pressure necrosis . these fistulas may also result from development of cysts along the mediogenital raphae at the frenulum during prenatal period . one is isolated fistula , which is associated with normal foreskin , no chordee or hypospadias and an intact distal urethra and spongiosum as in our cases . the other type is associated with hypospadia - like characteristics , that is , chordee , a dorsal hood with or without distal urethra or spongiosal defect . congenital anterior urethrocutaneous fistula is uncommon as compared with posterior urethrocutaneous fistula , which usually represents y - type duplication of the urethra with anorectal atretic malformations . it is important to exclude urethral duplication , which produces y type of fistula and is associated with anorectal malformations . probing the fistula , radiographic dye study or cystourethroscopic examination may be required to corroborate the diagnosis . in case of an isolated fistula with intact spongiosum , repair with local flaps is sufficient , but if they are associated with deficient distal urethra or spongiosum , associated chordee , hypospadias or stenosis of external meatus , then formal hypospadias repair is recommended . various techniques are proposed to repair these fistulas including primary closure via thiersch - duplay urethroplasty , turned - down flap urethroplasty and pedicled island tube or onlay urethroplasty . in this article , we have highlighted a rare variant of hypospadias , which is often confused with coronal or distal penile hypospadias . treatment of this entity is individualized according to the site of fistula and associated anomalies , as well as the condition of the distal urethra .
we are presenting two cases of congenital urethrocutaneous fistula on ventral penile shaft . 
 congenital urethral fistula is an extremely rare , but easily manageable anomaly that may be confused with hypospadias . 
 awareness of the entity will avoid complications . 
 this condition may be associated with other anomalies like congenital hernias and anorectal malformations . 
 treatment of this entity is individualized according to site of fistula , associated anomalies and condition of the distal urethra . 
 all the principles of hypospadias surgery should be strictly followed .