Congenital anterior urethrocutaneous fistula

Purpose: Congenital anterior urethrocutaneous fistula is a rare anomaly tha t may present in an isolated fashion or in association with other penile ab normalities, such as chordee or hypospadias. There have been 18 cases of co ngenital anterior urethrocutaneous fistula reported in the literature. We p resent 14 additional cases of congenital anterior urethrocutaneous fistula. Materials and Methods: We treated 14 patients with congenital anterior uret hrocutaneous fistula, of whom 9 were uncircumcised at presentation. Two pat ients had evidence of chordee and 4 had distal hypospadias. Results: The type of repair was determined by the anatomical variations of this anomaly. All cases were corrected electively by various techniques bas ed on the degree of the defect, including primary closure via a Thiersch-Du play urethroplasty, pedicle flap urethroplasty, hinged flap urethroplasty a nd interpositioned island pedicle tube or onlay urethroplasty. Conclusions: To our knowledge the embryological events that cause anterior urethrocutaneous fistula are unclear but they likely result from a defectiv e urethral plate or an abnormality of the infolding of the urethral groove. Surgical technique must be individualized to fit the defect. While there h as been considerable skepticism regarding the existence of congenital ureth rocutaneous fistula, the fact that 9 of our 14 patients were uncircumcised confirms the congenital nature of this lesion.