Congenital anterior urethrocutaneous fistula: A case report and review of literature.

Congenital anterior urethrocutaneous fistula is an extremely rare anomaly which may present in an isolated form or accompanying hypospadias or chordee. We describe an 8-month-old boy who presented to our outpatient clinic with a congenital anterior urethrocutaneous fistula on the ventral aspect of the penile shaft. He underwent single stage tubularized incised plateurethroplasty with a dartos flap cover. Postoperatively, the patient recovered without any complications and is doing fine on the follow-up visits.

Introduction

While most of the causes of urethrocutaneous fistulae are complications of hypospadias repair surgery, congenital anterior urethrocutaneous fistula (CAUF), in the isolated form or with anorectal malformations, hypospadias and other genito-urinary anomalies, is an extremely rare anomaly that had been reported in few cases in the literature. It was first described by Gupta in 1962 and the largest series was conducted by Caldamone et al. on 14 cases. We describe a case of CAUF, with emphasis on its etiology as well as reviewing the literature.

Case presentation

An 8-month-old uncircumcised boy attended to our outpatient clinic complaining of passing urine through an opening located on the ventral side of his penis since birth, as stated by his parents. He had neither history of trauma nor surgical intervention. On enquiry about family history, there was no history of such condition or any other genito-urinary tract malformations. The systemic examination of the patient was unremarkable. On local examination, a clearly apparent fistula was seen on the ventral side of the shaft of the penis, just proximal to the coronal sulcus, measuring 4 × 2 mm (Fig. 1). Distal to that fistula, the glans is intact with normal wide urethral meatus at its tip; however, the prepuce on the ventral aspect was deficient. The rest of the local examination revealed absence of chordee, normal scrotum harboring both testicles, and normal anus. Crede maneuver demonstrated that most of the urinary stream was passing through the fistula, while the minority passed from the external urethral meatus. On catheterization an 8 Fr Nelaton catheter passed proximally with ease from the tip of the glans to thebladder (Fig. 2). Ultrasound abdomen and cystourethrogram revealed no other genito-urinary anomalies. The boy was prepared for an elective operation. At operation, a formal tabularized incised urethroplasty (Snodgrass urethroplasty) was done. Closure of the fistula was done in double layer, continuous subepithelial manner using 6/0 Polydioxanone (PDS) sutures with a protective ventral dartos flap (Fig. 3). The patient was discharged on the same day, while the catheter was removed 7 days later. The patient is behaving well after 6 months of follow up visits.

Fig. 1

Congenital anterior urethrocutaneous fistula with deficient ventral prepuce.

Fig. 2

An 8 Fr Nelaton catheter passing easily from the normal meatus and the tube is obviously seen at the fistula site.

Fig. 3

An intraoperative photo after completing the repair and the ventral dartos coverage.

Discussion

Primary anterior CAUF is an extremely rare anomaly. To date, only 50 cases had been reported in literature, where half of which were in the distal shaft, 15 at the mid-shaft, 3 at the penoscrotal junction, and at unspecified location in 7 cases. It occurs more commonly in an isolated form (29/50; 58%), with a normal urethra, meatus and foreskin, or associated with anorectal malformations, hypospadias and other genito-urinary anomalies.

Clinically, children present when their parents are concerned by frequent dribbling through the fistula despite a normal urethral meatus is present. In this condition, history is crucial as previous trauma, circumcision for example, or surgical procedures can give the clue about the cause of the fistula. Adjunct imaging such as ultrasound abdomen & cystourethrogram are necessary to evaluate for other genito-urinary anomalies. The latter can show the presence of double urinary flows, one from the tip and the other from the fistula.

Many methods of surgical repair have been described by different authors. This includes local skin flap, preputial skin flap, Thiersch-Duplay urethroplasty, Denis Brown urethroplasty, buccal mucosal graft, and tubularized incised plate urethroplasty with dartos flap and primary closure. The choice of the method of repair should be individualized according to many variables, eg. the size and the location of the fistula as well as existing other abnormalities, such as hyposapadias or chordee and the condition of the distal urethra. If the fistula was an isolated abnormality with intact urethra beyond it, primary closure is indicated with success. However, if defective, then this method is abandoned and the defect should be opened to be converted and managed as a hypospadias.

Conclusion

CAUF is a rare anomaly with a success rate of almost 90% after single surgical procedure. Nevertheless, associated anomalies should be excluded before planning for surgical correction.

Conflicts of interest

The authors declare that there are no conflicts of interest regarding the publication of this article.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.