Congenital anterior urethrocutaneous fistula

Congenital anterior urethrocutaneous fistula (CAUF) is an uncommon penile malformation characterized by the urethra's connection to the skin on the ventral aspect of the penis. It can occur alone or with genitourinary or anorectal anomalies. Due to the scarcity of reported cases, treatment strategies vary, prompting a systematic review to evaluate their clinical presentation, surgical outcomes, and recurrence rates. A systematic review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, searching PubMed, ScienceDirect, EBSCO, Cochrane, and Google Scholar for studies published up to December 12, 2024. Eligible studies focused on CAUF, its variants, surgical interventions, complications, and recurrence rates. Data were extracted and analyzed, including fistula location, associated anomalies, surgical techniques, and patient outcomes. Seventy-eight patients from 46 studies were reviewed. The most common fistula sites were subcoronal (42.3%) and mid-penile (39.7%), followed by penoscrotal (8.9%), proximal penile to subcoronal (5.1%), and scrotal/perineal (3.8%). Chordee occurred in 11 cases, while 19.2% had associated genitourinary anomalies and 16.7% systemic anomalies, mainly anorectal malformations. Surgical repair, especially with a ventral dartos flap, showed high success. Recurrence was 8.9%, with 5.4% closing spontaneously. CAUF is a rare anomaly with variable presentations and frequent associations with other anomalies. Surgical repair generally achieves good outcomes with low recurrence, though spontaneous resolution is possible in select cases. Larger multicenter studies are needed to standardize management and clarify genetic and embryologic factors.