High and intermediate anorectal malformation in males with perineal fistula: Beware of urethral involvement

Abstract

Anorectal malformation (ARM) with recto-perineal fistula is considered low-type anomaly and standard management of perineal anoplasty without colostomy is recommended. Unusual variants are scarcely reported and present unique challenges in management. We present two cases of ARM with unusual fistula managed in our hospital. Case 1 A term baby boy status-post colostomy creation at birth in an overseas hospital for ARM presented to us at 4 months of age for definitive surgery. Distal loopogram demonstrated a very fine recto-penile fistulous opening at the peno-scrotal area hence perineal anorectoplasty was planned. Intra-operatively, the bulbar urethra was laid open for complete fistula tract excision due to a 3cm shared common wall between fistula tract and urethra. A double-layered urethral repair was performed and a neoanus created after mobilisation of a high rectal pouch. The baby recovered well post-operatively after 3 weeks of urethral stenting and is currently awaiting stoma closure. Case 2 A term male newborn was noted to have ARM with a recto-scrotal fistula. After initial stabilisation and workup, he was scheduled for perineal anoplasty on day 2 of life. Intra-operatively, we noted a fine, long cutaneous track running in the scrotal raphe close to the bulbar urethra. The bulbar urethra was inadvertently entered during fistula tract excision and needed repair. The rectal pouch located above the levator muscle was mobilised and anorectoplasty was performed. The baby was managed post-operatively with gut rest and total parenteral nutrition, and recovered well. Rare-variant ARM with recto-penile/recto-scrotal fistulae can be associated with high or intermediate type anomalies in contrary to classical recto-perineal fistula. Management of these cases should be planned carefully, with possible need for urethral repair during anorectoplasty.