Anorectal malformations and perineal hemangiomas: The Arm-Net Consortium experience.

AIM: Perineal hemangiomas rarely occur in patients with anorectal malformations (ARMs), but they can pose a significant challenge and warrant special attention. Surgical incision of posterior sagittal anorectoplasty (PSARP) may involve the hemangioma site resulting in hemorrhage, damage to blood supply, leading to complications and adversely affecting outcome. The aim of this study was to review the experience of the ARM-Net Consortium in the management of perineal hemangioma associated with ARM and evaluate treatment strategies.

MATERIALS AND METHODS: Data on all patients with ARM and a perineal hemangioma located in the planes of the PSARP dissection who were managed at participating ARM-Net centers were collected retrospectively by questionnaire, as follows: ARM type, hemangioma distribution and penetration, imaging findings, medical/surgical management, timing of definitive repair, complications and outcome.

RESULTS: Ten patients from eight centers were included. Three patients each had a rectobulbar or rectovestibular fistula, 2 had a rectoperineal fistula, and one had a rectoprostatic fistula; in one patient, the hemangioma was too disfiguring to determine malformation type. Mean follow-up time was 36.6months (median 29months). Colostomies were performed before definitive repair in 8 patients. Five patients received systemic beta-blockers before PSARP: 3 were operated uneventfully following partial/complete involution of the hemangioma, and 2 are awaiting surgery. The two patients with rectoperineal fistula were managed expectantly. The remaining 3 patients underwent surgery with no preoperative medical treatment, and all had complications: mislocated neoanus in three and complete perineal dehiscence in one.

CONCLUSION: Attempting PSARP in the presence of a perineal hemangioma may lead to complications and adversely affect outcome. This study confirms the benefits of beta blocker treatment before surgical reconstruction.

LEVEL OF EVIDENCE: Treatment study, level III.