Department of Pediatric and Adolescent Surgery, Schneider Children's Medical Center, Petach Tikva, affiliated to Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel.
Pediatric Surgery Department, Hospital Universitari Vall d'Hebron Barcelona, Spain.
J Pediatr Surg. 2019 Oct;54(10):1993-1997. doi: 10.1016/j.jpedsurg.2018.10.111. Epub 2018 Dec 29.
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.
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.
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 36.6 months (median 29 months). 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.
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.
Treatment study, level III.
肛门直肠畸形（ARM）患者中很少发生会阴血管瘤，但它们可能构成重大挑战，需要特别关注。后矢状位肛门直肠成形术（PSARP）的外科切开可能涉及到血管瘤部位，导致出血、供血损伤，从而导致并发症并对结果产生不利影响。本研究旨在回顾 ARM-Net 联盟在管理与 ARM 相关的会阴血管瘤方面的经验，并评估治疗策略。
通过问卷调查，回顾性收集了在参与 ARM-Net 中心接受治疗的所有 ARM 患者和位于 PSARP 解剖平面的会阴血管瘤患者的数据：ARM 类型、血管瘤分布和穿透、影像学发现、医疗/手术管理、确定性修复的时机、并发症和结果。
来自 8 个中心的 10 名患者被纳入研究。3 名患者各有直肠球部或直肠前庭瘘，2 名患者有直肠会阴瘘，1 名患者有直肠前列腺瘘，1 名患者因血管瘤太畸形而无法确定畸形类型。平均随访时间为 36.6 个月（中位数 29 个月）。8 例患者在确定性修复前行结肠造口术。5 例患者在 PSARP 前行全身β受体阻滞剂治疗：3 例患者在血管瘤部分/完全消退后手术顺利，2 例患者正在等待手术。2 例直肠会阴瘘患者采取期待治疗。其余 3 例患者未行术前药物治疗而行手术治疗，均出现并发症：3 例新肛门位置错位，1 例完全会阴裂开。
在存在会阴血管瘤的情况下尝试 PSARP 可能会导致并发症并对结果产生不利影响。本研究证实了在手术重建前使用β受体阻滞剂治疗的益处。
治疗研究，III 级。
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