Calligaro K D, Sedlacek T V, Savarese R P, Carneval P, DeLaurentis D A
Section of Vascular Surgery, Pennsylvania Hospital/University of Pennsylvania School of Medicine, Philadelphia.
J Vasc Surg. 1992 Jul;16(1):100-8.
Congenital arteriovenous malformations confined to the soft tissues of the pelvis are rare and challenging lesions. Multitudinous embryonic macrocommunication and microcommunication between the arterial and venous systems, with resultant shunting of blood to the low-resistance veins, produce massive venous and tissue engorgement. No well-established guidelines exist concerning their management. Percutaneous arterial embolization and surgery are associated with high recurrence rates. Surgical excision is frequently not possible and can result in massive hemorrhage. Only 52 cases have been reported in the English-language literature, and only two patients were followed for longer than 6 years. This report presents two additional patients with massive pelvic arteriovenous malformations monitored for 9 and 17 years. One patient was initially treated with arterial percutaneous embolization and the other with attempted operative ablation. A review of the literature has allowed us to develop the following management guidelines: Asymptomatic, nonenlarging lesions require no intervention and can be safely followed by clinical and radiologic (ultrasonography, CT scanning) examinations every 6 months. Symptomatic or rapidly enlarging lesions should be treated with preoperative arterial embolization and surgical excision attempted only if the arteriovenous malformation is localized and does not involve adjacent organs. Arterial embolizations may be repeated to provide symptomatic relief of diffuse, large lesions that involve the bladder or bowel. This protocol will avoid unnecessary and frequently life-threatening surgery in the management of pelvic arteriovenous malformations.
局限于骨盆软组织的先天性动静脉畸形是罕见且具有挑战性的病变。动脉和静脉系统之间存在大量胚胎期的大交通和微交通，导致血液分流至低阻力静脉，从而引起大量静脉和组织充血。目前尚无关于其治疗的成熟指南。经皮动脉栓塞和手术的复发率都很高。手术切除往往无法进行，且可能导致大出血。英文文献中仅报道了52例，仅有2例患者随访时间超过6年。本报告介绍另外2例患有巨大骨盆动静脉畸形的患者，随访时间分别为9年和17年。1例患者最初接受经皮动脉栓塞治疗，另1例尝试手术切除。文献回顾使我们制定了以下治疗指南：无症状且无增大的病变无需干预，每6个月进行临床和影像学（超声、CT扫描）检查即可安全随访。有症状或迅速增大的病变应先行术前动脉栓塞治疗，仅当动静脉畸形局限且不涉及相邻器官时才尝试手术切除。对于累及膀胱或肠道的弥漫性大病变，可重复进行动脉栓塞以缓解症状。该方案将避免在骨盆动静脉畸形治疗中进行不必要且常危及生命的手术。
