Fishman S J, Shamberger R C, Fox V L, Burrows P E
Department of Surgery, Children's Hospital, Boston, Massachusetts 02115, USA.
J Pediatr Surg. 2000 Jun;35(6):982-4. doi: 10.1053/jpsu.2000.6947.
BACKGROUND/PURPOSE: Lower intestinal venous malformations are rare anomalies resulting from errors in vascular morphogenesis. These lesions may cause significant chronic and acute gastrointestinal hemorrhage. Venous malformations are unresponsive to angiogenesis inhibitors. Although these anomalies generally are incompletely resectable because of diffuse pelvic and mesenteric involvement, the authors sought to abate bleeding by excluding the lesion from the gastrointestinal lumen.
Three patients with circumferential transmural venous malformations of the colorectum, pelvis, and mesentery were identified. Imaging findings were similar among the patients and included circumferential septated bright signal on T2-weighted magnetic resonance imaging (MRI) contrast enhancement, and multiple phleboliths, seen best on computed tomography (CT). The lesion extended from the anus to the splenic flexure in 2 patients and throughout the entire colorectum in the other. Each had daily hematochezia for many years and required transfusions and chronic iron therapy. Although bleeding began in childhood in each patient, no therapy was successful until ages 7, 24, and 45. Colectomy, anorectal mucosectomy (through the pelvic venous malformation), and endorectal pull-through and anastomosis was performed (coloanal in 2 and ileoanal in 1).
Bleeding essentially has been eradicated in all 3 patients with 10- to 57-month follow-up. One patient received a 3-unit transfusion intraoperatively, and the other 2 received none. The most recent patient to undergo surgery, who has residual venous malformation in the remaining 1 cm of anal mucosa, has some mild difficulty with fecal control if her diet results in loose stool.
Colectomy with mucosectomy and endorectal pull-through should be considered for diffuse venous malformations of the colorectum before the development of large transfusion requirements.
背景/目的：低位肠道静脉畸形是血管形态发生错误导致的罕见异常。这些病变可能引起严重的慢性和急性胃肠道出血。静脉畸形对血管生成抑制剂无反应。尽管由于盆腔和肠系膜弥漫性受累，这些异常通常无法完全切除，但作者试图通过将病变与胃肠道管腔隔绝来减少出血。
确定了3例患有结直肠、盆腔和肠系膜环形透壁静脉畸形的患者。患者的影像学表现相似，包括在T2加权磁共振成像（MRI）上环形分隔的明亮信号、对比增强以及多个静脉石，在计算机断层扫描（CT）上显示最佳。2例患者的病变从肛门延伸至脾曲，另1例患者的病变累及整个结直肠。每位患者多年来每天都有便血，需要输血和长期铁剂治疗。虽然每位患者在儿童期就开始出血，但直到7岁、24岁和45岁时才成功进行治疗。进行了结肠切除术、肛门直肠黏膜切除术（通过盆腔静脉畸形）以及直肠内拖出术和吻合术（2例为结肠肛管吻合术，1例为回肠肛管吻合术）。
在10至57个月的随访中，所有3例患者的出血基本得到根除。1例患者术中接受了3单位输血，另外2例未输血。最近接受手术的患者，在剩余1 cm的肛门黏膜中有残余静脉畸形，如果饮食导致大便稀溏，在控制排便方面有一些轻度困难。
对于结直肠弥漫性静脉畸形，在出现大量输血需求之前，应考虑行结肠切除联合黏膜切除及直肠内拖出术。
