Fishman Steven J, Smithers C Jason, Folkman Judah, Lund Dennis P, Burrows Patricia E, Mulliken John B, Fox Victor L
Department of Surgery, Children's Hospital Boston and Harvard Medical School, Boston, MA 02115, USA.
Ann Surg. 2005 Mar;241(3):523-8. doi: 10.1097/01.sla.0000154689.85629.93.
We report the largest clinical experience to date of surgically treated patients with blue rubber bleb nevus syndrome (BRBNS).
BRBNS is a rare congenital disorder presenting with multifocal venous malformations of the skin, soft tissues, and gastrointestinal (GI) tract. Patients with BRBNS develop anemia from chronic GI bleeding, and require lifelong treatment with iron and blood transfusions. An aggressive surgical approach to treat the GI venous malformations of BRBNS has been considered unlikely to be successful because of the large number of lesions, their position throughout the GI tract, and the likelihood of recurrence. Based on our belief that eradicated lesions would not recur, we undertook the removal of all GI tract lesions in an effort to eliminate bleeding.
Ten patients with BRBNS were treated from 1993 to 2002. Lesions were identified using complete GI endoscopy. The multiple venous malformations were removed by a combination of wedge resection, polypectomy, suture-ligation, segmental bowel resection, and band ligation.
Patient ages ranged from 2 to 36 years, and patients received an average of 53 prior blood transfusions. A mean of 137 focal GI venous malformations per patient were resected at operation (range 4-557), with a mean operative duration of 14 hours (range 7-23 hours). Only 1 patient who had a less extensive procedure developed recurrent GI bleeding. The mean follow-up period was 5.0 years (range 2.9-10.3 years).
We believe that an aggressive excisional approach is indicated for the venous anomalies that cause GI bleeding in BRBNS.
我们报告了迄今为止手术治疗蓝橡皮疱痣综合征（BRBNS）患者的最大规模临床经验。
BRBNS是一种罕见的先天性疾病，表现为皮肤、软组织和胃肠道（GI）的多灶性静脉畸形。BRBNS患者因慢性胃肠道出血而出现贫血，需要终身接受铁剂和输血治疗。由于病变数量众多、遍布胃肠道以及复发的可能性，一种积极的手术方法来治疗BRBNS的胃肠道静脉畸形被认为不太可能成功。基于我们认为根除的病变不会复发的信念，我们进行了所有胃肠道病变的切除以消除出血。
1993年至2002年期间对10例BRBNS患者进行了治疗。通过完整的胃肠道内镜检查识别病变。采用楔形切除术、息肉切除术、缝合结扎术、节段性肠切除术和套扎术相结合的方法切除多个静脉畸形。
患者年龄从2岁到36岁不等，患者平均接受过53次输血。术中每位患者平均切除137个局灶性胃肠道静脉畸形（范围4 - 557个），平均手术时间为14小时（范围7 - 23小时）。只有1例手术范围较小的患者出现复发性胃肠道出血。平均随访期为5.0年（范围2.9 - 10.3年）。
我们认为对于导致BRBNS患者胃肠道出血的静脉畸形，应采取积极的切除方法。
