摘要
目的总结现有蓝色橡皮疱痣综合征病例的临床资料,提高临床医生对本病的认识。方法检索pubmed上所有蓝色橡皮疱痣综合征病例(1961年至2018年7月),进行回顾分析。结果共得到221篇文献,含250例患者。皮肤病变见于94.8%的患者,消化道血管瘤见于77.2%的患者。69.2%的患者有消化道出血,11.4%的患者发生肠梗阻或肠套叠。45.1%的患者行皮肤病变的治疗,术后复发率为85.2%。29.7%的患者因消化道血管瘤行外科手术,并发症发生率及术后复发率分别为10.8%和28.4%。另有26.5%的患者行内镜治疗,其并发症发生率和术后复发率分别为7.6%和21.2%。抗血管生成药物除西罗莫司外,绝大多数不能有效的控制症状。5例(2.0%)患者因疾病并发症死亡,部分患者生活质量收到严重影响。结论蓝色橡皮疱痣综合征目前的治疗效果不尽如人意,值得关注和研究。
Objective To improve the clinical understanding of the rare disease.Methods We reviewed all previously reported cases on pubmed by using‘Blue rubber bleb nevus syndrome’or‘Bean syndrome’as the search term.Information on clinical features,diagnosis,treatment and prognosis of BRBNS was analyzed retrospectively.Results The clinical data of 250 patients was analyzed.Cutaneous angiomas were observed in 94.8%of patients.Gastrointestinal hemangiomas were presented in77.2%of patients.There were 69.2%of patients had gastrointestinal bleeding including 56.4%of patients with obvious bleeding and 12.8%of patients with occult blood losing.Gastrointestinal angiomas resulted in intestinal obstruction or intussusception in11.4%of patients.There were 107 cases(45.1%)received treatment for the cutaneous lesions due to cosmetic or functional impairment.However,cutaneous lesions had a very high recurrence rate(85.2%)after ablated.There were 29.7%of patients experienced gastrointestinal tract surgery.The recurrence rate of lesions was 28.4%and 10.8%of patients developed complications during or after surgical treatment.Sixty-six(26.5%)patients underwent endoscopic therapy in whom 21.2%of patients had lesions recurred and 7.6%of patients developed postoperative complications.Five(2.0%)cases died of complications of the syndrome.Otherwise,some patients had a poor quality of life for the impaired appearance,the recurrent gastrointestinal bleeding,and repeated treatment for cutaneous and gastrointestinal lesions.Conclusion The treatment effect of Blue rubber bleb nevus syndrome is not satisfactory and still need more investigation in the future.
作者
潘燕
李良平
李汉华
Pan Yan;Li Liangping;Li Hanhua(Department of Gastroenterology and Hepatology,Sichuan Provincial People′s Hospital,School of Medicine,University of Electronic Science and Technology of China,Chengdu,Sichuan 610072,China)
出处
《四川医学》
CAS
2019年第11期1134-1137,共4页
Sichuan Medical Journal
关键词
血管瘤
蓝色橡皮疱痣综合征
消化道出血
血管畸形
blue rubber bleb nevus syndrome
gastrointestinal bleeding
hemangioma
vascular malformations
