Uhlig H H, Stephan S, Deutscher J, Kiess W, Richter T
Universitätsklinik und Poliklinik für Kinder und Jugendliche Universität Leipzig.
Klin Padiatr. 2004 Jan-Feb;216(1):41-4. doi: 10.1055/s-2004-817685.
Whereas in adults angiodysplasia is a frequent cause of gastrointestinal bleeding, in children this disorder is extremely rare. A 7 10/12 year old girl is presented suffering over 3-4 months from mild but recurrent rectal bleeding. Blood count and serum ferritin and transferrin levels were normal. The rectosigmoideoscopy revealed a rectal lesion, which was confirmed histologically as angiodysplasia. Pathological investigation of the biopsies included HE staining and immunohistological staining of endothelial cells with anti-CD34 and anti-von Willebrand factor. A follow-up period of three years revealed spontaneous regression of the angiodysplastic lesion at the rectosigmoideal localisation, which could be confirmed by endoscopy.
The outcome of the few pediatric patients described in the literature was reviewed. Due to the lack of conclusive understanding of the nature of this extremely rare vascular disorder and the variable outcome described, a wait and see attitude should be assumed in cases of less clinical affection.
血管发育异常在成人中是胃肠道出血的常见原因,而在儿童中这种疾病极为罕见。本文报告一名7又10/12岁女孩,在3 - 4个月内反复出现轻度直肠出血。血常规、血清铁蛋白和转铁蛋白水平均正常。直肠乙状结肠镜检查发现直肠病变,经组织学证实为血管发育异常。活检的病理检查包括苏木精-伊红(HE)染色以及用抗CD34和抗血管性血友病因子对内皮细胞进行免疫组织化学染色。三年的随访显示,直肠乙状结肠部位的血管发育异常病变自发消退,这一点可通过内镜检查得到证实。
回顾了文献中描述的少数儿科患者的治疗结果。由于对这种极为罕见的血管疾病的性质缺乏确凿的认识,且所描述的治疗结果存在差异,对于临床症状较轻的病例,应采取观察等待的态度。