Deutsch J, Weissenbacher G, Widhalm K, Wolf G, Barsegar B
Klin Padiatr. 1976 Sep;188(5):464-71.
Up until now 39 cases of combined Klippel-Trénaunay syndrome and Sturge-Weber syndrome have been described. Here follows the report of a girl, now 4 years of age, displaying a full combination of these syndromes. Only a small part of the body surface is not covered with naevi teleangiectatici laterales. The patient has clear hypertrophy of the left cheek and of the left lower extremity, less noticeable on the left upper extremity. For therapeutic reasons the left side of the head and the left lower extremity were thoroughly angiographically examined--this revealed typical abnormalities. The vessel-alteration of the lower extremity are not extremely far developed and arteriovenous fistulas on a large scale are also absent. This allows us to dismiss the F.P. Weber syndrome on the one hand, while it explains the absence of complications of the Klippel-Trénaunay syndrome, as described in literature, on the other. The significance of the alterations of lymph nodes in this disease, which we are the first to describe, is at present not fully clear. The cerebral attacks have until now showed only a temporary response to medication.
截至目前,已有39例Klippel-Trénaunay综合征与Sturge-Weber综合征合并的病例被报道。以下是一名4岁女孩的病例报告,她表现出这两种综合征的完全合并症状。仅一小部分体表未被外侧毛细血管扩张痣覆盖。患者左侧脸颊和左侧下肢明显肥大,左上臂不太明显。出于治疗目的,对头部左侧和左下肢进行了全面的血管造影检查——结果显示出典型异常。下肢血管改变并未发展到极其严重的程度,也不存在大规模动静脉瘘。这一方面使我们可以排除F.P. Weber综合征,另一方面也解释了如文献中所述的Klippel-Trénaunay综合征并发症为何未出现。我们首次描述了该疾病中淋巴结改变的意义,目前尚不完全清楚。迄今为止,脑部发作仅对药物治疗有暂时反应。