Reipschläger Maria, Huebner Uwe, Seemann Joerg, Kutzner Heinz, Hoeger Peter H
Department of Paediatrics, Catholic Children's Hospital Wilhelmstift, Hamburg, Germany.
Department of Paediatric Surgery, Catholic Children's Hospital Wilhelmstift, Hamburg, Germany.
JAAD Case Rep. 2018 Apr 30;4(5):458-461. doi: 10.1016/j.jdcr.2018.01.017. eCollection 2018 Jun.
Bone involvement is relatively rare in vascular malformations. Gorham-Stout disease, also referred to as , is characterized by osteoclast activation and osteolysis caused by proliferating lymphatic endothelial cells. We present the case of a 12-year-old boy who had Gorham-Stout disease at the age of 8 years. The clinical course was complicated by pathological fractures and localized intravascular consumption coagulopathy. Sclerotherapy and embolization therapy led to normalization of the coagulation parameters and significant improvement of the clinical findings. We speculate that this effect may be attributable to the elimination of lymphatic endothelial cells.
骨受累在血管畸形中相对少见。戈谢病，也被称为，其特征是由增殖的淋巴管内皮细胞引起破骨细胞活化和骨质溶解。我们报告一例12岁男孩，其8岁时患戈谢病。临床过程因病理性骨折和局部血管内消耗性凝血病而复杂化。硬化疗法和栓塞疗法使凝血参数恢复正常，并使临床症状显著改善。我们推测这种效果可能归因于淋巴管内皮细胞的消除。
