关键词: Bockenheimer综合征, 　静脉畸形, 　真性弥漫性静脉扩张

Abstract: Objective　Bockenheimer disease is a rare and diffuse venous malformation. Seven typical cases of Bockenheimer disease were reported and the related literature was reviewed. Methods　The general information, coagulation tests, radiographic results and treatment of 7 patients with Bockenheimer disease from April 2013 to July 2021 were retrospectively analyzed. And the related literature was reviewed to summarize the diagnostic criteria and management of Bockenheimer disease. Results　Seven patients with Bockenheimer disease were enrolled into this study, including 3 males and 4 females, aged from 14 to 62 years. The lesions were almost first noted at birth or childhood with slow progression, and appeared as extensive phlebectasia of the limb. Symptoms included pain, swelling, loss of function and so on. Coagulation tests revealed localized intravascular coagulopathy (LIC). MRI, CTV and plain radiograph demonstrated diffuse vein malformation under the skin and soft tissue of the affected limbs, which can involve in muscles and bones. Thromboses and phleboliths were common. The treatments included sclerotherapy, intralesional copper wire retention, and surgery. Symptoms and signs were relieved after treatment. Conclusion　Bockenheimer disease has typical appearance, symptoms, and radiographic features. The lesion is progressive and diffuse which requires early intervention and comprehensive treatment.

Key words: Bockenheimer disease, 　Venous malformation, 　Genuine diffuse phlebectasia