Boyle Matthew J, Alison Peter, Taylor Graeme, Lightbourne Bernadette A
Department of Cardiothoracic Surgery, Auckland City Hospital, Auckland, New Zealand.
Heart Lung Circ. 2008 Feb;17(1):64-6. doi: 10.1016/j.hlc.2007.01.009. Epub 2007 Sep 5.
Gorham's disease is a very rare disorder of uncertain aetiology, characterised by non-neoplastic proliferation of vascular or lymphatic channels within bone leading to resorption and replacement of osseous matrix with angiomatous tissue. Fewer than 200 cases have been reported in the literature. The complication of chylothorax is associated with a high morbidity and mortality. We present the case of a 17-year-old male with Gorham's disease of the sternum, clavicle and rib, complicated by bilateral chylothorax. Despite thoracic duct ligation and radiotherapy, the patient progressively deteriorated and died 36 weeks following initial presentation.
戈勒姆病是一种病因不明的极为罕见的疾病，其特征是骨内血管或淋巴管的非肿瘤性增生，导致骨基质被血管瘤组织吸收和替代。文献报道的病例不足200例。乳糜胸并发症的发病率和死亡率很高。我们报告一例17岁男性患者，患有胸骨、锁骨和肋骨的戈勒姆病，并伴有双侧乳糜胸。尽管进行了胸导管结扎和放疗，但患者病情逐渐恶化，在首次就诊后36周死亡。
