Gorham-Stout综合征伴乳糜腹一例

A Case Report of Gorham-Stout Syndrome with Chyloperitoneum

1.
Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
2.
Department of Orthopaedics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
3.
Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China

摘要: Gorham-Stout综合征是一种以骨质溶解为特征性改变的罕见疾病，部分可伴发乳糜胸。本文报道一例因腹腔积液就诊于北京协和医院消化内科的Gorham-Stout综合征患者，通过淋巴管显像证实其乳糜腹的产生途径，以提高临床医生对该疾病的认识。
- Gorham-Stout综合征 /
- 乳糜胸 /
- 乳糜腹
Abstract: Gorham-Stout syndrome is a rare disease characterized by osteolysis, in which idiopathic intraosseous angiomatous proliferation leads to progressive resorption of bone. Chylothorax can be associated with Gorham-Stout syndrome in some cases, but chyloperitoneum is seldom seen. This paper reports a case of Gorham-stout syndrome that was admitted to the gastroenterology department because of ascites. The way of chyloperitoneum formation was confirmed by lymphangiography.
- Gorham-Stout syndrome /
- chylothorax /
- chyloperitoneum
注释:

作者贡献：王强、吴晰负责收集临床资料及数据，起草并撰写文章；周熹负责协助完成患者手术，采集相关数据；霍力负责协助完成下肢淋巴管显像，采集并分析相关数据。

利益冲突：无

图 1 腹盆影像学改变

A.CT示骶骨骨质破坏；B.CT示腹腔积液、脾囊肿；C.MRI示腰椎及骶骨多发骨质破坏、囊性改变(箭头)

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图 2 双下肢淋巴管显像

从左至右依次为注射⁹⁹锝标记的示踪剂20 min、1 h、3.5 h、22 h后, 双下肢淋巴管单光子发射计算机断层成像结果

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图 3 淡血性乳靡样腰椎穿刺液

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