Department of Anesthesiology, Faculty of Medicine, University of Yamanashi, Chuo, Japan.
Department of Surgery 2, Faculty of Medicine, University of Yamanashi, Chuo, Japan.
Medicine (Baltimore). 2023 Jun 2;102(22):e33950. doi: 10.1097/MD.0000000000033950.
Gorham-Stout disease (GSD) is a rare disease that causes massive osteolysis and proliferation of abnormal lymphangiomatous tissues. Patients with GSD often experience pain associated with bone fractures and chylothorax. However, bleeding caused by abnormal lymphangiomatous tissue or hematological dysfunction rarely occurs.
A 22-year-old female patient with GSD presented with severe left hip and lower limb pain. The GSD had disappeared her right pelvic bone and femur, but no abnormalities were found in the bones at the site of the pain.
The patient presented with a chylothorax and cerebrospinal fluid leakage. She was treated with sirolimus and an epidural blood patch, and her symptoms resolved. Computed tomography and magnetic resonance imaging revealed an epidural hematoma extending from L3 to the caudal region, and blood results revealed a consumption coagulopathy.
We presumed that the hematoma caused pain and prescribed pregabalin and morphine. The pain gradually subsided.
An unexpected liver subcapsular hemorrhage occurred 4 months later, and the patient went into hemorrhagic shock. Transcatheter arterial embolization was promptly performed, and the patient recovered.
GSD infrequently causes bleeding related to abnormal lymphangiomatous tissues and coagulopathy, yet it can lead to serious events if it occurs.
Gorham-Stout 病(GSD)是一种罕见疾病,可导致大量骨质溶解和异常淋巴管瘤组织增生。GSD 患者常因骨骨折和乳糜胸而出现疼痛。然而,异常淋巴管瘤组织或血液功能障碍引起的出血很少发生。
一名 22 岁女性 GSD 患者出现严重左髋和下肢疼痛。GSD 已使她的右侧骨盆和股骨消失,但疼痛部位的骨骼无异常。
患者出现乳糜胸和脑脊液漏。她接受了西罗莫司和硬膜外血贴治疗,症状缓解。计算机断层扫描和磁共振成像显示硬膜外血肿从 L3 延伸至尾骨区域,血液检查结果显示消耗性凝血病。
我们推测血肿引起疼痛,开了普瑞巴林和吗啡。疼痛逐渐减轻。
4 个月后意外发生肝包膜下出血,患者发生出血性休克。立即进行了经导管动脉栓塞,患者恢复。
GSD 很少引起与异常淋巴管瘤组织和凝血病相关的出血,但如果发生,可能会导致严重事件。