METHODS: 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.
METHODS: 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.
METHODS: We presumed that the hematoma caused pain and prescribed pregabalin and morphine. The pain gradually subsided.
RESULTS: 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.
CONCLUSIONS: GSD infrequently causes bleeding related to abnormal lymphangiomatous tissues and coagulopathy, yet it can lead to serious events if it occurs.
方法:一名22岁女性GSD患者表现为严重的左髋和下肢疼痛。GSD消失了她的右骨盆骨和股骨,但是在疼痛部位的骨骼中没有发现异常。
方法: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,她的症状得到了缓解.计算机断层扫描和磁共振成像显示硬膜外血肿从L3延伸到尾部,和血液结果显示消耗凝血病。
方法:我们推测血肿引起疼痛,并服用普瑞巴林和吗啡。疼痛逐渐消退。
结果:4个月后出现意外的肝包膜下出血,病人进入失血性休克.及时行导管动脉栓塞术,病人康复了.
结论:GSD很少引起与异常淋巴管瘤组织和凝血病相关的出血,然而,如果发生,它可能会导致严重的事件。