Kohno Motonori, Aota Yoichi, Kawai Takuya, Murata Hidetoshi, Saito Tomoyuki
Department of Spine Surgery, Yokohama Stroke and Brain Center, Yokohama, Kanagawa.
Department of Neurosurgery, Yokohama City University Hospital, Yokohama, Kanagawa.
NMC Case Rep J. 2014 Dec 6;2(2):80-84. doi: 10.2176/nmccrj.2014-0141. eCollection 2015 Apr.
Gorham's disease is a rare disorder of unknown etiology and variable clinical presentation and is characterized by the proliferation of lymphatic vessels associated with massive regional osteolysis. Although 10 cases involving the skull and cervical spine have been reported in the literature, little is available concerning the surgical treatment of either atlantoaxial dislocation or basilar impression. Most cases have experienced universally unsuccessful treatment with bone grafts, which have led to dissolution. This case report describes the clinical course, and radiotherapeutic, medical, and surgical treatment for Gorham's disease with basilar impression and massive osteolysis of the skull and upper cervical spine. The case of a 27-year-old man with progressive massive osteolysis of the skull and cervical spine is reported. Multiple surgical treatments to decompress the spinal cord and stabilize the skull and upper cervical spine with autologous fibular grafts were performed in order to prevent the progression of atlantoaxial dislocation and basilar impression. Pathologically, radiotherapy failed to show any effect. The efficacy of antiresorptive therapy with bisphosphonates could not be confirmed either clinically or radiologically. Although solid bone fusion was not obtained, the patient has achieved a satisfactory functional outcome and remains completely active after repeated surgeries. Surgical treatment is extremely difficult in cases of Gorham's disease involving the skull and upper cervical spine. Fibular bone grafts seem to show resistance to erosion to osteolytic tissue.
戈勒姆病是一种病因不明、临床表现多样的罕见疾病，其特征是淋巴管增生并伴有大片区域性骨质溶解。尽管文献中已报道了10例累及颅骨和颈椎的病例，但关于寰枢椎脱位或基底凹陷的外科治疗的资料却很少。大多数病例采用骨移植治疗均未成功，导致骨质溶解。本病例报告描述了戈勒姆病合并基底凹陷及颅骨和上颈椎大片骨质溶解的临床病程、放射治疗、内科治疗和外科治疗情况。报告了1例27岁男性患者，其颅骨和颈椎进行性大片骨质溶解。为防止寰枢椎脱位和基底凹陷进展，多次进行手术以减压脊髓，并用自体腓骨移植稳定颅骨和上颈椎。病理检查显示放射治疗无效。双膦酸盐抗吸收治疗的疗效在临床和影像学上均未得到证实。尽管未实现坚固的骨融合，但患者在多次手术后获得了满意的功能结果，并且仍然完全活跃。对于累及颅骨和上颈椎的戈勒姆病病例，外科治疗极其困难。腓骨移植似乎对溶骨组织的侵蚀具有抵抗力。
