Surgical Treatment of Gorham’s Disease with Massive Osteolysis of the Skull and Cervical Spine: A Case Report and Review of Literature

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.