Surgical management of gorham disease involving the upper cervical spine with occipito-cervical-thoracic fusion: a case report

• Published in: The spine journal Vol. 16; no. 7; pp. e467 - e472
• Main Authors: Schell, Adam, MD, Rhee, John M., MD, Allen, Abigail, MD, Andras, Lindsay, MD, Zhou, Feifei, MD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.07.2016
• Subjects: Adult; Female; Humans; Orthopedics; Osteolysis, Essential - diagnostic imaging; Osteolysis, Essential - surgery; Spinal Fusion - methods; Tomography, X-Ray Computed; gorham disease; occipito-cervicalthoracic fusion; cervical spine; osteolysis
• ISSN: 1529-9430; 1878-1632
• DOI: 10.1016/j.spinee.2016.02.020

Abstract Background context Gorham disease is a rare osteolytic condition with only 17 reported cases primarily affecting the cervical spine. Purpose We report the case of a 31-year-old woman with severe kyphotic instability and myelopathy of the upper cervical spine secondary to massive osteolysis of the posterior elements. The surgical management, clinical outcome, and review of the relevant literature are discussed. Study Design Case report and literature review Patient Sample One patient with Gorham disease of the cervical spine. Methods We report the case of a 31-year-old woman with Gorham disease affecting the upper cervical spine. Combined posterior and anterior stabilization and fusion was performed from the occiput to the thoracic spine. Results Six-year follow-up with annual CT imaging showed solid fusion from occiput to T2. No spread or local recurrence has developed to date. Conclusions Correction of severe instability with myelopathy due to Gorham disease of the upper cervical spine was achieved though posterior and anterior occipito-cervical-thoracic fusion.