Adam Schell1, John M Rhee2, Abigail Allen3, Lindsay Andras4, Feifei Zhou5. 1. University Hospitals of Cleveland, Case Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA. 2. Department of Orthopaedic Surgery, Emory Spine Center, Emory University, Atlanta, GA 30329, USA. Electronic address: john.rhee@emory.org. 3. Pediatric Orthopaedic Clinic, Leni & Peter May Department of Orthopaedics, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA. 4. Children's Orthopedic Center, Children's Hospital Los Angeles, Los Angeles, CA 90027, USA. 5. Department of Orthopaedic Surgery, Emory Spine Center, Emory University, Atlanta, GA 30329, USA; Orthopaedics Department, Peking University Third Hospital, Beijing 100191, China.
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: This is a case report and a literature review. PATIENT SAMPLE: This is a report of 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 computed tomography imaging showed solid fusion from the 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 through posterior and anterior occipito-cervical-thoracic fusion.
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: This is a case report and a literature review. PATIENT SAMPLE: This is a report of 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 computed tomography imaging showed solid fusion from the 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 through posterior and anterior occipito-cervical-thoracic fusion.