Surgical Management of Gorham-Stout Disease in Cervical Compression Fracture with Cervicothoracic Fusion: Case Report and Review of Literature

Abstract

BACKGROUND:

Gorham-Stout disease (GSD) or "vanishing bone" disease is characterized by progressive osteolysis with intraosseous lymphangiomatosis (hemangiomatosis). Given its rarity, with about 300 reported cases, its pathophysiology, etiology, and treatment guidelines are not established yet.

CASE DESCRIPTION:

A 22-year-old man was admitted to Severance Hospital with the chief complaint of neck pain from an injury due to falling. Initial cervical radiography showed a C4 burst fracture, and cervical magnetic resonance imaging revealed diffuse osteolytic lesions with coarse trabeculation with T2 hyperintensity and T1 enhancement in the entire cervical and upper thoracic area. He had a previous history of chylothorax that was still noticeable on a chest radiograph at the time of admission. A 2-stage operation was conducted. First, anterior corpectomy of C4 and anterior plate fixation of C3-5 were performed. Second, a week later, posterior fixation of C3-5 was performed. Thereafter, the patient was discharged without any neurologic complications. However, during the 1-month follow-up, asymptomatic progressive kyphosis was detected via radiography, and posterior cervical fusion of C2-T4 was performed. A minimal postoperative symptom of an intermittent left arm pain of 4-5 on the visual analog scale was experienced. No further deformity progression was noted until the last outpatient follow-up.

CONCLUSIONS:

Spinal GSD can cause severe deformity and neurologic deficits such as paralysis. Although treatment for GSD is not established, surgical treatment is recommended in severe deformity or aggravated neurologic deficit. The appropriate timing of surgery is after the arrest of osteolysis. Magnetic resonance imaging could be helpful in determining stable GSD.