J Korean Soc Spine Surg.  2006 Mar;13(1):54-58. 10.4184/jkss.2006.13.1.54.

Gorham's Disease Misdiagnosed as a Simple Compression Fracture in the Thoracic Spine: A Case Report

• Lee JC
• Nam JS
• Soh JW
• Cho YI
• Kim YI
• Shin BJ

• KMID: 2040781
• DOI: http://doi.org/10.4184/jkss.2006.13.1.54

Abstract

Gorham's disease is a rare condition of unknown etiology that is characterized by progressive osteolysis. A 48 year-old woman had a burst fracture at T10, which was treated by pedicle screw instrumentation at another hospital. She was transferred due to progressive paraparesis, which was not observed initially. An MRI demonstrated severe cord compression at the T10 level. Under the assumption that the patient had a highly vascular metastatic tumor, an anterior decompression with instrumentation was performed. However, neurologic symptoms and bone destruction worsened after six weeks postoperatively. A repeat decompression was performed through the posterior route and long-level pedicle screw instrumentation was applied. After the second operation, Gorham's disease was confirmed histologically. Care must be taken not to overlook a pathologic fracture caused by a spinal tumor as a simple fracture, especially an osteoporotic one.

Keyword

Thoracic Spine; Gorham's Disease

MeSH Terms

Decompression
Female
Fractures, Compression*
Fractures, Spontaneous
Humans
Magnetic Resonance Imaging
Middle Aged
Neurologic Manifestations
Osteolysis
Paraparesis
Spine*

Figure

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  Fig. 1. Initail X-ray showing decreased T10 vertebral body height.

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  Fig. 2. Preoperative MRI. (A) Sagittal T2-weighted image showing heterogeneous high signal intensities in T10 (B) Sagittal T1-weighted image showing low signal intensities in T10 vertebral body with spinal cord compression.

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  Fig. 3. Histological findings (A) High power view of the tumor. The vascular channels are lined by flat normal appearing endothelial cells without atypia. (H-E× 100). (B) Immunohistochemical finding of the tumor. The lining cells of vascular channels are positive for CD34. (ABC× 100).

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  Fig. 4. At 1 year postoperatively, radiograph showing no recurrence of Gorham's disease.

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