Gorha-Stout Disease of the upper jaw: case report and review of the literature

Abstract

Gorham’s disease, or vanishing bone disease, is a rare condition of unknown etiology characterized by bone absorption. The disease can affect any part of the skeleton, however the pelvis, humerus, axial skeleton and the mandible are more frequently involved. The mechanism of bone resorption is unclear, but localized endothelial proliferation of lymphatic vessels is shown in osteolytic lesions The diagnosis is based on clinical, radiological and histological features after excluding other infectious, inflammatory, endocrine and neoplastic etiological factors. Medical treatment for Gorham’s disease includes anti-osteoclastic medications (bisphosphonates), and alpha-2b interferon, radiation therapy induced sclerosis of the proliferating vascular tissue within the bone. Also surgical treatment options are available including resection of the lesion and reconstruction with bone grafts and/or prostheses. We present a case of Gorham’s disease of the right maxilla in a 67 years old female affecting the alveolar process, zygoma and the floor of the orbit. The initial clinical manifestation at the onset of the disease was the mobility of the upper right molars, mimicking periodontal disease followed by the worsening of a preexisting diplopia with undefined origin. The patient received a medical treatment with zoledronic acid, vitamin D and calcium carbonate for 12 months which proved to be effective in controlling the progression of the disease.

Keywords

• vanishing bone disease
• bone resorbtions
• endothelial proliferation
• zoledronic acid therapy