Gorham-Stout Disease. Case report and narrative literature review
Authors
Francesca Romana Federici, Giovanni Barbini, Gianluca Pingitore, Gabriele Miccoli, Massimo Galli
Abstract
Gorham’s disease, also known as vanishing bone disease, is a rare condition of unknown etiology characterized by progressive destruction and bone resorption. The disease can affect any part of the skeleton, but it is more frequently found in the head and neck, upper extremities, pelvis, humerus, and the axial skeleton. The mechanism of bone resorption is unclear; however, osteolytic lesions exhibit localized endothelial proliferation of lymphatic vessels. The diagnosis is based on clinical, radiological, and histological features after excluding other infectious, inflammatory, endocrinologic, and neoplastic etiologies. The medical treatment for Gorham’s disease includes anti-osteoclastic medications (bisphosphonates), alpha-2b interferon, sirolimus, and propranolol. Radiation therapy acts by inducing sclerosis of proliferating vascular tissue within the bone. The surgical treatment options include resection of the lesion and reconstruction using bone grafts and/or prostheses. In this paper, we present a case of Gorham’s disease affecting the right maxilla, alveolar process, zygoma, and floor of the orbit in a 67-year-old female. At the onset of the disease, the clinical manifestation was mobility of the upper right molars, mimicking a periodontal disease, followed, after some weeks, by increased diplopia already present. The patient received medical treatment with Zoledronic acid, vitamin D, and calcium carbonate, which proved effective in controlling the disease’s progression for 12 months.
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