Title : Vicious disorganization deformities in connection with Gorham-Stout Syndrome
Abstract:
Gorham–Stout Disease (GSD, or the so called massive osteolysis, vanishing bone disease, and or phantom bone disease. GSD is characterised by abnormal proliferation of non-neoplastic vascular and lymphatic tissue (angiomatous proliferation) and at a later stage, by fibrous tissue resulting in a massive destruction and resorption of affected bones, which lead to skeletal deformities and functional impairment often associated with swelling. Etiological understanding is the corner stone in the management of skeletal deformities. Multi-centre study of patients with deformities in connection with diverse etiological backgrounds. We aimed to study ten patients with variable axial and appendicular deformities in connection with a vanishing bone disorder. Axial deformities such as scoliosis, kyphoscoliosis, compressed fused vertebrae, appendicular fractures, dislocations, and vicious disorganization deformities of the joints were in connection with the vanishing bone disorder, namely Gorham-Stout syndrome.
It is mandatory to establish proper clinical and radiological phenotypic characterization in children and adults presented with unusual skeletal deformities. Identifying the reason behind these deformities is the key factor to draw a comprehensive management plan. There was no evidence of a malignant, neuropathic, or infectious elements involved in the causation of this disorder in our patients. There are no known definitive methods to treat this disorder but various forms of antiangiogenic invasive agents and/or drugs are under study. In addition, various forms of chemotherapeutic agents are under trial as well. Radiotherapy has been used but often proved ineffective. The aim of this study is to alert physicians and orthopaedic surgeons, the necessity to distinguish fractures and or osteolysis in connection with vanishing bone disorder from other diagnoses such as osteogenesis imperfecta. Comprehensive clinical and the radiological phenotypic characterizations are the base line tools toward proper management.
