Gorham's disease affecting the maxillofacial skeleton

Twenty-one cases of Gorham's disease in the maxillofacial region are described in the literature. This case is an additional one. The disease has a slight male predominance. All cases involved either the mandible alone or in association with the maxilla or other bones of the head and neck. The most common symptoms were pain, tooth mobility, mandibular pathological fractures, and facial deformity. The mean duration of symptoms prior to diagnosis was 6.4 years. Treatment included surgery, radiotherapy, and various medications, alone or combined. Four patients died of their disease. The diagnosis of the initial biopsy was incorrect in 45% of these cases. The current case appears to be the only one successfully treated by excision and autogenous bone graft reconstruction. In order to make an accurate diagnosis, the histologic features should be correlated with the clinical features.