title: Fatal Progression of Gorham Disease: A Case Report and Review of the Literature.
abstract: Gorham disease is an idiopathic massive osteolysis, and maxillofacial involvement is rare. This report describes a case of a 12-year-old boy with severe progressive osteolysis in the mandible, hyoid bone, mastoid process, and cervical spine. Radiation therapy and interferon-α therapy were followed by bisphosphonate therapy. The patient died of respiratory failure. To describe the clinicopathologic features of Gorham disease of the jaws with an emphasis on the fatal types, 64 cases in the literature were reviewed (female-to-male ratio, 1:1.78; average age, 33.02 ± 19.38 yr). Most lesions were located only in the mandible or in other locations in combination with the mandible, except for 3 cases. During follow-up, there were 7 cases of disease-specific death, resulting in a mortality rate of 10.94%. The main causes of death were chylothorax, rib fractures secondary to osteolysis, or spinal fractures. Although most patients received surgical treatment (43.75%), the type of treatment was not related to prognosis.
authors.affiliation: Associate Professor and Chairman, Department of Oral and Maxillofacial Surgery, College of Dentistry, Gangneung-Wonju National University, Gangneung, Korea.
authors.forename: Min-Keun
authors.initials: MK
authors.lastname: Kim
authors.affiliation: Professor, Department of Oral and Maxillofacial Surgery, Samsung Medical Center, Seoul, Korea.
authors.forename: Jong-Rak
authors.initials: JR
authors.lastname: Hong
authors.affiliation: Professor, Department of Oral and Maxillofacial Surgery, College of Dentistry, Gangneung-Wonju National University, Gangneung, Korea. Electronic address: kimsg@gwnu.ac.kr.
authors.forename: Seong-Gon
authors.initials: SG
authors.lastname: Kim
authors.affiliation: Professor, Department of Oral and Maxillofacial Pathology, College of Dentistry, Gangneung-Wonju National University, Gangneung, Korea.
authors.forename: Seok-Keun
authors.initials: SK
authors.lastname: Lee
mesh_terms: D002648:Child; D003131:Combined Modality Therapy; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D007571:Jaw Diseases / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D008297:Male; D010015:Osteolysis, Essential / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D014057:Tomography, X-Ray Computed