Management of a
Fracture in a Known Case of Gorham's Vanishing Bone Disease
Contact Person: Myles Clough (email@example.com)
Gorham's Disease is an extremely rare condition of bone with less than 200 cases in the literature since the condition was originally reported. Although the disease is quite well described, it does not occur often enough to be recognised readily. It often takes many years for the condition to be recognized and there is no agreement about the most suitable treatment. Because of this rarity it is most unusual for a case of Gorhams disease to be known from the beginning of an incident and for ancillary treatment to be commenced at once. Yet because of the nature of the disease, repeat fracture might be expected to be common.
In most instances, including the index presentation of our patient, the condition is not recognised until a fracture occurs and bone healing is delayed or subverted. The pathological process is the replacement of normal bone by an aggressively expanding but non-neoplastic vascular tissue 62 90 113 134 172 183 190 , similar to a haemangioma or lymphangioma 28 51. It is unknown what stimulus sets off this change in bone 172 and also whether all bones of the affected individual are equally likely to react in this adverse fashion. Since it is most commonly seen in trauma it is reasonable to deduce that the traumatic episode initiates the sequence of events. Since neovasularisation of the blood clot that forms in between fragments of a fracture is the first step in bone healing it is tempting to speculate that some error in this process, or in the control of this process, is the root cause of Gorham's Disease. Wildly proliferating neovascular tissue appears to be the agent of the massive bone loss in this condition, aptly summed up in the title of one paper 62 "Posttraumatic oseolysing haemangiomatosis".
In our case the patient presented with a relatively straightforward fracture but the history of Gorham's Disease having been initiated and treated several years previously. The previous episode had affected the distal radius and ulna, whereas the current episode involved the proximal ulna on the same side. The patient was sophisticated about his condition and was able to state the name and something of the nature of the problem. He was naturally concerned that he would have severe problems with healing in the current instance and looked to the treating team to head off this eventuality in some way.
Apart from standard orthopaedic treatment for fractures, non unions and deformities the medical treatment for Gorhams Disease falls into three groups, radiotherapy 61 80 84 95 114 137 191, anti-osteoclastic medication (biphosphonates) 191 192 and specific treatment for haemangiomata 175 192. A rapid review of the literature and an appeal 198 on the "Orthopod" mailing list on the Internet led to the conclusion that there was no consensus about the most efficacious treatment. We therefore undertook four unrelated forms of treatment, immediate reduction and internal fixation of the fracture, electrical stimulation 202 to speed up the normal process of bone healing, biphosphonate infusion to slow osteoclastic mediated osteolysis 201 and radiation to slow the proliferation of haemangiomatous tissue. This "blunderbuss" approach makes it difficult to say with certainty which element was critical. However, the apparant success more than makes up for this lack of elegance, at least in the eyes of the patient.
© 1998 Author(s) Hold Copyright