Authors' reply.

Sir,

We appreciate Tripathy et al.1 for the interest shown in our paper2 and thank them for sharing their experience on the long term followup of Gorham's disease managed with radiotherapy and bone grafting.

Gorham's disease is a rare disorder of the musculoskeletal system. The etiology and pathophysiology of the disease remains undetermined. Being uncommon with an unpredictable course, the management of the disease has not been standardized. A number of treatment methods including bisphosphonates, estrogens, magnesium, calcium, vitamin D, vitamin B12, fluoride, calcitonin, cisplatin, actinomycin D, thalidomide, somatotrophin, interferons, amino acids, placental extracts, and transfusions of placental blood have been used, but none has been found to be effective in majority of cases. Surgery and radiation therapy remain the principal treatment modalities of the condition.

Surgical treatment usually has involved prosthetic replacement of the affected bone (especially in proximal femur) or amputation in severe cases, with most of the studies showing good long term results.3 Bone grafting has been tried in a few cases, but it has usually yielded poor results as the graft is usually found to resorb, generally within the first year, or after a number of years, as stated by Tripathy et al.

Radiotherapy has shown promise in the treatment of Gorham's disease and halting the disease process, probably due to the radiosensitive nature of the proliferating capillary-like or lymphatic-like vessels which play a role in the pathogenesis of the disease. Most of the studies showing lack of effect of radiotherapy have used low doses, generally less than 20 Gy. Definitive radiation therapy in moderate doses (40–45 Gy) appears to result in a good outcome and few long term complications.4 Radiotherapy appears to be the best bet in managing this condition. This view is supported by a recent literature review wherein radiotherapy was seen to prevent the disease progression in 77.3% patients of Gorham's disease.5

Since the course of Gorham's disease is unpredictable, we agree with Tripathy et al. that the patient needs to be kept under followup for a longer period.