THE CHILD’S HAND IN OLLIER DISEASE

Abstract

Purpose: Hand localisations predominate in Ollier disease. There have however been few studies devoted to this topic and the one report available only presented a few cases. Treatment of multiple enchondromatosis is sometimes discussed with the treatment of solitary enchondromas despite different recurrence behaviour and aggressiveness. A specific approach is indicated.

Material and methods: We present a retrospective series of 22 children treated for multiple enchondromatosis of the hand. One hand was involved in 11 and both hands in 11. We thus identified 246 enchondromas on 33 hands. One girl with Maffucci syndrome died at the age of six years due to angiosarcomatous degeneration of the leg.

Results: At diagnosis, mean age was six years nine months. Pain and pathological fractures were exceptional, observed in three patients. Overall hand function remained satisfactory. We performed surgery for 14 patients (mean age 8 yr 8 mo) due to increasing symptoms and tumour volume. Five patients required two operations and one a third. We thus performed 18 interventions on 37 hand bones (51 surgical procedures). Simple curettage was performed in 21 cases, combined with autologous cancellous grafting in 30 others. One finger had to be amputated due to aggressive recurrence. Radio-lucent residual lesions were found in 62% of the children at mean 19 months follow-up.

Discussion: Hand function remained satisfactory despite a worrisome radiographic aspect. The decision for surgery was basically made to improve the aesthetic aspect of the hand. Many surgical techniques have been proposed. In our experience, it is difficult to eradicate a treated lesion but grafting appears to be more effective than simple curettage.

Conclusion: Function remains good during growth, fractures are exceptional. Lesions stabilise at the end of growth. We have not observed any case of chondrosarcomatous degeneration in our series of paediatric patients. There is however a risk of malignant degeneration which should be suspected in the event of pain, increased tumour volume or pathological fracture.