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Genu Valgum and Limb Length Discrepancy in Multiple Enchondromatosis

  • Hae-Ryong Song,
  • Kwang-Won Park

摘要

Genu valgum and leg-length discrepancy are common deformities in patients with multiple enchondromatosisMultiple enchondromatosis. However, deformity correction can be fraught with complications, mainly due to the poor bone qualityPoor bone quality of the extensive lesion and the large amount of lengthening required. Curettage and bone grafting and internal fixation are not sufficient methods to treat this disease. A possible problem is defective bone mineralizationDefective bone mineralization, and the question of whether an external fixator can be applied across the bone lesion, as well as making osteotomy cuts at the site of the lesion, should be addressed. There can also be recurrence of the shortening and deformity. Another complication is pathologic fracture caused by delayed formation of regenerate, defective bone mineralization and an increased time in the external fixator. This usually occurs at the osteotomy site, where regenerate bone is believed to still be abnormal tissue. Another area of contention is the site of osteotomy. It is believed that osteotomy at the disease focus is detrimental because of the problems listed above, as well as possible malignant transformation of the lesions. Recurrence of the leg-length discrepancy as well as the deformity is also common, especially in children with severe deformities. In fact, the use of distraction osteogenesis has made the management of problems associated with Ollier’s disease Ollier's diseasemore achievable.