Multiple bone chondromatosis (Оllier’s disease) affected the chest in children (small clinical series)

Abstract

Multiple bone chondromatosis (Ollier’s disease) is 2 times more common in girls and most often manifests itself in childhood from 1 to 5 years, less often in adolescence. In clinical practice, enchondromatosisis usually divided into 3 options, Ollier’s disease, Maffucci’s syndrome (with the highest risk of malignisation), and lesions of predominantly small bones of the hands. In adulthood, almost in half of cases detected (48%) the disease is exarcerbated by ma-lignisation of one or several foci consecutively. Ollier’s dis-ease needs to be differentiated from multiple hereditary tumour-like diseases and true tumours of the skeleton, exostoses, chondrosarcomas, periosteal osteosarcoma, osteoblastoclastoma, neurofibromatosis. Key in diagnos-ing and defining the treatment tactics is histological ex-amination. 3 patients aged from 19 months to 16 years were operated for multiple chondromatosis with affected chest wall complicated by orthopedic or pulmonary dis-orders. The diagnosis was confirmed by morphological study. Objective. The analysis of a small clinical series of multiple bone chondromatosis (Ollier’s disease) of rare lo-cations in children. Маterials and methods. A retrospec-tive analysis of 3 cases of bones’ multiple chondromatosis with rib cage involvement in children is given, that led to orthopedic and pulmonary complications. Results. Inall-cases, the diagnosis was veri fiedhistologically. Surgical treatment allowed to resolve complaints and potential chondromas’ malignisation, however, gave rise to sec-ondary orthopedic pathology (spinal deformity) in one case. Conclusions. The examples presented de monstrate diverse clinical manifestations of rib cage lesions in chil-dren with Ollier’s disease — from minimal to highly ag-gressive, demanding, first and foremost, exclusion of chondromas’ malignisation at early age; and this is impos-sible otherwise than by total resection. Treatment of such children should not be limited to surgical correction only; to improve prognosis, constant monitoring is needed by pediatrician, oncologist and geneticist.