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 malignisation of one or several foci consecutively. Ollier’s disease needs to be differentiated from multiple hereditary tumour-like diseases and true tumours of the skeleton, exostoses, chondrosarcomas, periosteal osteosarcoma, osteoblastoclastoma, neurofibromatosis. Key in diagnosing and defining the treatment tactics is histological examination. 3 patients aged from 19 months to 16 years were operated for multiple chondromatosis with affected chest wall complicated by orthopedic or pulmonary disorders. The diagnosis was confirmed by morphological study.

Objective. The analysis of a small clinical series of multiple bone chondromatosis (Ollier’s disease) of rare locations in children.

Маterials and methods. A retrospective analysis of 3 cases of bones’ multiple chondromatosis with rib cage involvement in children is given, that led to orthopedic and pulmonary complications.

Results. Inallcases, the diagnosis was veri fiedhistologically. Surgical treatment allowed to resolve complaints and potential chondromas’ malignisation, however, gave rise to secondary orthopedic pathology (spinal deformity) in one case.

Conclusions. The examples presented de monstrate diverse clinical manifestations of rib cage lesions in children with Ollier’s disease — from minimal to highly aggressive, demanding, first and foremost, exclusion of chondromas’ malignisation at early age; and this is impossible 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.