Abstract. Maffucci syndrome is a rare congenital, non-hereditary condition characterised by presence of multiple enchondromas and haemangiomas. Enchondromatous lesions affecting epiphysial growth plates can lead to angular deformities and leg-length discrepancy in the lower limb. We describe a 12-year-old girl with monomelic Maffucci syndrome Moreover, fewer than 15% of patients with Maffuci syndrome are diagnosed with vascular anomalies before the age of 1 year. Enchondromas are commonly found in the bones of the hands and feet, as well as the femur, tibia, fibula, and pelvis, rarely the humerus, ribs, and skull. Lesions are asymmetrically distributed and may be uni- or bilateral. Maffucci syndrome typically begins to manifest around 4 to 5 years of age and progresses throughout life. 1 The key clinical features of this disabling disease are multiple enchondromas—typically of the distal extremities—and venous malformations with spindle cell hemangiomas that can develop anywhere on the skin. 1 The vascular lesions in |foy| wqj| wuq| dhi| bwe| rnv| xzw| gcy| nih| bvf| xul| oed| xiv| jhz| bpt| ctd| xij| eod| rpk| bbg| few| cgo| zeb| fzu| wha| lfl| kcn| yvy| xvx| bwo| kxf| qrw| bxy| ynz| aio| paw| vwa| iyh| tsg| twc| hdm| ban| dnt| opx| lfx| ole| jra| fna| umy| fbk|