Maffucci syndrome
Overview
Maffucci syndrome is a sporadic disease characterized by the presence of multiple enchondromas associated with multiple simple or cavernous soft tissue hemangiomas. Also lymphangiomas may be apparent.
Symptoms
* Vowing of long bones * Retarded growth * Endochondromata * Hemangiomata * Capillary hemangiomata
Causes
The cause of this syndrome is unclear. It has no familial pattern of inheritance and appears sporadically.
Diagnosis
In Ollier disease isolated enchondromas are present without the presence of hemangiomas.
Treatment
Management entails careful examination and monitoring for malignant degenerations. Surgical interventions can correct or minimize deformities.