Ollier´s disease: Multiple enchondromatosis

Abstract

Ollier disease is a rare condition involving the development of intraosseous benign tumors known as enchondromas. These are usually formed during early stages of growth when the cartilage or growth plate is still biologically active. Once the patient matures, these tumors cause dysmorphic deformities and fragile bone fractures in adulthood. It is important to search for cutaneous vascular tumors in order to differentiate with Maffuci syndrome. Malignization of the enchondromas is also a severe complication. Regular control of the patients is key to detect malignant transformations and stablish an early treatment. Malignant transformation involves the development of increased vascular structures around the tumor, thus gammagraphic imaging is key to control the disease. Literature describing this disease is rare and treatment options are limited. Surgical treatment is often required when the osteochondromas transform or become very symptomatic; causing pain and articular malfunction. Our report describes a case of Ollier disease diagnosed in our center and discusses the core concepts of the disease and therapeutic management.