RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164077573 T1 Maffucci Syndrome T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164077573 RD 2024/03/28 AB It is a sporadic disorder characterized by an association of multiple enchondromas (benign tumors of cartilage), and cavernous hemangiomas are often involving the head and neck. The enchondromas affect the extremities and their distribution is asymmetrical. The most common sites of enchondromas are the metacarpal bones and phalanges of the hands. Lymphangiomas can also be present. Patients are normal at birth. The bone and cartilage deformities appear during childhood, usually between the ages of 1 to 5 years. However, it can also appear at puberty. Patients affected have pathologic fractures and sarcomas. There is a normal intelligence.