MAFFUCCIS-SYNDROME AS CAUSE OF CHRONIC LIQUORRHEA

We present the case of a 14-year-old girl suffering from Maffucci's sy ndrome, a rare ailment belonging to the group of osteochondrodysplasia s. At the age of 6 months, a diffuse swelling first appeared in the gi rl's right cheekbone region and the periauricular area. Because of rec urrent meningitis with massive otoliquorrhea, several surgical revisio ns were performed, beginning at the age of 4 years. The histological a nd immunohistochemical diagnosis showed hemangioma and lymphangioma wi th enchondroma. As a peculiarity of our patient's diagnosis, we found multiple bone defects apparently caused by venous and lymphomatous ang iomatosis. There was also en chondromatosis of the skull base and the upper cervical vertebrae, which caused the recurrent otoliquorrhea and rhinoliquorrhea. A fistula closure was undertaken through a retromast oidal, suboccipital approach and fascial graft of the posterior crania l fossa.