Maffucci's Syndrome as Cause of Chronic Liquorrhea

Abstract

We present the case of a 14-year-old girl suffering from Maffucci's syndrome, a rare ailment belonging to the group of osteochondrodysplasias. At the age of 6 months, a diffuse swelling first appeared in the girl's right cheekbone region and the periauricular area. Because of recurrent meningitis with massive otoliquorrhea, several surgical revisions were performed, beginning at the age of 4 years. The histological and immunohistochemical diagnosis showed hemangioma and lymphangioma with enchondroma. As a peculiarity of our patient's diagnosis, we found multiple bone defects apparently caused by venous and lymphomatous angiomatosis. There was also enchondromatosis of the skull base and the upper cervical vertebrae, which caused the recurrent otoliquorrhea and rhinoliquorrhea. A fistula closure was undertaken through a retromastoidal, suboccipital approach and fascial graft of the posterior cranial fossa.

Key Words:

• enchondroma
• hemangioma
• liquorrhea
• Maffucci's syndrome
• skull base

Additional information

Notes on contributors

R.-D. Stenger

Joyce Laing works in the Department of Child and Family Psychiatry, Playfield House, Cupar, Fife, and is a Consultant Art Therapist to Psychiatric Hospitals and Prisons and Chairwoman of the Scottish Society of Art and Psychology.

Dr Niculescu Dan, Centrul de Reumatologie, Str. J. (F)ucick no. 5, Bucuresti, Romania

Anni Vilppula, Department of Medicine, Paimio Hospital, Preitilä, Finland

G. Tausch, Department of Rheumatology, Municipal Hospital of Vienna-Lainz, Wolkersbergenstraße 1, A-1130Wien, Austria

Dr Guido Gothoni, Medica Pharmaceutical Company Ltd., P.O. Box 325, SF-00101 Helsinki 10, Finland

A. Elman, Dept. of Rheumatology, Karolinska sjukhuset, Stockholm, Sweden

Hannu Paitälä, Rheumatism Foundation Hospital, Heinola, Finland

Jonas Jonsson, National Bacteriological Laboratory, S-105 21 Stockholm, Sweden