Format

Send to

Choose Destination
Pediatr Hematol Oncol. 1998 Mar-Apr;15(2):179-85.

Maffucci's syndrome as cause of chronic liquorrhea.

Author information

1
Department of Paediatrics, Ernst Moritz Arndt University, Greifswald, Germany.

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.

PMID:
9592845
DOI:
10.3109/08880019809167233
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Taylor & Francis
Loading ...
Support Center