Maffucci's Syndrome as Cause of Chronic Liquorrhea
| Autor: | S Wiersbitzky, J A Assaf, E Werner, H Wiersbitzky, M R Gaab, P Müller, R D Stenger |
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| Rok vydání: | 1998 |
| Předmět: |
medicine.medical_specialty
Adolescent Cerebrospinal Fluid Otorrhea Cerebrospinal Fluid Rhinorrhea business.industry Enchondromatosis Hematology Angiomatosis medicine.disease Surgery body regions Hemangioma medicine.anatomical_structure Oncology Posterior cranial fossa Recurrent meningitis Pediatrics Perinatology and Child Health Lymphangioma medicine Enchondroma Humans Female business Cervical vertebrae |
| Zdroj: | Pediatric Hematology and Oncology. 15:179-185 |
| ISSN: | 1521-0669 0888-0018 |
| DOI: | 10.3109/08880019809167233 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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