Neuro-ophthalmologic manifestations of Maffucci's syndrome and Ollier's disease.
Patients with Ollier's disease (multiple skeletal enchondromas) and Maffucci's syndrome (multiple enchondromas associated with subcutaneous hemangiomas) may develop skull base chondrosarcomas or low-grade astrocytomas as a delayed consequence of these disorders. We report three patients with Ollier's disease and Maffucci's syndrome who had diplopia as the initial manifestation of intracranial tumors. Since patients with Maffucci's syndrome and Ollier's disease are at risk for the delayed development of brain and systemic neoplasms, neuroophthalmologists must be aware of the need for long-term surveillance in patients affected by these conditions.
Balcer
L J
LJ
Department of Neurology, Hospital of the University of Pennsylvania, Scheie Eye Institute, University of Pennsylvania School of Medicine, Philadelphia, USA.
Galetta
S L
SL
Cornblath
W T
WT
Liu
G T
GT
D000010:Abducens Nerve / Q000473:pathology; D000293:Adolescent; D000328:Adult; D001254:Astrocytoma / Q000175:diagnosis*; D001921:Brain / Q000473:pathology; D001932:Brain Neoplasms / Q000175:diagnosis*; D002813:Chondrosarcoma / Q000175:diagnosis*; D003389:Cranial Nerve Diseases / Q000175:diagnosis; D004172:Diplopia / Q000175:diagnosis*; D004687:Enchondromatosis / Q000175:diagnosis*; D005154:Facial Nerve / Q000473:pathology; D005155:Facial Nerve Diseases / Q000175:diagnosis; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010243:Paralysis / Q000175:diagnosis; D012886:Skull / Q000473:pathology; D019292:Skull Base Neoplasms / Q000175:diagnosis*; D014276:Trigeminal Nerve / Q000473:pathology