Successful surgical repair of progressive exophthalmos caused by a meningocele in a patient with neurofibromatosis Type 1. Case report.
A case of surgical repair of progressive exophthalmos of the right eye in a 43-year-old woman with neurofibromatosis Type 1 (NF1) is presented. Preoperatively, the patient's ocular movements and visual fields were intact. Visual acuity was 20/30 on the right side and 20/20 on the left. Computerized tomography scanning demonstrated complete absence of the superolateral orbital wall on the right side with a large meningocele protruding into the right orbit. Intraoperatively, a new superolateral wall was constructed using the inner table of the left frontal bone as a bone transplant. A free galeoperiosteum flap was used for water-tight dural reconstruction. A few weeks postoperatively the patient's exophthalmos showed remarkable resolution. Her ocular movements, visual acuity, and visual fields remained unchanged. In conclusion, reconstruction of the superolateral wall and repair of a meningocele in a patient with NF1 is worthwhile and can be followed by excellent cosmetic results. More important, the patient's visual functions remain preserved.
de Vries
J
J
Department of Neurosurgery, University of Nijmegen, The Netherlands.
Freihofer
H P
HP
Menovsky
T
T
Cruysberg
J R
JR
D000328:Adult; D005094:Exophthalmos / Q000209:etiology* / Q000601:surgery*; D005260:Female; D006801:Humans; D008588:Meningocele / Q000150:complications* / Q000000981:diagnostic imaging; D009456:Neurofibromatosis 1 / Q000150:complications*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome