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Show ] Ollmal of Clinical Nellro- ol'lithalrnology 8( 1): 69- 71, 1988. Neuroradiological Clinical Pathological Correlation ~ ; 988 Raven Press, Ltd., New York Congenital Glaucoma and Buphthalmos In a Child with Neurofibromatosis Mauricio Castillo, M, D., Robert M. Quencer, M. D., * Joel Glaser, M. D., and tNolan Altman, M. D. A I- month- old boy was admitted to Bascom Palmer Eye Institute with a history of an enlarged left eye since birth. He was born by cesarian section at term to a 43- year- old multigravida. The father, paternal grandfather, and several aunts and uncles had cutaneous stigmata of neurofibromatosis. Initial physical examination revealed multiple cafe- au- lait spots and left exophthalmos. Palpebral movement was normal, but the left upper eyelid appeared swollen, The left proptosis increased when the child cried. Under general anesthesia, ocular tension was 40 mm Hg, left eye, and 15 mm Hg, right eye. Corneal diameters were 11.5 mm, left, and 10.0 mm, right. Funduscopy revealed cupping of the left optic disk. Orbital ultrasonography showed no optic nerve or soft tissue abnormalities. Clinical diagnosis of primary congenital glaucoma was made, and goniotomy of the left eye was performed. Follow- up examination at the age of 6 months showed persistent glaucoma of the left eye and corneal edema. Preoperatively, the patient was treated with acetozolamide, and the intraocular pressures obtained under general anesthesia were 28 mm Hg, left, and 10 mm Hg, right. The corneal From the Departments of Diagnostic Radiology and ' Ophthalmology, University of Miami School of Medicine/ Jackson Memorial Medical Center; and the tDepartment of Radiology, Miami Childrens Hospital. Miami, Florida. Address correspondence and reprint requests to Dr. Robert M. Quencer, M. D., Director, Magnetic Resonance Imaging, Department of Radiology ( R- 308), University of Miami School of Medicine, P. O. Box 016308, Miami, FL 33101, U. S. A. 69 diameters were 12.4 mm left, and 11.25 mm, right. A second goniotomy was performed. When the patient was 17 months of age, ultrasound examination of the eyes showed an enlarged left globe and mild enlargement of the left optic nerve ( 3.9 mm) when compared with the right optic nerve ( 3 mm). Under general anesthesia, intraocular tensions were 43 mm Hg, left, and 12 mm Hg, right. The corneas measured 13.8 mm, left, and 11.5 mm, right. A trabeculotomy was performed. Computed tomography ( CT) scan of the orbits using 3- mm sections before and after administration of iodinated contrast material confirmed the presence of an enlarged left globe ( Fig. lA). Both optic nerves were of normal and equal size, but the left superior orbital fissure was expanded. A lobulated soft tissue- enhancing density was noted within the left superior orbital fissure. The left greater sphenoid wing was hypoplastic, and there was an area of low attenuation anterior to the left temporal lobe ( Fig. 18). DISCUSSION Proptosis occurring in patients with neurofibromatosis may be pulsatile or nonpulsatile. Pulsatile exophthalmos is more common and is frequently associated with hypoplasia of the ipsilateral greater sphenoidal wing and with other defects of the orbital walls ( 1,2). Herniation of the brain into the orbit through a bony defect and transmission of the cerebral arterial pulsations are believed to be CONGENITAL GLAUCOMA AND BUPHTHALMOS 71 sphenoid wing is probably a subarachnoid cyst or a widened subarachnoid space ( Fig. 1B). The right globe, appendages, and bony orbit were normal. In summary, buphthalmos is a relatively rare sign of infantile glaucoma and is seen in association with neurofibromatosis. Evaluation of the retrobulbar space is easily done by contrast- enhanced CT, and the presence of neurofibromatosis related tumors can be established, as well as any associated bone abnormalities. REFERENCES 1. Binet EF, Kieffer SA, Martin SH, Peterson HO. Orbital dysplasia in neurofibromatosis. Radiology 1969; 93: 829- 33. 2. LeWalt LT. Congenital absence of the superior orbital wall associated with pulsating exophthalmos. Am J Roentgenol 1933; 30: 756- 64. 3. Zimmerman RA, Bilaniuk LT, Metzger RA, et al. Com-puted tomography of orbito- facial neurofibromatosis. Radiology 1983; 146: 113- 16. 4. Rubenstein AE, Mytilineoau C, Yahr MD, et al. Neurological aspects of neurofibromatosis. Adll Neural 1978; 29: 1121. 5. McKeen EA, Bodurtha J, Meadows AT, et al. Rhabdomyosarcomas complicating multiple neurofibromatosis. J Pediatr 1978; 93: 992- 3. 6. Burrows EH. Bone changes in neurofibromatosis. Br / RadioI1963; 36: 549- 61. 7. Kobrin JL, Block FC, Weingest TA, et al. Ocular and orbital manifestations of neurofibromatosis. SlIrv Ophthalmol 1979; 24: 45- 51. 8. Davis FA. Primary tumors of the optic nerve ( Phenomena of Recklinghausen's disease). Arch OphthalmoI1940; 23: 735821, 957- 1018. 9. Satran L, Letson RD, Seljeskog EL. Neurofibromatosis with congenital glaucoma and buphthalmos in a newborn. Am J Dis Child 1980; 134: 182- 3. 10. Shaffer RN. Congenital glaucoma. lnt Ophtha/ mol Clin 1963; 3: 107- 16. 11. Morin JD. Glaucoma. In: Crawford JS, Morin JD, eds. The eye in childhood, 1st ed. New York: Grune & Stratton, 1983: 331- 48. I Clill Nellro- ophthalmol, Vol. 8. No. 1. 1988 |
