The unusual case of a 4-yr, 5-mo-old boy with multiple meningiomas without neurofibromatosis is reported from the Children’s Memorial Hospital and Northwestern University Medical School, Chicago, Illinois. The boy had presented with an external deviation, severe loss of vision and retinal “scar“ in the right eye at 2 yrs of age and a history of progressive painless proptosis of the same eye for one year. At the time of diagnosis, a complete oculomotor nerve palsy with dilated pupil unreactive to direct and consensual light stimulation, ptosis, and a visual acuity of 20/300 were present on the right. Intracranial, intraorbital, and asymptomatic epidural cervical spine meningiomas were demonstrated by CT and MRI scans and by angiography. All tumors were surgically excised successfully. [1]

COMMENT. Proptosis was a late sign of the intraorbital meningioma in this case, preceded by external deviation of the eye and loss of vision for a period of 18 mos. Meningiomas may involve the nasal wall of the orbit and invade the olfactory groove as in the present case, or may originate at the sphenoidal ridge and cause the classical syndrome of unilateral anosmia, optic atrophy or papilledema, and exophthalmos. With extension of the tumor to the cribriform plate, the anosmia can be bilateral and more readily detected even in children.

The young child is unlikely to react to an oil of wintergreen or tooth-paste odor test. However, taste is partially perceived through the olfactory system, and the mother may have noticed a loss of appetite sufficient to excite an index of suspicion of involvement of the first cranial nerve, that often neglected part of the neurologic and eye examinations. Early diagnosis and treatment of meningiomas involving the orbit and presenting with loss of vision led to improved visual acuity in 9 of 10 patients with tumors less than 3 cm diameter and in 56% of a total of 85 patients followed postoperatively. [2]