Surgical removal of an "inoperable" neurofibroma

An 8-month-old girl had ipsilateral ptosis, proptosis, and glaucoma caused by a massive left-sided plexiform neurofibroma. The tumor extended from the cavernous sinus into the orbit, involving the optic nerve, extraocular muscles, and eyelid. Although the prognosis in such cases is considered very poor, most of the mass was excised by a radical neurosurgical and ophthalmologic procedure. Today, the patient remains asymptomatic with no further neurologic defects. This case is unusual in that the glaucoma developed prior to clinically evident eyelid involvement.