Ocular manifestations of intracranial chordomas.

Sixty-three patients with confirmed intracranial chordoma were studied retrospectively to determine the incidence of various presenting symptoms and signs. Most of the ocular signs were due to cranial nerve involvement. Sixth cranial nerve palsy occurred as the sole presenting sign in 29% of patients, whereas extraocular muscle palsies of various combinations were present in 62%. Visual field defects were demonstrated in 24% of patients, but only 19% had papilledema or optic atrophy. The chordomas arose from the clivus in 92% of patients, but different patterns of cranial nerve involvement occurred which correlated with the different sites of extension of the tumor.