We reviewed 61 @entity1 seen from 1975 to 1994 with a biopsy-confirmed optic chiasm @entity816 or a characteristic clinical and imaging presentation. The mean age at diagnosis was 72 months, including 30 who were less than 5 years old. The mean @entity5 diameter at presentation was 31 mm among 54 @entity5 measured. @entity5 involved the optic nerve in 41, hypothalamus in 38, optic tract in 15, and optic radiations in 2. Four @entity1 , all with @entity4786 , received no treatment. Forty @entity1 received conventional radiation therapy at a mean age of 89 months. Chemotherapy was given to 19 @entity1 and 7 of these were followed for greater than 1 year. Six of these 7 had progressive disease which required subsequent surgery and/or radiation therapy. Thirty-six @entity1 underwent surgery. We restricted our analysis of outcome to those 38 @entity1 followed for longer than 60 months. Development was normal in 13%, mildly disabled in 53%, severely disabled in 21% and dead in 13%. @entity1 were severely disabled or dead in 47% if younger than 5 years at diagnosis, and 21% if older. There was no endocrine dysfunction in 18% and new endocrine failure was seen in 61%. More @entity5 did not portend a poorer prognosis. @entity1 less than 5 years old had a poorer prognosis. @entity4786 did not have a marked effect on outcome. Intracranial @entity101 was an indicator of a poor prognosis. No specific treatment modality was clearly superior in terms of long-term survival or improvement in quality of life.

Optic chiasm XXXX of childhood. 1. Long-term follow-up.

@entity1 :: ('9606', 'Species') :: ['children', 'patients', 'Children'] @entity101 :: ('MESH:D006973', 'Disease') :: ['hypertension'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor', 'Tumors', 'posterior tumors', 'tumors'] @entity816 :: ('MESH:D001254', 'Disease') :: ['astrocytoma'] @entity4786 :: ('MESH:D017253', 'Disease') :: ['Neurofibromatosis', 'neurofibromatosis']
@entity816 :: (MESH:D001254,Disease) :: ['astrocytomas']
