Visual recovery after a year of craniopharyngioma-related amaurosis: report of a nine-year-old child and a review of pathophysiologic mechanisms

Background: The probability of visual recovery in tumor-related optic ne ur opathy usually correlates with the severity and duration of optic pathway c ompromise. Recovery of visual acuity to normal levels is unexpected after p rofound loss of vision extending for a period of weeks and months. Methods: A 9-year-old girl who had neurosurgical resection of a craniopharyngioma c ompressing the optic chiasm and optic tra ct was followed up serially with neuroimaging and clinical examinations over a 6-year period. Results: Withi n 3 months of the diagnosis of craniopharyngioma, the girl's vision was red uced to no-light-perception blindness when she viewed with the more involve d eye. The blindness correlated with an amaurotic tie, >3.6 log unit) relat ive afferent pupillary defect and an absence of any response when tested wi th visual field perimetry. After more than a year of total blindness and ce ssation of all neurosurgical and radiation therapy, visual acuity recovered to a normal level (20/25), the afferent pupillary defect improved, and sen sitivity in a portion of the temporal hemivisual field was restored. In the follow-up that has extended for 5 years from the time of recovery, stabili ty of the restored vision has been documented. Conclusion: Children who hav e tumor-related loss of vision due to damage to the anterior visual pathway s may be capable of recovery after intervals of blindness that would be con sidered irreversible in adults. The mechanism of the recovery in our patien t may have been decompression-related restoration of axoplasmic flow, follo wed by gradual remyelination of visual fibers, which allowed reorganization of connections to the lateral geniculate nucleus to optimize synaptic tran smission.