Progressive visual loss is the most common neurologic finding in osteopetro
sis. Several mechanisms mall explain this phenomenon, including compression
of the optic nerves caused by bony overgrowth of the optic canals and reti
nal degeneration. We report a child with osteopetrosis and progressive visu
al loss, even though patent optic canals were demonstrated by computed tomo
graphy and digital holography. This patient's visual loss was caused by inc
reased intracranial pressure secondary to obstruction of cerebral venous ou
tflow at the jugular foramen. This case points to the importance of a full
evaluation of the skull base foramina in the diagnostic workup of visual lo
ss in patients with osteopetrosis. (Surv Ophthalmol 43:487-490, 1999. (C) 1
999 by Elsevier Science Inc. All rights reserved.).