Hydranencephaly

General: Rare; development disorder in which cerebral hemispheres are replaced by a cystic space filled with cerebrospinal fluid, covered by intact meninges; short life expectancy of weeks to months, but adulthood is possible; positive association with cocaine.

Ocular: Pupillary abnormalities; strabismus; nystagmus; ptosis; optic nerve hypoplasia; chorioretinitis; retinal vessel attenuation; incomplete anterior chamber cleavage; microphthalmia; blepharospasm.

Clinical: Irritability; convulsions; muscular rigidity; decerebrate posturing; increasing head circumference; quadriplegia; psychomotor retardation.

Dominguez R, et al. Brain and ocular abnormalities in infants with in utero exposure to cocaine and other street drugs. Am J Dis Child 1991; 145:688-695.

Herman DC, et al. Ophthalmic findings of hydranencephaly. J Pediatr Ophthalmol Strabismus 1988; 25: 106-l11.

Hill K, et al. Ocular signs associated with hydranencephaly. Am J Ophthalmol 1961; 51:267-275.

Manschot WA. Eye findings in hydranencephaly. Ophthalmologica 1971; 162: 151-l59.