Oculo cerebral dysplasia


A very rare syndrome characterized by small eyes, optic nerve abnormality and a brain anomaly (Dandy-Walker cyst)


* Small eyes * Optic nerve abnormality * Dandy-Walker cyst * Optic disc abnormality


Hydrocephalus may result from an obstruction in CSF flow (noncommunicating hydrocephalus) or from faulty absorption of CSF (communicating hydrocephalus). (See Normal circulation of CSF.) In noncommunicating hydrocephalus, the obstruction occurs most frequently between the third and fourth ventricles, at the aqueduct of Sylvius, but it can also occur at the outlets of the fourth ventricle (foramina of Luschka and Magendie) or, rarely, at the foramen of Monro. This obstruction may result from faulty fetal development, infection (syphilis, granulomatous diseases, meningitis), a tumor, cerebral aneurysm, or a blood clot (after intracranial hemorrhage). In communicating hydrocephalus, faulty absorption of CSF may result from surgery to repair a myelomeningocele, adhesions between meninges at the base of the brain, or meningeal hemorrhage. Rarely, a tumor in the choroid plexus causes overproduction of CSF, producing hydrocephalus. Hydrocephalus occurs most commonly in neonates but can also occur in adults as a result of injury or disease. It affects 1 of every 1,000 people.