Intracranial abnormalities and ventricular patterns associated with meningoencephaloceles

Twenty-eight patients with meningoencephalocoeles with a mean age of 11 months were studied. CT scans showed abnormal ventricular patterns and other intracranial abnormalities in 78%, about 2 3 of whom had multiple defects. There was a direct relationship between the number of intracranial abnormalities and the amount of herniated brain and not with the size of the meningocele sac per se. We believe that traction on the intracranial contents by progressive brain herniation is a primary mechanism leading to the development of multiple abnormalities. It is therefore recommended that early closure of the bone and dural defects is necessary to prevent further brain herniation. We have utilized multiple stage operations including cerebrospinal fluid shunting for hydrocephalus, when present, transcranial extradural closure of the bone and dural defects, and cosmetic repair of the mengingocoele sac at a much later date.