Neurocognitive Sequelae of Scaphocephaly

Abstract

Objective. Early cranioplasty for scaphocephaly has become routine in most countries. In addition to normalizing the shape of the skull, it has been found to decrease intracranial hypertension. Whether corrective surgery benefits the child's cognitive outcome has been poorly documented. Design. Eighteen children whose sagittal suture showed premature fusion at birth or soon thereafter were operated on at age 1 week to 7 months. All patients healed without complications and were followed-up at regular intervals. At the age of 7.8 to 16.3 years they were examined to clarify their neurocognitive development and to compare the results with their age- and gender-matched healthy controls. Results. Originally scaphocephalic children, although operated on, had mild deficiencies in auditory short-term memory and language development when examined with the general comprehension, similarities, and digit span subscales of the Wechsler Intelligence Scale for Children–Revised. In all other respects their developmental outcome was equal to that of the controls. Conclusions. Despite relative early correction of the skull shape, originally scaphocephalic children's neurocognitive performances do not reach the same level in all of the neurocognitive domains as their matched controls at school age. Early operation (≤1 month) may decrease this developmental delay. This implies that impairment of brain function has already taken place in utero. For the same reason an early operation seems to be justified not only for correction of the skull shape, but also to allow unrestricted development for the brain. Postponement of the operation would not serve either of these aims.