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Neurocognitive sequelae of scaphocephaly
R Virtanen1, T Korhonen, J Fagerholm
1Department of Pediatric Surgery, Turku University Central Hospital, Turku, Finland.
Insights
Early cranioplasty for scaphocephaly improves skull shape but may not fully restore cognitive development. Early surgical intervention (within 1 month) might mitigate some developmental delays in children with this condition.
Area of Science:
- Pediatric Neurosurgery
- Developmental Neuroscience
- Craniofacial Surgery
Background:
- Scaphocephaly, a skull deformity due to premature sagittal suture fusion, is often treated with early cranioplasty.
- While cranioplasty normalizes skull shape and reduces intracranial pressure, its impact on neurocognitive outcomes remains unclear.
Purpose of the Study:
- To evaluate the long-term neurocognitive development of children treated for scaphocephaly with early cranioplasty.
- To compare the neurocognitive outcomes of these children with age- and gender-matched healthy controls.
Main Methods:
- Eighteen infants with scaphocephaly underwent cranioplasty between 1 week and 7 months of age.
- Patients were followed and neurocognitive development assessed at school age (7.8–16.3 years) using the Wechsler Intelligence Scale for Children-Revised.
Main Results:
- Children operated on for scaphocephaly exhibited mild deficits in auditory short-term memory and language development compared to controls.
- Overall developmental outcomes were comparable to controls in all other assessed neurocognitive domains.
Conclusions:
- Early cranioplasty for scaphocephaly does not fully normalize neurocognitive performance by school age, suggesting potential in-utero brain development impairment.
- Intervention within the first month of life may reduce developmental delays, supporting early surgery for both skull correction and brain development.
- Delaying surgery is not advisable as it may hinder both skull normalization and optimal brain development.
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.