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Longitudinal Assessment of Developmental Outcomes in Infants Undergoing Late Craniosynostosis Repair
Stefani C Fontana1, Skylar Belinger2, Debby Daniels2
1Department of Plastic Surgery.
Insights
Craniosynostosis surgery in infants over 12 months old showed improved neurocognitive development in 80% of cases. This study highlights the benefits of cranial vault remodeling for late-presenting developmental delays.
Area of Science:
- Craniofacial Surgery
- Pediatric Neurosurgery
- Developmental Pediatrics
Background:
- Surgical repair of craniosynostosis is typically recommended between 3 and 12 months of age.
- Limited data exists on neurocognitive outcomes for infants with uncorrected craniosynostosis presenting after 12 months.
- The impact of cranial vault surgery on neurocognitive development requires further investigation.
Purpose of the Study:
- To evaluate the neurocognitive development of infants with nonsyndromic craniosynostosis presenting after 12 months of age.
- To assess the impact of cranial vault remodeling on cognitive development in this patient group.
- To investigate the association between surgical intervention and cognitive improvement.
Main Methods:
- A prospective, nonrandomized study enrolled infants with nonsyndromic craniosynostosis presenting after 12 months.
- The Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) was used for pre- and postoperative cognitive assessment.
- Developmental delay was defined as a Bayley-III score below 85.
Main Results:
- Four out of five infants (80%) presented with developmental delay (Bayley-III scores: 60-80).
- All infants with preoperative developmental delay showed cognitive improvement after cranial vault remodeling (postoperative scores: 75-90).
- The infant without preoperative delay maintained normal cognitive scores post-surgery.
Conclusions:
- Cranial vault surgery is associated with cognitive improvement in infants with nonsyndromic craniosynostosis presenting after 12 months of age.
- Late surgical intervention may positively impact neurocognitive development in this population.
- Further research is warranted to understand the long-term neurocognitive effects of early and late surgical repair.
Abstract:
Evaluation of infants with craniosynostosis for surgical intervention, as opposed to conservative management, remains a challenge within the field of craniofacial surgery. Studies have consistently demonstrated that surgical repair of craniosynostosis is ideally performed between 3 and 12 months of age. As such, there is limited data regarding neurocognitive development in infants who initially present with uncorrected craniosynostosis after 12 months of age. Moreover, the impact of cranial vault surgery on neurocognitive development at all ages remains under investigation. A prospective, nonrandomized study was performed. All children with nonsyndromic craniosynostosis who presented for initial evaluation after 12 months of age were enrolled. The Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) was utilized to assess pre- and postoperative cognitive development and comparisons were made to normative values. Developmental delay is defined as scoring < 85. Five infants, average age 26 months (13-43 months) at initial presentation, underwent cranial vault remodeling and developmental testing. Fused cranial sutures involved: metopic (n = 4), and right coronal (n = 1). Cognitive testing demonstrated that 4 of 5 infants (80%) were developmentally delayed at presentation (scores: 60, 70, 72, and 80), and 1 infant was within normal limits (score: 100). Postoperative testing was performed between 2 and 12 months postoperatively. Universal improvement was observed in infants who were delayed prior to surgery (80, 80, 75, and 90, respectively). The infant who was not delayed prior to surgery remained within normal limits after surgery. This study demonstrates an association between cranial vault surgery and cognitive improvement in infants presenting late with developmental delay.