Introduction: Cranial remodeling for craniosynostosis is typically undertaken within the first year-of-life while the benefits of delayed surgical intervention for older patients are poorly characterized. We present a consecutive single center series of patients, which presented after one year-of-age with uncorrected craniosynostosis.
Methods: This retrospective study includes nine children; age at presentation was 6.6 years (range 4-17 years). Type of craniosynostosis, medical history, surgical findings, developmental abnormalities, ophthalmologic findings and clinical course were reviewed.
Results: Seven of nine patients presented with developmental delay and decline in school grades and did show significantly impaired neurocognitive function. Four patients presented with daily non-focal headaches. With the exception of the 17 yo male patient with pan suture synostosis, none of the patients were noted to have papilledema. On imaging, three patients presented with Chiari malformation w/wo syringohydromyelia. Preoperative ICP monitoring revealed significant elevated ICP with A- and B-wave activity overnight in the 17 yo male with pan suture synostosis, who had a history of Chiari decompression at the age of 11. Two of the cases with associated Chiari required a posterior fossa decompression (w/o cranial vault remodeling). Five of the patients underwent posterior cranial vault remodeling applying a split calvarial bone graft cranioplasty technique. Two of those patients had intraoperative intracranial pressure monitoring, which revealed moderate pressure elevation in one. There were no peri- or postoperative complications, including infection or residual bony defects at a follow-up period of 3 years. 2/4 patients who preoperatively presented with headaches experienced improvement, one after Chiari decompression and one after posterior cranial vault remodeling.
Conclusions: Our findings suggest that children who present with uncorrected craniosynostosis associated with headache, developmental delay, or Chiari malformation benefit from cranial reconstruction. Prospective studies are warranted to prove the impact of surgery on neurological improvement.
Patient Care: Patients above the age of one year may benefit from surgical treatement of uncorrected craniosynostosis.
Learning Objectives: A better understanding of surgical treatment for uncorrected cranioplasty.