Rajiv J Iyengar1, Petra M Klinge, Wendy S Chen, Jerrold L Boxerman, Stephen R Sullivan, Helena O Taylor. 1. *Warren Alpert Medical School of Brown University †Department of Neurosurgery ‡Division of Ophthalmology, Warren Alpert Medical School of Brown University, Rhode Island Hospital and Hasbro Children's Hospital §Department of Diagnostic Imaging, Rhode Island Hospital and Warren Alpert Medical School of Brown University ||Division of Plastic Surgery, Mount Auburn Hospital, Cambridge, MA.
Abstract
BACKGROUND: The natural history of unrepaired craniosynostosis is not well defined. Delayed surgical intervention carries greater risk of postoperative complications and its functional benefits for older patients are poorly characterized. The authors reviewed patients in whom children presented beyond 1 year of age to better understand the natural history of craniosynostosis, and the risk-benefit relationship for delayed reconstruction. METHODS: After institutional IRB approval the authors conducted a retrospective review of patients who presented after 1 year of age with craniosynostosis. Type of craniosynostosis, age at evaluation, medical history, surgical findings, developmental abnormalities, ophthalmologic findings, and clinical course were reviewed. RESULTS: Ten patients with delayed presentation for craniosynostosis were identified. The mean age at presentation was 6.8 years ± 4.2 years (range, 3-17 years). Seven of 10 patients presented with developmental delay. Five patients presented with debilitating headaches. Five patients presented with comorbid Chiari malformations, 3 of whom required surgical decompression. Two patients had papilledema. Four patients underwent intracranial pressure monitoring, with elevated pressures found in 3 patients. Six patients underwent delayed cranial vault remodeling. There were no peri- or postoperative complications, including infection or residual bony defects, in those undergoing delayed operation. CONCLUSIONS: Children who present in a delayed fashion with unrepaired craniosynostosis have high rates of debilitating headaches, developmental delays, head shape anomalies, and Chiari malformation. Five patients reporting preoperative headaches noted subjective improvements in headaches following delayed operation. Cranial reconstruction can be safely performed at an older age and is appropriate to consider in carefully selected patients for aesthetic and/or functional concerns.
BACKGROUND: The natural history of unrepaired craniosynostosis is not well defined. Delayed surgical intervention carries greater risk of postoperative complications and its functional benefits for older patients are poorly characterized. The authors reviewed patients in whom children presented beyond 1 year of age to better understand the natural history of craniosynostosis, and the risk-benefit relationship for delayed reconstruction. METHODS: After institutional IRB approval the authors conducted a retrospective review of patients who presented after 1 year of age with craniosynostosis. Type of craniosynostosis, age at evaluation, medical history, surgical findings, developmental abnormalities, ophthalmologic findings, and clinical course were reviewed. RESULTS: Ten patients with delayed presentation for craniosynostosis were identified. The mean age at presentation was 6.8 years ± 4.2 years (range, 3-17 years). Seven of 10 patients presented with developmental delay. Five patients presented with debilitating headaches. Five patients presented with comorbid Chiari malformations, 3 of whom required surgical decompression. Two patients had papilledema. Four patients underwent intracranial pressure monitoring, with elevated pressures found in 3 patients. Six patients underwent delayed cranial vault remodeling. There were no peri- or postoperative complications, including infection or residual bony defects, in those undergoing delayed operation. CONCLUSIONS:Children who present in a delayed fashion with unrepaired craniosynostosis have high rates of debilitating headaches, developmental delays, head shape anomalies, and Chiari malformation. Five patients reporting preoperative headaches noted subjective improvements in headaches following delayed operation. Cranial reconstruction can be safely performed at an older age and is appropriate to consider in carefully selected patients for aesthetic and/or functional concerns.