Related Experiment Videos
Raised intracranial pressure in minimal forms of craniosynostosis
J F Martínez-Lage1, L Alamo, M Poza
1Regional Service of Neurosurgery, Virgen de la Arrixaca University Hospital, National Institute of Health, Murcia, Spain.
Insights
Occult craniosynostosis, a mild form of premature skull fusion, can present in childhood with severe intracranial pressure (ICP) despite minimal skull deformity. Surgical intervention relieved ICP symptoms in affected children.
Area of Science:
- Pediatric Neurosurgery
- Craniofacial Surgery
- Developmental Pediatrics
Background:
- Craniosynostosis is typically diagnosed in infancy.
- Mild forms may go undetected until late childhood.
- These mild cases often present with minimal neurological symptoms.
Observation:
- Two children, aged 9 and 6, presented with significant signs of raised intracranial pressure (ICP).
- Skull deformity in these patients was negligible.
- These cases were termed 'occult craniosynostosis'.
Findings:
- Differential diagnosis focused on excluding other causes of benign intracranial hypertension.
- Bilateral expanding craniotomies successfully resolved symptoms of raised ICP.
- This highlights a potential presentation of craniosynostosis.
Implications:
- Neurosurgeons should consider occult craniosynostosis in children with unexplained benign intracranial hypertension.
- Early recognition and surgical treatment can alleviate severe neurological symptoms.
- This expands the understanding of craniosynostosis presentation and management.
Abstract:
Most cases of craniosynostosis are diagnosed during early infancy, but occasionally craniosynostosis evolves with minimal cranial involvement and goes unnoticed until late childhood. Seemingly these mild forms of craniosynostosis cause few, if any, symptoms of neurological involvement. We describe the cases of a 9-year-old girl and a 6-year-old boy who presented with evident signs of raised intracranial pressure (ICP), together with a negligible skull deformity. We have termed these cases as occult craniosynostosis. Differential diagnosis in our patients was established against known causes of benign intracranial hypertension. Bilateral expanding craniotomies afforded total relief from the symptoms and signs of raised ICP. Neurosurgeons treating children with symptoms and signs of benign intracranial hypertension should be aware of the possibility of minimal forms of craniosynostosis evolving with marked manifestations of raised ICP.