Concurrent myelomeningocele and sagittal craniosynostosis: illustrative case

Myelomeningocele and sagittal craniosynostosis are 2 neurosurgical pathologies with complications such as increased intracranial pressure (ICP) and hydrocephalus. While the 2 defects commonly occur independently, their simultaneous occurrence is exceptionally rare.

The authors report the case of a newborn male diagnosed with a simultaneous myelomeningocele and sagittal craniosynostosis. The patient underwent surgical repair of the myelomeningocele 2 days after birth. Wound breakdown and ventriculomegaly were observed 13 days after birth. Surgical wound repair was performed, and a neonatal reservoir was inserted because of concerns for wound healing and increased ICP. The reservoir was tapped daily, and the ventricle size was monitored via biweekly ultrasounds. Due to the increased risk of ICP presented by the sagittal craniosynostosis, an open strip craniectomy was performed at 34 days of life. Following surgery, cranial ultrasound studies demonstrated improving ventricle size and neonatal reservoir tapping was spaced to weekly. The patient was discharged at 68 days of life in stable condition, with no permanent cerebrospinal fluid diversion needed at the 8-month follow-up.

Surgical repair of sagittal craniosynostosis in the presence of a myelomeningocele should be considered earlier in life than generally indicated to limit the risk of developing elevated ICP, hydrocephalus, and subsequent complications. https://thejns.org/doi/10.3171/CASE24616.