From the Guidelines
Craniosynostosis can affect normal brain development in children if left untreated, leading to increased intracranial pressure and potential developmental delays, visual problems, or cognitive impairments. The condition is characterized by the premature closure of one or more fibrous joints between the bones of a baby's skull, causing the skull to grow abnormally. According to the clinical practice recommendations for the diagnosis and management of x-linked hypophosphataemia 1, craniosynostosis can occur as early as 1 year of age and usually involves an abnormal fusion of the sagittal suture.
Key Considerations
- Craniosynostosis should be suspected in children with signs of intracranial hypertension, such as headache, neck pain, or papilledema 1.
- Regular developmental monitoring is essential for children with craniosynostosis, and early intervention services may be beneficial even after surgical correction.
- The prognosis is generally good with timely intervention, with most children achieving normal cognitive development, though some may require additional support for specific learning or behavioral challenges.
- The risk of developmental issues increases with more complex forms involving multiple sutures or when craniosynostosis is part of a genetic syndrome.
Recommendations
- Treatment typically involves surgery to separate the fused sutures, allowing the brain adequate space to grow 1.
- The timing of surgery is crucial, usually performed between 3-12 months of age depending on the type and severity.
- Follow-up care should include measuring height, weight, head circumference, and blood pressure, as well as assessing bone age and growth potential in children with growth impairment 1.
From the Research
Effects of Craniosynostosis on Brain Development
- Craniosynostosis is a rare congenital disease of the skull that arises when one or more cranial sutures ossify prematurely, causing an obstruction to normal brain growth and leading to specific deformations of the skull 2.
- This condition may result in intracranial hypertension and cognitive delay, highlighting the potential impact on normal brain development in children 2, 3.
- The treatment of craniosynostosis is surgical and requires a multidisciplinary approach, with the primary aim of surgical treatment being to permit a normal development of the brain 2.
Neurocognitive Outcomes
- Children with craniosynostosis experience significant cognitive dysfunction later in life, with neurodevelopmental problems including cognitive and language impairments, motor delays or deficits, learning disabilities, executive dysfunction, and behavioral problems 3, 4.
- Studies have shown that children with multiple suture craniosynostosis have more impairment than children with single-suture craniosynostosis, and that children with isolated single-suture subtypes of craniosynostosis can have distinct neurocognitive profiles 4.
Importance of Early Diagnosis and Treatment
- When left untreated, craniosynostosis can cause serious complications, such as developmental delay, facial abnormality, sensory, respiratory and neurological dysfunction, anomalies affecting the eye, and psychological disturbances 5.
- Early diagnosis, expert surgical techniques, postoperative care, and adequate follow-up are of vital importance in treating craniosynostosis and potentially improving neurocognitive outcomes 2, 5.