From the Guidelines
Craniosynostosis is a condition where one or more of the fibrous joints between the bones of a baby's skull close prematurely, before the brain is fully formed, and can occur as early as 1 year of age, usually involving an abnormal fusion of the sagittal suture leading to a dolichocephalic conformation of the head with a reduced cranial index, as noted in a study published in Nature Reviews Nephrology 1.
Definition and Causes
Craniosynostosis is a birth defect that affects the growth of the skull, causing it to grow in an abnormal shape. Normally, the joints between the bones of the skull, called sutures, remain flexible during infancy to allow the brain to grow. When craniosynostosis occurs, the affected suture(s) transform into bone too early, limiting brain growth and potentially increasing pressure within the skull.
Diagnosis and Treatment
The condition can be suspected in the presence of signs of intracranial hypertension, such as headache, neck pain, or papilledema, as mentioned in the study 1. Treatment typically involves surgery to separate the fused bones and reshape the skull, ideally performed within the first year of life. The timing and type of surgery depend on which sutures are affected and the severity of the condition.
Management
According to the study published in Pediatrics 1, infants, children, and adolescents with congenital malformations of the skull, including craniosynostosis, should be referred to a pediatric plastic surgeon. Early diagnosis and treatment are crucial to prevent complications such as developmental delays, increased intracranial pressure, and vision problems. Some key points to consider in the management of craniosynostosis include:
- Referral to a pediatric plastic surgeon for treatment
- Surgery to separate the fused bones and reshape the skull
- Early diagnosis and treatment to prevent complications
- Monitoring for signs of intracranial hypertension, such as headache, neck pain, or papilledema.
From the Research
Definition of Craniosynostosis
- Craniosynostosis is a condition characterized by the premature fusion of one or more cranial sutures, resulting in abnormal skull shape and potential increased intracranial pressure 2.
- This condition can occur early in fetal development or postnatally, and its presentation may vary depending on the suture or sutures involved 2, 3.
Types of Craniosynostosis
- Single-suture craniosynostosis involves the premature closure of one cranial suture, while multiple-suture craniosynostosis involves the closure of two or more sutures 3, 2.
- Normocephalic craniosynostosis is a type of craniosynostosis where the skull shape appears normal despite premature suture fusion 4.
Complications of Craniosynostosis
- Increased intracranial pressure is a common complication of craniosynostosis, which can lead to devastating consequences if left untreated 2, 3, 5, 6.
- Other complications include midface hypoplasia, hypertelorism, papilledema, and anterior fontanelle bulge 2, 6.
Diagnosis and Treatment
- Diagnosis of craniosynostosis typically involves clinical and radiological evaluation, including computed tomography scans and optical coherence tomography 5, 4.
- Treatment of craniosynostosis usually involves surgical correction, which can help alleviate increased intracranial pressure and improve symptoms 2, 6.