Craniosynostosis

A condition in which one or more of the fibrous sutures in an infant skull prematurely fuses.

Craniosynostosis is a congenital condition characterized by the premature fusion of one or more of the cranial sutures, the fibrous joints between the bones of the skull. This early fusion restricts the growth of the skull and can lead to an abnormal head shape, increased intracranial pressure, and potential developmental delays.

Pathophysiology

The human skull is composed of several bones that are separated by sutures. These sutures allow the skull to expand as the brain grows during infancy and childhood. In craniosynostosis, one or more of these sutures close prematurely, which can affect the shape of the head and the growth of the brain.

Types of Craniosynostosis

Craniosynostosis can be classified based on the specific sutures that are affected:

• Sagittal synostosis: The most common form, where the sagittal suture fuses, leading to a long, narrow head shape known as scaphocephaly.
• Coronal synostosis: Premature fusion of one or both coronal sutures, resulting in a short, wide head shape called brachycephaly if bilateral, or plagiocephaly if unilateral.
• Metopic synostosis: Fusion of the metopic suture, causing a triangular forehead and a condition known as trigonocephaly.
• Lambdoid synostosis: The rarest form, involving the lambdoid suture, leading to a flat head shape at the back.

Clinical Presentation

The clinical presentation of craniosynostosis varies depending on the sutures involved. Common signs include:

• Abnormal head shape
• Asymmetry of the face
• Developmental delays
• Increased intracranial pressure, which can cause irritability, vomiting, and visual disturbances

Diagnosis

Diagnosis of craniosynostosis is typically made through a combination of physical examination and imaging studies. Computed tomography (CT) scans are often used to confirm the diagnosis and to assess the extent of suture fusion.

Management

The primary treatment for craniosynostosis is surgical intervention. The goals of surgery are to correct the shape of the skull, relieve any increased intracranial pressure, and allow for normal brain growth. Surgical options include:

• Cranial vault remodeling: Reshaping the bones of the skull to allow for normal growth.
• Endoscopic strip craniectomy: A minimally invasive procedure that involves removing the fused suture to allow the skull to expand.

Prognosis

With early diagnosis and appropriate surgical treatment, most children with craniosynostosis can achieve normal brain development and head shape. However, ongoing monitoring is essential to manage any potential complications.

Related pages

• Skull
• Suture (joint)
• Congenital disorder
• Neurosurgery