craniofacial

Bicoronal Craniosynostosis

What is Bicoronal Craniosynostosis?

Bicoronal craniosynostosis is a condition in which both coronal sutures in an infant’s skull close prematurely. The coronal sutures run from ear to ear over the top of the head, and their early fusion restricts the skull’s front-to-back growth. This results in a condition known as brachycephaly, where the head appears short and broad. In severe cases, the forehead may appear tall and flat, and the orbits (eye sockets) may also become shallow, sometimes leading to bulging eyes (proptosis).

The Procedure

How is it Diagnosed?

Diagnosis of bicoronal craniosynostosis typically begins with clinical examination. A noticeable change in head shape, especially a broad and shortened skull with a flat or tall forehead, raises suspicion. Medical professionals will also feel for ridges along the coronal sutures. Imaging studies such as CT scans with 3D reconstruction are used to confirm the premature fusion of both coronal sutures and evaluate the overall cranial structure. Additional assessments may be done to check for signs of increased intracranial pressure or syndromic features.

What Are the Causes?

Bicoronal craniosynostosis can be either non-syndromic or syndromic. Non-syndromic cases occur sporadically with no identifiable genetic cause. Syndromic cases are associated with genetic disorders such as Apert syndrome, Crouzon syndrome, Muenke Syndrome and Pfeiffer syndrome. These syndromes may involve other craniofacial, limb, or neurological abnormalities. Genetic testing is typically recommended in cases where a syndrome is suspected or other anomalies are present.

What Are the Implications?

The implications of bicoronal craniosynostosis can be significant. The restricted growth of the skull can lead to increased intracranial pressure,

Cosmetic concerns are also a major issue for many families, as the distinct skull shape can impact a child’s self-esteem and social interactions. In syndromic cases, additional physical and developmental challenges may arise, necessitating long-term multidisciplinary care.

What Are the Treatment Options?

Surgical treatment may be necessary to correct the skull shape and prevent or alleviate increased intracranial pressure. Common surgical approaches include:

Cranial Vault Expansion often by spring assisted cranioplasty is usually undertaken in early life to correct the head shape. cranial vault expansion often by spring. Assisted cranioplasty is usually undertaken in early life to correct the head shape.
Endoscopic-Assisted Surgery: In selected cases diagnosed early (typically under 6 months), a minimally invasive surgery may be done to release the fused sutures, followed by helmet therapy to guide skull shape.

What Are the Possible Complications?

Potential complications from surgery include:

Infection or bleeding
Anesthesia risks
Residual or recurrent cranial deformities
Need for revision surgeries
Increased intracranial pressure if untreated or inadequately treated

With timely diagnosis and skilled surgical care, many children with bicoronal craniosynostosis experience improved skull shape, brain growth, and quality of life. Long-term follow-up is often required to monitor development and address any additional concerns.