Trigonocephaly

Trigonocephaly

Illustration of trigonocephaly

Trigonocephaly is a type of craniosynostosis characterized by the premature fusion of the metopic suture, which is located at the forehead. This condition results in a triangular shape of the forehead, giving the head a keel-like appearance. The term "trigonocephaly" is derived from the Greek words "trigonon" meaning triangle and "kephale" meaning head.

Pathophysiology

Diagram showing the triangular shape of the forehead

The metopic suture is one of the major sutures in the skull that allows for growth of the brain and skull during infancy and early childhood. In trigonocephaly, the premature closure of this suture restricts the growth of the frontal bones, leading to a characteristic triangular forehead. This can also result in hypotelorism, where the eyes are closer together than normal.

Clinical Features

Clinical image of a patient with trigonocephaly

Patients with trigonocephaly often present with a prominent ridge along the metopic suture, a narrow forehead, and closely spaced eyes. The condition can vary in severity, with some individuals experiencing only mild cosmetic deformities, while others may have significant craniofacial abnormalities that can affect cognitive development and neurological function.

Diagnosis

Diagnosis of trigonocephaly is typically made through clinical examination and confirmed with imaging studies such as CT scans or MRIs. These imaging techniques allow for detailed visualization of the skull sutures and can help in assessing the extent of suture fusion and any associated cranial deformities.

Treatment

Image depicting single suture synostosis

The primary treatment for trigonocephaly is surgical intervention, which is usually performed in infancy to allow for normal brain and skull growth. The surgery involves the removal of the fused suture and reshaping of the forehead and orbital region to achieve a more typical cranial shape. Early intervention is crucial to prevent potential complications such as increased intracranial pressure and developmental delays.

Prognosis

With timely surgical treatment, many children with trigonocephaly can achieve normal cognitive and physical development. However, the outcome can vary depending on the severity of the condition and the presence of any associated syndromes or complications.

Related Pages

• Craniosynostosis
• Metopic suture
• Hypotelorism
• Neurosurgery