Sphenocephaly

Sphenocephalia (sphenocephalia; from the Greek words sphen - “wedge” and kephale - “head”) is a rare congenital disease characterized by a wedge-shaped deformation of the skull.

With this disease, the child's head has an unusual triangular or wedge-shaped shape due to premature fusion of the cranial sutures. This leads to limited growth of the braincase in the transverse and anteroposterior directions.

Sphenocephaly is often combined with microcephaly (small head size) and mental retardation. Other possible symptoms include delayed psychomotor and speech development, and seizures.

Treatment is mainly symptomatic and supportive. Surgery may be indicated to widen the skull and relieve pressure on the brain. The prognosis depends on the degree of brain damage. In severe forms of the disease, life expectancy can be significantly shortened.