Craniosynostosis: Symptoms, Treatments and Causes

What is craniofacial stenosis?

Craniofacial stenosis is a poor bone formation ("malformation" in the medical term) due to the absence or premature closure of the cranial and facial sutures (points where the bones meet) accompanied by orbital maxillary hypoplasia (precarious development of the maxilla and eyeball, which is responsible for skipped eyes). It can occur in isolation or associated with more than 70 types of syndromes, the most common being Crouzon and Apert.

The prevalence of the problem over the population is still not well established, but it is estimated that craniofacial stenosis affects one in every 2 thousand children in the world. Males are the most affected, with incidence three times higher than in females.

Causes

As it is a malformation of genetic character, the causes are indeterminate.

Diagnosis of craniofacial stenosis

The abnormality can be discovered by means of a radiological study, x-rays or CT scans with three-dimensional reconstruction. Magnetic resonance imaging may still show signs of brain atrophy or other abnormalities. Bone mapping can also be used to confirm the diagnosis as well as genetic research in some cases. However, once the problem is discovered, nothing can be done to prevent it.

Treatment of craniofacial stenosis

When craniofacial stenosis has impact only on the esthetic part, patient and physician should discuss the need for an early surgical intervention for the child's well-being. In cases where closure of the sutures endangers the child's life or development, the surgical procedure is essential and should be performed as soon as possible.

Possible Complications

Nonoperative surgery can lead to permanent deformities of the cranial cavity and associated facial structures, which include aesthetic, functional and psychosocial deficiencies. Some of them are:

• Microcephaly: when the brain atrophy because it has no room to develop
• Hydrocephalus: dilation of the ventricles and cavities inside the brain by the accumulation of CSF, which hydrates and protects the organ
• Ocular proptosis: displacement of the eyeball.

Living together / Prognosis

Some cases require physiotherapeutic, speech-language and orthodontic follow-up. If it is not linked to any syndrome, the problem is only aesthetic, not affecting the neurological and intellectual development of the individual.