Crouzon syndrome – Craniofacial dysostosis

Crouzon Syndrome (Craniofacial Dysotosis)

What is Crouzon syndrome?

Crouzon syndrome is a genetic disease, leading to abnormal splicing bones of the skull and face.

Infants have the joints between the bones of the face and skull. When the baby's brain grows, These joints allow the skull to expand. Seams completely overgrown when the brain is fully formed and its growth stops.

When Crouzon syndrome and facial bones of the skull fuse too early, skull and then forced to grow in the direction of the remaining open sutures. This leads to an abnormal head shape, face and teeth.

Causes Crouzon syndrome

Crouzon syndrome is caused by a genetic defect. It is not clear, What reasons prompted gene, Crouzon syndrome causes, mutate. Some of the mutated gene can be inherited from the genes of the parents.

Crouzon syndrome Risk Factors

Factors, which can increase the risk of Crouzon syndrome in a child, include:

• Parents with Crouzon syndrome;
• Parents, that do not have this disorder, but which have a gene, calling Crouzon syndrome;
• Adult or old age his father at conception.

The symptoms of Crouzon syndrome

The main signs and symptoms of Crouzon syndrome include:

• Flattened top and back of head;
• Flattened forehead and temples;
• The middle part of the face is small and pull more, than usual;
• Pointed nose;
• Compressed nasal passages, which often leads to a decrease in the flow of air through the nose;
• Greater, protruding lower jaw;
• Shifting teeth;
• Narrow sky, or cleft palate.

Other symptoms and complications, that may arise as a result of Crouzon syndrome include:

• The problems with the development of the inner ear and the hearing loss;
• Meniere's Disease – dizziness or ringing in the ears;
• Eye problems, including eye problems, Strabismus, or involuntary eye movement;
• Rachiocampsis;
• Headache;
• Acanthosis – small, dark, velvety patches of skin;
• Gidrocefaliя – accumulation of fluid in the cranium.

Diagnosis Crouzon syndrome

The doctor usually diagnose Crouzon syndrome based on physical signs and symptoms.

The doctor may need pictures of the skull, spine or arm bones. For their implementation, the following methods:

• Roentgen;
• MRT;
• CT scan.

The doctor may also do genetic testing, to confirm the diagnosis.

Treatment Crouzon syndrome

There is no method to cure Crouzon syndrome. Currently, many of the symptoms can be treated with surgery.

Treating the symptoms of Crouzon syndrome may include:

The operation for the treatment of Crouzon syndrome

There are a number of operations, used to treat the symptoms of Crouzon syndrome. These include:

• Craniectomy – removal and expansion of the skull bones. The operation to be performed as soon as possible after birth, to prevent brain damage. The procedure will also help fix, as much as possible, the shape of the skull;
• The operation for the treatment of protrusion of one or both eyeballs – adjustments are made of bone, surrounding the eye socket;
• The operation to treat protruding lower jaw, to normalize the appearance of the jaw;
• Operation of izlecheniyu volchyey pastes.

Orthodontic treatment Crouzon syndrome

Orthodontic treatment can help correct crooked teeth. It may include the installation of braces to align the bite.

Treatment of vision and hearing in children with Crouzon syndrome

Infants and children with Crouzon syndrome should be kept under the supervision of a specialist, which monitors the state of their hearing and vision, and in case of problems following treatment.

Supportive care of children with Crouzon syndrome

Children with learning disabilities or mental retardation, perhaps, need special assistance in training.

Prevention Crouzon syndrome

There is no way to prevent Crouzon syndrome. If you have Crouzon syndrome, or a family history of the disease, you can talk to a genetic counselor when deciding to have children.