What is Crouzon's syndrome?

initially described by the French neurologist Octave Crouzon in 1912, Crouzon's syndrome is a rare genetic deformity including premature closure of the coronal skull stitch. Characteristics of this condition include sloping lids down; wide set, convex eyes; and insufficiently developed upper jaw. In the United States, Crouzon's syndrome occurs once every 25,000 births.

also called craniophial dystosis, Crouzon's syndrome is similar to Apert syndrome that affects hands and legs, as well as the skull and face. While many physical malformations associated with apes are not expected in the patient Crouzon, both conditions are believed to have a similar genetic origin. In Crouzon's syndrome, the skull deformity is the same appearance as Apert syndrome; With Crouzon, however, there is no fusion of fingers and feet.

Crouzone syndrome patients generally share many same physical features, although the severity will differ the wkaždý individual. The forehead grows higher instead of, andwould grow forward because of closing the bones of the skull. In addition, because the bones between the eyes and the upper teeth are smaller than usual, the affected Crouzon syndrome seems to have large, convex eyes. In this state, it is also a dominant feature of a curved parrot.

Patients with this disease usually have normal intelligence, although some mental delay is possible. Breathing problems and abnormal speech patterns are common due to the smaller nose present. The cleft palate is sometimes associated with Crouzone syndrome and can cause hearing problems due to frequent ear infections.

Like most other syndromal craniosynosostoses, Crouzon's syndrome is considered to be caused by gene deviation. This is a very rare and scattered event. Crouzon patients have a 50% chance of handing over Dedorder to their children. However, if both parents are not affected by this disease, the probability of developing in other children isVery small.

Physical examination is most often used to diagnose Crouzon's syndrome because it is present at birth. Computer tomography (CT) Scanning, magnetic resonance imaging (MRI) and X -rays are also useful diagnostic tools. In addition, other ophthalmological, audiological and psychological testing can be used.

Treatment

Crouzon syndrome is extensive and complex and is best provided by an experienced craniophial team in one of several main health centers around the world. The typical treatment plan for this disease includes several staged operations. It is recommended that the Synostotic Skull Stites to relax in the first year of life to allow the brain to be properly growing. As the child ripens, the skull is often transformed to bring optimal results.