Surgical treatment of craniosynostoses and congenital facial and skull abnormalities

Craniosynostosis is a birth defect in which one or more sutures between the bones of your child’s skull close prematurely-before your child’s brain is fully formed.

Brain growth continues, giving the head a deformed appearance. Typically, a child has an anterior fontanel-you feel a soft spot just behind your baby’s forehead.

The next largest fontanel.is the posterior one, located at the back of the head. Each side of the skull has tiny fontanelles near the temples.

Craniosynostosis often affects a single cranial suture, but in some cases, multiple sutures can be involved.

In rare cases, craniosynostosis is caused by certain genetic syndromes.

Treatment includes surgery to correct the shape of the skull. Early diagnosis and timely medical intervention provide enough space for the brain to grow and develop. Although neurological damage can occur in severe cases, most children have normal cognitive development and achieve favorable cosmetic outcomes after surgery.

Early diagnosis and treatment are key.

Symptoms:

Signs of craniosynostosis are usually noticeable at birth, but they become more apparent during the first few months of life. The signs and severity depend on how many sutures are fused and at what stage the fusion occurred during development.

These may include:

A deformed skull, with a shape depending on which sutures is affected.

Poor palpation or disappearance of the fontanel on your child’s skull.

A raised, hard ridge along the fused sutures.

Slow head growth.

There are several types of craniosynostosis. Most of them are associated with the fusion of a single cranial suture. Some complex shapes involve the fusion of multiple seams. Most cases of multiple suture craniosynostosis are associated with genetic syndromes and are called craniosynostosis syndrome. The term given to each type depends on which sutures are affected.

Types of craniosynostosis include:

Scaphocephaly

Premature fusion of the sagittal suture, which runs from the front to the back at the top of the skull, causes the head to grow long and narrow. Sagittal craniosynostosis leads to a form called scaphocephaly and is the most common type of craniosynostosis.

Plagiocephaly

Premature fusion of one of the coronal sutures (unicoronal). These sutures run from each ear to the top of the skull. It can lead to a flattened forehead on the affected side and a bulge on the healthy side. It also leads to a deviation of the nose and a raised eye socket on the affected side.

When both coronal sutures merge prematurely (bicoronal synostosis), the head develops a short and wide appearance, often with a forward tilt of the forehead.

Trigonocephaly

Early fusion of the metopic suture. It runs from the top of the bridge of the nose

up through the middle of the forehead to the anterior fontanel and to the sagittal suture.

Its premature fusion gives the forehead a triangular appearance and may cause narrowing at the temples.

Lambdoid.

Lambdoid synostosis is a rare type that involves the lambdoid suture, located at the back of the skull. This can cause one side of your child’s head to appear flat, and one ear to appear higher than the other with the top of the head tilted to one side.

Other causes of head deformity:

A deformed head does not always indicate craniosynostosis. For example, if the back of the head appears to be flattened, this may be the result of the child lying on one side for a long time. Positional plagiocephaly. This can be treated with regular changes in position, or with the use of a special helmet (cranial orthosis).

Often the cause of craniosynostosis is not known, but sometimes it is associated with genetic disorders.

Nonsyndromic craniosynostosis is the most common type, and its cause is unknown, although it is believed to be a combination of genetic factors and the environment. Syndromic is caused by certain genetic syndromes, such as Apert, Pfeiffer, or Crouzon, which can affect the development of the skull. These syndromes usually also include other physical features and health problems.

In the absence of medical intervention, craniosynostosis can lead to permanent deformity of the head and face, poor self-esteem, and social isolation in the future. There is a risk of increased intracranial pressure in simple craniosynostosis, this risk is generally low if treated promptly and the head shape is monitored surgically.

Without proper treatment, increased intracranial pressure can lead to:

• Development delay.
• Cognitive impairment.
• Lethargy.
• Blindness.
• Eye movement disorders.
• Seizures
• And in rare cases, death.