Craniosynostosis

Alternative Names:Premature closure of sutures

Definition:
To allow for rapid brain growth, your baby’s skull cap is made up of five bones, held together by a fibrous-like material called “sutures”. Normally, they remain open as long as the brain grows, which gives the brain room needed to grow in all directions.

If any of the sutures close or fuse before the brain has finished growing, the condition known as craniosynostosis occurs. Other terms used to describe this problem include: synostosis, or cranial stenosis. Premature closure can take place before birth or at any time until the brain stops growing.

Because the brain is growing normally, it will take the path of least resistance which will result in a misshapen head or face.
The skull of an infant is made up of free-floating bones separated by fibers called sutures. This arrangement allows the infant’s head to pass through the birth canal and also enables the skull to grow with the brain in early infancy. Craniosynostosis is a birth defect of the brain characterized by the premature closure of one or more of the fibrous joints between the bones of the skull (called the cranial sutures) before brain growth is complete.Closure of a single suture is most common.

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Skull of a newborn

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The abnormally shaped skull that results is due to the brain not being able to grow in its natural shape because of the closure. Instead it compensates with growth in areas of the skull where the cranial sutures have not yet closed. The condition can be gene-linked, or caused by metabolic diseases, such as rickets or an overactive thyroid. Some cases are associated with other disorders such as microcephaly (abnormally small head) and hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain).

Causes :
The cause of craniosynostosis is unknown. Which suture is involved determines the abnormal shape of the head.

A person’s genes may play a role in craniosynostosis. The hereditary form often occurs with other defects that can cause seizures, diminished intellectual capacity, and blindness. Genetic disorders commonly associated with craniosynostosis include Crouzon, Apert, Carpenter, Chotzen, and Pfeiffer syndromes.

However, most cases of craniosynostosis occur in a family with no history of the condition and children with craniosynostosis are otherwise healthy and have normal intelligence.

There are different types of craniosynostosis. Sagittal synostosis (scaphocephaly) is the most common type. It affects the main (sagittal) suture on the very top of the head. The early closing forces the head to grow long and narrow, instead of wide. Babies with this type of craniosynostosis tend to have a broad forehead. It is more common in boys than girls.

Frontal plagiocephaly is the next most common form. It is the closure of one side of the suture that runs from ear to ear on the top of the head. It is more common in girls.

Metopic synostosis is a rare form of craniosynostosis that affects the suture close to the forehead. The child’s head shape may be described as trigonocephaly, and the deformity may range from mild to severe.

Symptoms :

The first sign of craniosynostosis is changes in the shape of the head and face . Other features can include :-

*a full or bulging fontanelle (soft spot located on the top of the head)

*sleepiness (or less alert than usual)

*scalp veins may be very noticeable

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*increased irritability

*high-pitched cry

*poor feeding

*projectile vomiting

*increasing head circumference

*seizures

*bulging eyes and an inability of the child to look upward with the head facing forward

*developmental delays

Diagnosis:
Craniosynostosis may be congenital (present at birth) or may be observed later, during a physical examination. The diagnosis is made after a thorough physical examination and after diagnostic testing. During the examination, a measurement of the circumference of your child’s head is taken and plotted on a graph to identify normal and abnormal ranges. In addition, the following tests may be ordered:

x-rays of the head – a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues and bones of the head onto film.
computerized tomography scan (Also called a CT or CAT scan.) of the head – a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the head.

Treatment:

Treatment for craniosynostosis generally consists of surgery to relieve pressure on the brain and the cranial nerves. The goal of treatment is to reduce the pressure in the head and correct the deformities of the face and skull bones. The optimal time to perform surgery is before the child is 1 year of age since the bones are still very soft and easy to work with. Surgery may be necessary at a much earlier age depending upon the severity of the condition. For some children with less severe problems, cranial molds can reshape the skull to accommodate brain growth and improve the appearance of the head.

Possible Complications :

Craniosynostosis results in head deformity that can be severe and permanent if it is not corrected. Increased intracranial pressure, seizures, and developmental delay can occur.

Prognosis:
The prognosis for craniosynostosis varies depending on whether single or multiple cranial sutures are involved or other abnormalities are present. The prognosis is better for those with single suture involvement and no associated abnormalities. The key to treating craniosynostosis is early detection and treatment. Some forms of craniosynostosis can affect the brain and development of a child. The degree of the problems is dependent on the severity of the craniosynostosis, the number of sutures that are fused, and the presence of brain or other organ system problems that could affect the child.

Prevention:
Be sure to bring your child to well-child visits, so your pediatrician can routinely chart the growth of your infant’s head over time. This will help identify the problem early if it occurs.Persons with hereditary craniosynostosis might consider genetic counseling.

When to Contact a Medical Professional:
Call your health care provider if you think your child’s head has an unusual shape. A referral to a pediatric neurologist or neurosurgeon should follow.
Click to see also:->
Abnormal Head Shapes in Infants
My Baby’s Head is Flat!

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
KidsGrowth.com
http://www.nlm.nih.gov/medlineplus/ency/article/001590.htm
http://www.craniosynostosis.net/what-is-craniosynostosis.shtml

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