Wednesday, July 17, 2013

Review of Craniosynostosis and Our Story

For those who are just finding this page or need a summary of what steps we went through to get to where we are today, here is a review...

May 29th 2013 - Referred to neurologist

June 11th 2013 - 1st opinion

June 18th 2013 - 2nd opinion

June 21st 2013 - fight for endoscopic surgery

July 8th 2013 - Hope - 3rd opinion

July 10th 2013 - Surgery is scheduled

July 12th 2013 - Surgery

What does Alice have?

 Left Coronal Craniosynostosis

Picture of Alice's skull
You can see on the right side of this picture, the suture is fused.
In the endoscopic surgery they opened this suture/recreated the suture

What Is Craniosynostosis?

The normal skull consists of several plates of bone that are separated by sutures. The sutures (fibrous joints) are found between the bony plates in the head. The function of the suture is to allow molding through the birth canal and adjustments for the growing brain. As the infant grows and develops, the sutures close, forming a solid piece of bone, called the skull.

Craniosynostosis is a condition in which the sutures close prematurely, causing problems with normal brain and skull growth and premature closure of the fontanelles (soft spots). Premature closure of the sutures may also cause the pressure inside of the head to increase and the skull or facial bones to change from a normal, symmetrical appearance.

What are the symptoms? The most common sign of craniosynostosis is an oddly shaped head at birth or by the time the child is a few months old. For example, the skull may become long and narrow. Or it may be very flat and broad in front or back or on the sides. This oddly shaped head may be the only sign of craniosynostosis.

In rare cases, the deformity causes pressure to build up on the baby’s brain. This can cause brain damage and can make the baby develop more slowly than other children.

The treatment of craniosynostosis requires surgical correction. Craniosynostosis occurs in one in 2000 births.

The different types:

Sagittal Craniosynostosis

Sagittal Craniosynostosis is the most common type of Cranioysnostosis (40%-58% of all cranio cases) and effects more males than females. Early closure of the sagittal suture results in scaphocephaly. Infants will often have a protruding forehead, an elongated head, and protruding at the back of the skull.

Coronal Craniosynostosis

Coronal Craniosynostosis is the second most common form of Craniosynostosis and effects mostly females. Coronal synostosis may occur on either side or may be bilateral. Infant will often have an elevation of the eye socket, flattening of the ridge of the eye and displacement of the nose on the affected side.

Metopic Craniosynostosis

Metopic synostosis is the third most common form of Craniosynostosis and is associated with trigonocephaly with a
male preponderance. The metopic suture is located between the soft spot and the nose. Early closure of this suture results in trigoncephaly. Infants will often have a prominent ridge down the middle of the forehead, a pointed forehead, and eyes that seem too close together.

Lambdoid Craniosynostosis

Lambdoid Craniosynostosis is one of the most rare type of Craniosynostosis. Most cases of lambdoid craniosynostosis
are unilateral and result in asymmetric posterior
plagiocephaly that needs to be differentiated from positional

Frontosphenoidal Craniosynostosis

Frontosphenoidal synostosis is one of the most rare types of Craniosynostosis. It is the small suture close to the eye socket. Premature closure of this suture will result in flattening on the side of the front of the head and can cause an indentation near the temple or eyebrow bones. Frontosphenoidal appears almost exactly like coronal craniosynostosis but the coronal sutures are open. This is believed to be one of the most undiagnosed forms of cranio as a child can appear to have coronal synostosis or positional plagiocephaly but the CT scans will show the sutures are not fused and the child will not be diagnosed correctly.

Multiple Suture Craniosynostosis

Craniosynostosis of multiple sutures accounts for approximately 5% of craniosynostosis. A child can have two or more sutures fused prematurely and is a more complex form of craniosynostosis. Complex craniosynostosis frequently causes increased inter cranial pressure and is associated with developmental delay and a high rate of re-operation.

Positional Plagiocephaly (Flat head syndrome)

Plagiocephaly (PLAY-gee-oh-SEF-uh-lee) is a flat spot on the back or side of a baby’s head. It is caused by pressure on the bones of the skull before or after birth. This usually happens because of the way a baby likes to lie (their positional preference) in their first few months of life.

There are many things that could cause positional plagiocephaly.

Children with multiple medical problems or delayed development may have a hard time changing positions. They are more likely to develop positional plagiocephaly than children who can move around more.

Some infants have a strong preference to look in one direction. This can lead to a limited range of motion in the neck from muscles being tight. This is called torticollis. Babies with torticollis might be more likely to develop plagiocephaly than other babies, since they always rest on the same spot on the back of their head.

Premature infants are sometimes more likely to develop plagiocephaly. This is because their skull bones are softer than the skulls of babies born at full term. They also tend to move their heads less often.

Positional Plagiocephaly does not require surgery and can usually be fixed by repositioning of the babies head while they lay down or sleep and some may require the use of a helmet.


For most infants, however, surgery is the primary treatment for craniosynostosis. The type and timing of surgery depend on the type of craniosynostosis and whether there's an underlying syndrome.

The purpose of surgery is to relieve pressure on the brain, create room for the brain to grow normally and improve your child's appearance. A team that includes a specialist in surgery of the head and face (craniofacial surgeon) and a specialist in brain surgery (neurosurgeon) often performs the procedure.

Traditional surgery

The surgeon makes an incision in your infant's scalp and cranial bones, then reshapes the affected portion of the skull. Sometimes plates and screws, often made of material that is absorbed over time, are used to hold the bones in place. Surgery, which is performed during general anesthesia, usually takes hours.

After surgery, your infant remains in the hospital for at least three days. Some children may require a second surgery later because the craniosynostosis recurs. Also, children with facial deformities often require future surgeries to reshape their faces.

Endoscopic surgery

This less invasive form of surgery isn't an option for everyone. But in certain cases, the surgeon may use a lighted tube (endoscope) inserted through one or two small scalp incisions over the affected suture. The surgeon then opens the suture to enable your baby's brain to grow normally. Endoscopic surgery usually takes about an hour, causes less swelling and blood loss, and shortens the hospital stay, often to one day after surgery.

If your baby has an underlying syndrome, your doctor may recommend regular follow-up visits after surgery to monitor head growth and check for increased intracranial pressure. Head growth will be routinely monitored at well-child visits.

The day we came home from the hospital.
Two days after her endoscopic surgery
WOW! What an improvement!