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Houston Cranio Facial

Craniosynostosis and Cranial Vault Reconstruction

What Is Craniosynostosis?

Craniosynostosis is a premature fusion of the bones that make up the skull. The skull is made up of several bones with gaps between the bones called sutures or bony joints. These sutures are the connections between the bones and are the growth centers of the skull. It is easier to understand as the bones grow from the edges rather than the center of the bones. Premature fusion of the sutures can lead to a number of conditions that affect intellectual development and quality of life, so diagnosis and treatment is essential. Dr. Eric Payne is an experienced craniofacial plastic surgeon with extensive training in craniosynostosis diagnosis and treatment. Dr. Payne and our entire team are dedicated to helping patients and their families through each stage of care with compassion and understanding.

When Does Craniosynostosis Develop?

Craniosynostosis occurs while a child is developing in the womb. When these edges of the bone at the sutures fuse together, then the two bones become one bone and can only continue to grow at the edges. This restricts skull growth in certain three-dimensional directions and causes an abnormal head shape with distortion of facial features. Sometimes this can also cause increased intracranial pressure which can lead to brain damage or blindness.

What Causes Craniosynostosis?

To date, there has not been one specific cause identified. Some animal studies done suggest intrauterine constraint may lead to premature cranial suture (bony joint) closure. That means if the child becomes stuck in a certain position in the womb this could constrain the child’s head preventing growth and lead to premature suture closure. It has also been suggested that the incidence of single suture closure is higher in boys because of the male sex hormone testosterone. The theory is testosterone increases the “stickiness” of cranial sutures which encourages them to close prematurely.

How Is Craniosynostosis Diagnosed?

Evaluation of your child’s head shape is predictive of craniosynostosis in 99% of cases. If one of the sutures (skull joints) fuses closed, the other sutures (joints) try to compensate for the loss of growth in this one area. The other sutures’ compensation lead to a three-dimensional head shape consistent with a particular suture fusion. As your child grows, this head shape becomes more characteristic of craniosynostosis. Your child would need to be examined by a craniofacial surgeon to help confirm the diagnosis.

Typically, a CT scan of the head is not always necessary. The diagnosis is usually determined on physical exam alone. A CT scan is helpful if more than a single suture is suspected to be fused. This will help in the planning surgical reconstruction of the skull. An MRI is useful if Arnold Chiari Malformation (brain herniation at the base of the skull to the spinal cord) is suspected, which can occur in lambdoid craniosynostosis.

What Are the Types of Craniosynostosis?

Typically, only one suture is affected, and this is known as Single Suture Craniosynostosis. This is usually not an inherited genetic condition but a spontaneous occurrence. The incidence of single suture craniosynostosis is around 1 in every 2,000 babies born. When more than one suture is involved then we consider this a Multiple Suture Craniosynostosis, which has a higher chance of having a genetic cause. This is more frequently seen in Syndromic Craniosynostosis such as Apert Syndrome, Pfeiffer Syndrome, or Crouzon Syndrome. With these conditions, a geneticist is involved to help guide you through what to expect and determine the cause. The types of craniosynostosis are:

Sagittal Craniosynostosis

The most commonly occurring form of craniosynostosis, sagittal craniosynostosis is the premature fusion of the sagittal suture, which is a suture that runs vertically along the top of the skull. If this suture fuses too soon, the head will expand lengthwise, creating an elongated shape.

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Metopic Craniosynostosis

About 25 percent of craniosynostosis patients have metopic craniosynostosis, which is the premature fusion of the suture located between the two frontal bones that form to create the forehead. This can cause a pinched or narrow forehead and enlarged back of the head, creating a triangular shape.

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Coronal Craniosynostosis

Two coronal sutures run from ear to ear meeting across the top of the head where the anterior fontanelle, or soft spot, is situated. Some patients may have single suture coronal craniosynostosis, some may have both. In a unilateral (single) suture fusion, the opposite side will bulge. In a bilateral fusion, the head shape will appear tall with flattening of the forehead.

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Lambdoid Craniosynostosis

The rarest form of craniosynostosis, and one that can be mistaken for deformational plagiocephaly, lambdoid craniosynostosis involves the two sutures located at the back of the skull. Unilateral fusion creates a tilted effect on the skull while bilateral fusion, which is extremely uncommon, causes a tall skull effect and flattening of the back of the head.

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What Craniosynostosis Treatments Are Available?

In all but the mildest of cases, craniosynostosis will require at least one surgical operation. Surgical treatment for craniosynostosis is called cranial vault remodeling or cranial vault reconstruction. The cranial vault is the space inside the skull where the brain resides. It is sometimes called the skullcap, though that more commonly refers to the top of the skull. The surgical technique used for craniosynostosis treatment will depend on the sutures affected, what other conditions are present, the age of the child, and other factors unique to the patient. Most individuals will require a team of medical professionals to address the variety of concerns that can develop from craniosynostosis. The interdisciplinary team involved with treating craniosynostosis includes the following:

  • Craniofacial Plastic Surgeon
  • Pediatric Neurosurgeon
  • Pediatric Ophthalmologist
  • Geneticist
  • Pediatric Anesthesiologist
  • Pediatric Intensive Care Specialist

How Much Does Cranial Vault Remodeling Cost?

Cranial vault reconstruction should be covered by your medical insurance policy, and we can help you submit paperwork and the necessary information to receive approval for your child’s operation. If you are working outside of the medical insurance system, cranial vault remodeling fees will vary depending on the sutures involved, the complexity of the treatment, whether other conditions are in play and require treatment, and other indirect costs. Our team will discuss all these factors with you and assist you as much as possible to ensure you receive the best care.

For more information about craniosynostosis and cranial vault remodeling, please contact our practice to schedule a consultation with Dr. Payne.