Isolated Craniosynostosis Treatment and Care
The treatment goals for patients with craniosynostosis are to increase the size of the skull to make room for the growing brain and to restore a normal head shape. We use many different surgeries and other treatments to achieve these goals. At Children’s Health we use several of the newer surgeries for skull reshaping such as:
- Extended strip craniectomy with helmet therapy
- Spring-assisted cranial expansion
- Cranial vault distraction
We also use traditional open cranial vault procedures such as Fronto-Orbital Advancement (FOA) and open cranial vault remodeling.
The differences between these various surgeries are described below. Different procedures may be recommended for patients with the same suture affected. We base our recommendation for the type of procedure based on what is safest for the patient while providing optimal results in function and appearance.
Extended Strip Craniectomy with Helmet Therapy: This procedure is primarily used to treat sagittal suture craniosynostosis, however it can also be used to treat other closed sutures. The ideal age for this procedure is age 3-4 months. The benefits of this procedure include:
- Less scarring in the scalp
- Less blood loss and need for blood transfusion
- Shorter hospitalization
In sagittal suture craniosynostosis, the skull is long and narrow from front to back. This surgery uses two incisions of about an inch and a half width in the scalp to see the skull bones. Through these incisions, a wide strip of skull, including the closed sagittal suture, is removed. Cuts are made in the bone just behind the coronal sutures and just in front of the lambdoid sutures. This allows the skull bones to hinge away from one another and widen like a clamshell opening.
Infants’ bones heal rapidly and new bone grows, even though a large gap is made in the top of the skull with this surgery. The bone gap is created to give the brain enough time to reshape the skull before the bone heals.
After surgery, we place the baby in a custom-fit molding helmet. This helmet fits up against the forehead and back of the head and does not apply pressure. Instead, it resists growth in these directions. The skull then grows to a more normal shape because the brain takes the path of least resistance and widens pushing the bones as it expands.
A specially trained professional will adjust the helmet regularly to allow for growth. We typically keep our patients in the helmet until 18 months of age.
Spring-Assisted Cranial Expansion: This surgery is most commonly used to treat sagittal suture craniosynostosis. It may be used to treat other closed sutures as well. The ideal age for this procedure is age 3-6 months. The benefits of this procedure when compared to open cranial vault procedures include:
- Less scarring in the scalp
- Less blood loss and risk for blood transfusion
- Shorter hospital stay
It is best used at younger ages when the bones in the head are soft and the scalp is thin allowing more effective reshaping of the skull bones. The surgeon removes the closed suture along with a thin strip of adjacent bone. Then, the surgeon places two to three springs near the edges. In this surgery, the expansion of the spring encourages a crossways expansion of the skull. Unlike extended strip with postoperative helmet, this surgery requires a second operation for removal of the springs. The springs are removed two to three months after placement.
Cranial Vault Distraction Osteogenesis
The goal of distraction osteogenesis (DO) is to make bones longer. The surgery uses the body’s ability to heal bones to generate new bone where it is needed.
The general idea is that a cut is made through the bone and a special device called a distractor is applied to the bone and the skin is closed. After a brief time allowing healing to of the bone to begin, the cut ends are gradually separated from one by turning the distractor. New bone tissue forms between the cut ends as they are moved away from one another. The ends are separated to the desired length and then distraction is stopped and the new bone tissue matures and hardens.
DO in the cranial vault has several advantages over single-stage reconstructive procedures. These benefits include:
- Production of new bone
- Gradual stretching of the skin and muscle over the bone
- Larger increases of the space inside the skull for the brain to grow into.
- Greater overcorrection in head shape
The gradual stretching of the scalp allows greater movements of the skull bones to be achieved and maintained compared to operations that expand the skull in one step. In our experience, DO provides twice as much expansion of the space inside the skull as single-stage operations. DO does have its disadvantages as well. These include:
- A brief second operation to remove the distraction devices
- Risk of breakage of the device
- Infection of the skin at the device site.
- Overall treatment tends to take longer, eight to 12 weeks.
Fronto-Orbital Advancement (FOA)
Fronto-orbital advancement has been used to treat craniosynostosis for decades. The surgical goals of a FOA are to expand the space inside the skull and to reshape the forehead and upper part of the eye socket (orbit). In some cases this can protect the eyes by improving the eyelid position and improves appearance. FOA can be performed in many different ways, because this surgery is used for patients with craniosynostosis affecting either the metopic, one or both coronal sutures. It is a surgery that is used in both syndromic and non-syndromic patients. This surgery tends to have better, more lasting effects in non-syndromic patients because the remainder of their bones grow at a normal rate.
In these surgeries, doctors open the scalp to expose closed cranial sutures. Doctors then cut out these sutures to allow the brain to expand and push the skull bones out. This is one of the oldest types of surgeries for craniosynostois, but we use it rarely because long-term results are not desirable. Today, we only use this surgery in very young patients (birth to 4 months old) with a fused suture or signs of increased pressure on the brain. This is mostly done in patients with syndromic craniosynostosis. In these cases, patients can benefit from removing of the closed sutures to let the brain and skull to expand. Typically, another surgery is done to ensure the patient's head maintains a healthy shape, around ages 6-12 months, after thickening of the skull has occurred.
Open Cranial Vault Remodeling Procedures
These surgeries are the oldest and most common procedures used to treat craniosynostosis. The key feature of these types of surgeries is that they require complete removal of the skull bones in order to reshape the head in the area of deformity. When the bone is completely removed it also removes the blood supply from the bones that may affect the growth of the bone and slightly increases the risk of the bone becoming infected when compared to procedures that leave the bones attached to the outer covering of the brain such as extended strip craniectomy. Open cranial vault procedures are typically performed between 6-12 months of age. This is the time period when the bones are still flexible enough that they can be easily reshaped by hand but they are also strong enough to hold the resorbabe plates and screws The goal is to place the bones in a position that will provide an even appearance to the skull and face a child is fully grown.
Open cranial vault surgeries take longer because there is more work required in reshaping and fixing of the skull bones into their new positions. Most patients require a blood transfusion.