Currently, the only definitive available treatment for craniosynostosis is surgery. First done in the 1890’s, many procedures have been developed over the years. Since the 1970’s the most common surgical approach involves a number of techniques that fall under the category of Calvarial Vault Remodel or CVR. Depending on the affected suture, the operation will vary but commonly involves several common steps:
- Wide exposure of the skull
- Removal of large sections of bone
- Reshaping of the deformed bone
- Replacing and fixation of removed bone to skull to obtain a normal head shape.
Calvarial Vault Remodel (CVR)
CVR is a term used to treat patients with craniosynostosis by removing sections of the skull and reshaping them to resemble a normal skull shape. The specific technical details of each procedure will vary from surgeon to surgeon and center to center. However, there are some basic underlying principles associated with the treatment of each suture. The CVR needs to be done in children older than 10 or 12 months of age depending on the severity of the child’s deformity. These procedures produce immediate excellent results which tend to deteriorate over time. These procedures are associated with long surgical times (3-9 hours), universal blood transfusions, large amounts of blood and other blood product transfusions, significant postoperative swelling, pain and discomfort as well as longer hospital stays (4-7 days).
Sagittal Synostosis CVR
Depending on the surgical center, parts or all of the front, middle and back of the baby’s skull will be removed and reshaped. The overall goal is to decrease the distance between the front and the back of the head and at the same time increase the width of the head. A. number of anchoring materials are used in order to keep the bones of the skull form shifting and moving out of place. These include absorbable plates and screws, absorbable and non-absorbable sutures, wires and mini titanium plates and screws. In order to access the entire skull, a scalp incision is made that extends from ear to ear and the scalp is mobilized front and back. Most centers will leave drains under the scalp for several days after the surgery.
Metopic Synostosis CVR
Also known as a fronto-orbital advancement (FOA), The CVR approach to the coronal suture synostosis includes exposure to the entire forehead. Similarly, an ear to ear scalp incision is made as to not cause facial and visible scars. (Bicoronal Incision) The surgeons then remove both frontal bones as well as a rim of bone that contains the upper edges of the bony orbits (orbital bandeau). With this condition, the bandeau has a pointed and triangular shape. Once removed, the bandeau is reshaped into a rounded, normal forehead shape. The frontal bones are also reshaped, replaced and repositioned with the selected anchoring materials. One problem with this type of operation is that normal forward skull growth can be restricted by the anchoring materials which often leads to a tall and high (turricephaly) forehead and skull.
Figure 1. Top view of the head of a child with metopic synostosis. (A) marks the left frontal bone, (B) the right frontal bone and (C) the soft spot or anterior fontanelle. Notice triangular head shape.
Figure 2. CVR surgery exposure of the patient's frontal area with the frontal bones (A &B) and the soft spot (C) are well visualized.
Figure 3. After the entire forehead has been removed, the frontal bones are (A&B) are reshaped and the band of bone (D) over the eye sockets and lower forehead (orbital bandeau) has now been rounded and reshaped to a normal contour.
Coronal Synostosis CVR
Premature closure of this suture leads to significant cranial and facial deformities which are very difficult to treat and correct in older children. The process is designed to treat this problem attempts to correct the recession of the involved forehead and eye socket by removing the forehead and orbital rim (orbital bandeau) and advancing it forward several centimeters with an FOA. However, only the superior orbital rim is advanced and no changes are made to the abnormally elevated bony orbit. As such, the misalignment of the eyes is not properly corrected and tends to persist.
A Less Invasive Technique for Babies with Craniosynostosis
Twenty three years ago our craniofacial team was not satisfied with the results of CVR surgery. The very traumatic nature of the surgery and the poor long term results led us to search for novel ways to treat our patients. Dr. Jimenez and Plastic Surgeon Dr. Constance Barone designed and created the concept of using endoscopes to minimize exposure and approach the stenosed suture in very young infants, followed by treatment with custom made helmets . Our first publication was presented in the prestigious Journal of Neurosurgery in 1998 which was also the first of its kind.
The basic and fundamental principle with our Craniosynostosis treatment, is to operate on the baby with Craniosynostosis as early as possible. As the brain is growing very rapidly during this period, releasing the closed suture allows the brain to reshape it self to normal with the aid of the helmet.Best results are obtained when the infant receives the procedure by 12 weeks of age. However, successful skull reshaping can be achieved for older babies, with appropriate postoperative cranial helmet therapy.
Our approach is to release the prematurely closed suture and to allow the infant’s misshapen rapidly growing brain to remodel the skull and face to a normal shape. Depending on which suture is affected, our craniosynostosis procedures have been designed to provide the most optimal results. Following surgery, the desired shape of the skull is attained with the use of custom made cranial helmets that are worn continuously by the infant over the ensuing months.
Cranial Helmet Therapy
The Making of the Helmet
To maximize normalization and correction of your baby's cranial and facial deformities, specially crafted and designed custom made helmets are used after surgery and worn for a number of months. These helmets help guide brain development and promote growth in specific areas and apply gentle pressure in others. However, overall brain growth is NOT restricted in any way or form. Normal brain development takes place in all cases. Adjustments are made to the helmet over time to allow for rapid brain and head growth. Cushioning is used to to prevent the infant head from further flattening when tilted or resting on a flat surface. Made of plastic and foam, allergies to the helmet are rare.
We encourage you to start treatment as early as possible since the growth of the head begins to slow down after the first 12 months after birth. The average craniosynostosis treatment typically lasts 12 months with careful and frequent monitoring. However, this time frame is dependent on the age of your baby and severity of craniosynostosis.
Your infant’s cranial helmet will be manufactured by Orthomerica Products, Inc, the largest, most trusted cranial remolding manufacturer in the world. Currently, Orthomerica is one of only two manufacturers that have FDA clearance to construct this apparatus. These highly specialized custom cranial helmets require multiple design options, clinical expertise, and technology in order to achieve optimal clinical outcomes for the safety of your baby. The custom post-operative cranial remolding orthosis (cranial helmet) is a Class II device regulated by the FDA, which requires stringent quality, safety, and labeling information. In June of 2009, Orthomerica Products, Inc was awarded FDA approval.
Dr Jimenez does not have any financial interests or conflicts with Orthoamerica or any of the companies that make the endoscopes or instruments used in your baby's surgery.