Craniosynostosis is the premature closure of skull sutures in infants or children. The skull of an infant includes five main bones, which are separated by sutures. These sutures allow the skull to easily expand over the rapidly growing brain. For the most part, they remain open until adulthood.
Four of these sutures meet at the anterior fontanelle (soft spot), and are the metopic, sagittal, and paired coronal sutures. The paired lambdoid sutures are located in the back of the head. Of these, only the metopic suture closes during childhood, normally doing so by age one or two.
Craniosynostosis may occur when either a single or multiple sutures close prematurely. This occurs in one of every 2,500 live births. Only rarely (1 in 100,000 births) is there a genetic component, which generally affects multiple sutures. These include Apert, Crouzon, Muenke, Pfeiffer and Saethre-Chotzen syndromes.
Impact of Craniosynostosis
There is growing evidence that when untreated, single-suture craniosynostosis may have small, negative effects on neurologic development. Untreated multiple-suture craniosynostosis is known to cause severe neurologic impacts on children.
All forms of craniosynostosis are associated with abnormal head shapes.
- Scaphocephaly (boat-shaped) refers to an elongated head shape which occurs with sagittal suture fusion, and is the most common type of craniosynostosis (40-50 percent of cases).
- Trigonocephaly (triangular-shaped) occurs with metopic suture fusion, and is the second most common form of craniosynostosis (25 percent of cases).
- Coronal suture fusion results in anterior plagiocephaly if one suture is fused, or brachycephaly and turricephaly (shortened and tall shape) if both sutures are fused (20 percent of cases).
- Lambdoid suture fusion results in posterior plagiocephaly (3 percent of cases).
To address both development as well as appearance, Wake Forest’s plastic surgeons and neurosurgeons work collaboratively to provide infants and children with effective treatments for craniosynostosis.
To treat craniosynostosis, a plastic surgeon and a neurosurgeon work together to surgically remove the prematurely fused suture.
There are two ways this can be done:
- Open surgery, or cranial vault remodeling (CVR): Traditionally, craniosynostosis is treated using open surgery to reshape the affected portions of the skull. CVR surgery typically requires a three- to four-day hospital stay, including one day in the intensive care unit, and may require a blood transfusion.
- Minimally invasive surgery: Certain types of craniosynostosis can be successfully treated with minimally invasive surgeries, in which the fused suture is removed using a limited incision. The patient’s skull shape is then gradually corrected with a molding helmet or with springs. These surgeries typically involve just an overnight hospital stay, and blood transfusions are rare. These techniques are typically reserved for those under 6 months of age.