Normally, the bony plates that make up a baby’s skull remain unfused until the brain is done growing rapidly in the first two years of life. If the bony plates fuse together at sutures in the skull prematurely, this results in craniosynostosis. Craniosynostosis typically causes pressure to build inside the head, and the skull or facial bones shift from their normal, symmetrical appearance to provide room for the growing brain.

Craniosynostosis that occurs by chance is common, occurring in 1 out of every 2,500 births and usually is characterized by the premature fusing of one suture in the skull. Genetic syndromes, such as Apert, Crouzon, Muenke, Pfeiffer and Saethre-Chotzen syndromes are rarer, occurring in only 1 in 100,000 births, and cause craniosynostosis characterized by the fusion of multiple sutures. 


The most common signs of craniosynostosis are changes in the shape of the head and face. Other, less common signs may include a bulging fontanelle (the soft spot located at the top of a baby’s head), trouble sleeping, trouble eating, headaches and vision problems.

Types of Craniosynostosis

All forms of craniosynostosis are associated with abnormal head shapes but differ based on the number of sutures fused. 

  • Scaphocephaly occurs when the sagittal suture fuses. This suture runs front to back, down the middle of the top of the head, so when it fuses, the skull becomes long and narrow. Scaphocephaly is the most common type of craniosynostosis, occurring in 40 to 50 percent of cases. 
  • Trigonocephaly occurs with metopic suture fusion. This suture runs from the top of the head down to the forehead, in the direction of the nose, so when it fuses, the forehead looks like a triangle. It is the second most common form of craniosynostosis, occurring in 25 percent of cases. 
  • The coronal suture runs across the skull, from ear to ear. Either the left or right side of this suture can fuse, resulting in plagiocephaly and producing a flattening of the forehead and the brow on the affected side. This type of craniosynostosis occurs in 20 percent of cases. 
  • The lambdoid suture is located at the back of the skull. When it fuses prematurely, the back of the head can become flattened. The ear on the affected side may also be positioned lower down and further back than the other. This condition is the rarest type of craniosynostosis, occurring in only three percent of cases. It is frequently confused with positional plagiocephaly, which is a benign flattening that does not require surgical treatment.

The plastic and neurosurgeons at the North Carolina Cleft and Craniofacial Center address both development and appearance by working collaboratively to provide infants and children with effective treatments for craniosynostosis.


Early diagnosis and consultation with a specialist are important. The ideal time for surgery and treatment is within your child’s first year when the bones are still very malleable. If your child’s condition is severe, our team may recommend surgery as early as one month of age.


To treat craniosynostosis, our plastic surgeons and neurosurgeons work together to surgically remove the prematurely fused suture using one of two methods.

  • Open surgery or cranial vault remodeling is the traditional treatment for craniosynostosis, which reshapes the affected portions of the skull and typically requires a three- to four-day hospital stay with one day in the intensive care unit and potential blood transfusions.
  • Minimally invasive surgery is an option for certain types of craniosynostosis. Here, the fused suture is removed using limited incision and the skull shape is then gradually corrected with a molding helmet or with springs. These surgeries typically involve just an overnight hospital stay and rarely involve blood transfusions. These techniques are typically reserved for children six months or younger.