An infant’s skull has multiple bony plates separated by flexible seams called cranial sutures. These sutures allow the skull to be pliable for birth and accommodate the rapid brain growth that occurs during the first few years of life. The sagittal suture runs lengthwise along the top of the head, from the soft spot near the front to the back. Sagittal craniosynostosis is a condition where this suture fuses prematurely, before birth, restricting the skull’s natural growth and leading to a characteristic shape.
Physical Signs of Sagittal Craniosynostosis
The most apparent sign of sagittal craniosynostosis is a distinctive head shape called scaphocephaly. Because the sagittal suture has fused early, the skull cannot expand sideways. To compensate for the growing brain, the skull grows longer from front to back, resulting in a long, narrow head. This is often accompanied by a prominent or bulging forehead, known as frontal bossing, and a pointed appearance at the back of the head.
Parents may also notice a narrowing of the head at the temples. In many infants with this condition, a hard, raised ridge, which is the prematurely closed suture, can be felt along the top of the skull. This condition is observed more frequently in boys than in girls.
The Diagnostic Process
Diagnosis begins with a physical examination by a pediatrician or pediatric specialist. The doctor will observe the baby’s head shape and feel the skull, paying close attention to the fontanelles and suture lines. The characteristic head shape and a palpable ridge along the suture line are strong indicators of the condition.
Although a physical exam provides strong clues, imaging tests are used to confirm the diagnosis. A computed tomography (CT) scan is used to create detailed, cross-sectional images of the skull. These images clearly show the fused sagittal suture and help rule out other causes for the abnormal head shape. The scan also provides a view of the brain and helps assess for any signs of increased pressure inside the skull.
Evaluating for elevated intracranial pressure (ICP) is also part of the diagnosis. While not all children develop high ICP, the restricted skull can create pressure on the brain. X-rays or specialized 3D photographic imaging may be used as supplementary tools to monitor bone growth.
Surgical Treatment Approaches
Treatment for sagittal craniosynostosis is surgical, aiming to correct the head shape and allow for normal brain growth. The surgical approach depends on the infant’s age at diagnosis. For infants under four to six months old, a minimally invasive procedure is often an option.
This surgery, known as an endoscopic strip craniectomy, involves making one or two small incisions in the scalp. Through these openings, a surgeon uses an endoscope and specialized instruments to remove the fused sagittal suture. This procedure is less invasive, involves a shorter hospital stay, and has a lower risk of requiring a blood transfusion. Following the endoscopic procedure, the infant must wear a custom-molded helmet for several months. The helmet guides the subsequent growth of the brain and skull into a more rounded shape.
For infants diagnosed after six months of age, or for those with more severe deformities, an extensive procedure called open cranial vault remodeling (CVR) is performed. This surgery is performed when the child is older, as the skull bones are thicker and more suitable for reshaping. Surgeons make a larger incision across the scalp, expose the skull bones, and then cut, reshape, and reposition them to create a more normal head shape. This complex operation allows for significant, immediate correction of the skull’s form.
Post-Surgery Recovery and Outlook
Following either surgical procedure, the child will be monitored in the hospital for a few days. The hospital stay for open remodeling is longer than for an endoscopic procedure. Once home, parents will manage wound care and pain as directed by the surgical team. Regular follow-up appointments with the craniofacial team are scheduled to monitor the healing process and the child’s head shape.
The prognosis for children who undergo surgical correction for sagittal craniosynostosis is positive. Surgery effectively corrects the head shape, which can prevent future psychological issues related to appearance. With timely treatment, most children experience normal brain development and cognitive function, and go on to lead healthy lives with excellent long-term cosmetic results.