Craniosynostosis symptoms:

Signs of craniosynostosis include:

• A misshapen skull, with the shape depending on which of the cranial sutures are affected
• An abnormal feeling or disappearing “soft spot” (fontanel) on your baby’s skull
• Slow or no growth of the head as your baby grows
• Development of a raised, hard ridge along affected sutures
• Increased pressure within the skull (intracranial pressure)

The signs of craniosynostosis may not be noticeable at birth, but they become apparent during the first few months of your baby’s life.

Craniosynostosis types and characteristics

There are many different types of craniosynostosis. The term given to each type depends on what sutures are affected. Some of the most common types of craniosynostosis are:

• Sagittal synostosis (scaphocephaly). Premature fusion of the suture at the top of the head (sagittal suture) forces the head to grow long and narrow, rather than wide. Scaphocephaly is the most common type of craniosynostosis.
• Coronal synostosis (anterior plagiocephaly). Premature fusion of a coronal suture — one of the structures that run from each ear to the sagittal suture on top of the head — may force your baby’s forehead to flatten on the affected side. It may also raise the eye socket and cause a deviated nose and slanted skull.
• Bicoronal synostosis (brachycephaly). When both of the coronal sutures fuse prematurely, your baby may have a flat, elevated forehead and brow.

Craniosynostosis is often classified as nonsyndromic or syndromic. Nonsyndromic craniosynostosis is the most common type of craniosynostosis, and its cause is unknown.

However, syndromic craniosynostosis is a complication caused by certain genetic syndromes, such as Apert syndrome, Pfeiffer syndrome and Crouzon syndrome, which can affect your baby’s skull development. Besides craniosynostosis, other conditions also may accompany such syndromes — including hand and foot deformities, dental abnormalities, and heart problems.

Surgery:

For most babies, however, surgery is the primary treatment for craniosynostosis. The type and timing of surgery depend on the type of craniosynostosis and whether there’s an underlying syndrome that needs treatment.

The purpose of surgery is to relieve pressure on the brain, create room for the brain to grow normally and improve your child’s appearance. A team that includes a specialist in surgery of the head and face (craniofacial surgeon) and a specialist in brain surgery (neurosurgeon) often performs the procedure.

• Traditional surgery. The surgeon makes an incision in your baby’s scalp and cranial bones, then reshapes the affected portion of the skull. Sometimes plates and screws, often made of material that is absorbed over time, are used to hold the bones in place. Surgery, which is performed during general anesthesia, usually takes hours.After surgery, your baby remains in the hospital for at least three days. Some children may require a second surgery later because the craniosynostosis recurs. Also, children with facial deformities often require future surgeries to reshape their faces.
• Endoscopic surgery. This less invasive form of surgery isn’t an option for everyone. But in certain cases, the surgeon may use a lighted tube (endoscope) inserted through one or two small scalp incisions over the affected suture. The surgeon then opens the suture to enable your baby’s brain to grow normally. Endoscopic surgery usually takes about an hour, causes less swelling and blood loss, and shortens the hospital stay, often to one day after surgery.

If your baby has an underlying syndrome, your doctor may recommend regular follow-up visits after surgery to monitor head growth and check for increased intracranial pressure. Head growth will be routinely monitored at well-child visits.