Craniosynostosis

Reviewed by Kristie Rivers, MD, FAAP, Board Certified Pediatrician

Craniosynostosis could be a misshapen skull, missing fontanel, or your baby’s head does not grow with the rest of the body.

Craniosynostosis could be a misshapen skull, missing fontanel, or your baby’s head does not grow with the rest of the body.

Newborn babies are works in progress. For those first few months, they are rapidly developing and changing as they adjust to life outside the womb. When babies are born, the seven separate bones in their heads haven’t yet fused together to form the hard protective case of the skull, making it possible for babies to travel down the birth canal without injury. However when the bones fuse together too early, craniosynostosis occurs. As a result, your baby’s head becomes abnormally shaped and in severe cases, the brain doesn’t have sufficient room to grow. If your baby’s pediatrician suspects this condition, they will likely order an x-ray or CT scan of your baby’s brain to look closely at the bones.

Signs of craniosynostosis include:

• A misshapen skull, which may be evident at birth or within a few months.
• A missing or oddly shaped fontanel (“soft spot”) on the top of your baby’s head.
• The growth of your baby’s head does not keep up with the rest of the body.
• The development of a raised, hard ridge between the affected skull bones.
• Increased intracranial pressure (which can only be determined by medical tests). Symptoms include vomiting, irritability, and extreme sleepiness.

The only treatment for craniosynostosis is surgical repair. The surgery typically involves a craniofacial surgeon and a neurosurgeon. If your baby was born with this condition, an evaluation should be performed as soon as possible after birth, before much skull growth has occurred. The best surgical results are obtained in the first 6 months of life. If your baby does not have the concerning signs of increased intracranial pressure recognized by your doctor, the surgery is purely for cosmetic reasons, as neurologic damage does not occur.

However, you should not assume your child has craniosynostosis because of a misshapen head at birth; many babies are born with slightly misshapen or flattened skulls that smooth out over the next few weeks. The backs of newborns’ heads can also be flattened if they consistently sleep in the doctor-recommended position on their backs; this type of “positional molding” (or plagiocephaly) is usually harmless and can often be remedied by allowing your baby plenty of tummy time. Severe cases may require a cranial helmet that helps reshape your baby’s developing skull. Your baby’s doctor will be able to determine the difference by a simple exam and possibly x-rays.