Pediatric craniosynostosis is a condition in which the bones of a newborn’s skull fuse too early. This premature fusion leads to an abnormal head shape; often causing the skull to bulge out at the back of the head, or the face and head to elongate abnormally. If pediatric craniosynostosis is very severe, it can even slow down or inhibit brain growth.
The skull of an infant is made up of 5 main bones: two frontal bones, two parietal bones, and the occipital bone. These bones are joined together by sutures, which are fibrous bands of collagen that connect the skull bones and allow them to move and grow appropriately. If one suture closes too early, the rest of the skull will compensate for that, and may grow outward, more than normal. This leads to the appearance of a bulge in the skull, or an abnormal lengthening of the head.
Pediatric craniosynostosis is usually only an aesthetic problem, but sometimes it can lead to increased intracranial pressure (high pressure inside the brain). Severe craniosynostosis can lead to developmental and intellectual disabilities as well. Even if disabilities do not exist, uncorrected craniosynostosis may be a source of stress or anxiety for a child and may increase their risk of bullying and depression
The most obvious symptom of pediatric craniosynostosis is usually an abnormal head shape, but there are other symptoms that can occur in some cases:
Most of the time, the exact cause of craniosynostosis is not known. These cases are known as non-syndromic craniosynostosis, and it is due to a combination of genetic and environmental factors. Other times, craniosynostosis is due to a genetic disorder, and is known as syndromic craniosynostosis. Syndromic craniosynostosis is a part of some conditions like Apert syndrome, Crouzon syndrome, and Carpenter syndrome. These disorders are usually not inherited and occur randomly or sporadically, due to certain mutations in a variety of genes.
There are several risk factors for pediatric craniosynostosis. Many of the risk factors are maternal risk factors, meaning the health and environment of the mother affects the likelihood of her baby developing this condition.
Diagnosis of craniosynostosis usually involves three things – physical exam, imaging, and genetic testing. A paediatrician, and some specialists like a paediatric plastic surgeon, a paediatric neurologist and a paediatric neurosurgeon will likely perform a physical exam, with special focus on your child’s head. Then imaging – usually CT-scans and MRIs of the brain – are often the next step. If craniosynostosis is syndromic, genetic testing may also be done, to find out which genes are causing the symptoms.
Craniosynostosis is treated based on its severity. If it’s mild or moderate, sometimes no treatment is needed, or a special helmet may be given to you by your doctor to help mould or reshape your babies head. For more severe cases, surgery may be necessary to correct craniosynostosis. The surgery can both improve the aesthetic appearance of your baby’s head, decrease intracranial pressure, and give your baby’s head more room to grow.
If your child has other symptoms like seizures, blindness, or intellectual disability, they may need other medicines or other forms of therapy as well.