Craniosynostosis is a disorder characterized by premature fusion of the skull bones, before or after the birth. It can affect the baby’s brain growth and could lead to complications, which might last for a lifetime. However, if intervention is sufficiently early, it is a correctable disorder although the benefits of such early interventions depend on other underlying disorders associated with Craniosynostosis.
What is the difference between a normal newborn skull and a skull with Craniosynostosis?
The skull of a newborn baby would be composed of seven bony plates, which are bound to each other by fibrous joints known as ‘cranial sutures’. When the brain expands, the space within the skull would not be adequate and therefore the cranial sutures will allow the bony plates to expand according to the growing demands. Furthermore, it will help maintain the symmetry of the skull as well. After few years, the fibrous sutures will lose its elasticity and separate bony plates will fuse to form a single bony skull. As described earlier, babies with Craniosynostosis would not be having such flexible cranial sutures and therefore the expanding brain would be deprived of adequate space.
What can cause Craniosynostosis?
At present, researchers are unable to distinguish a specific reason for its occurrence although it has shown to be associated with several syndromes. These include Apert’s syndrome and Crouzon syndrome, which indicates a possible genetic predisposition in developing this condition. However, scientists relate some incidences of Craniosynostosis with maternal smoking, mothers living in high altitudes during pregnancy and for being one of twins.
What symptoms are associated with this condition?
The symptoms related to Craniosynostosis may not be apparent at birth. However, when the brain grows, the manifestations can appear within the first year of life. The degree of manifestation would depend on the type of Craniosynostosis, the sutures involved and any other underlying genetic disorders.
The most noticeable manifestation among affected infants would be a misshapen skull. The skull may appear elongated, flattened, or else asymmetrical. However, presence of a misshapen skull does not necessarily mean the baby would have Craniosynostosis as it can also manifest with the baby’s head position during sleep and its position inside the womb.
Presence of prominent sutures and abnormal fontanels (‘soft spots’ on the surface of the skull in infants) can also characterize Craniosynostosis. At the same time, these ‘soft spots’ could disappear prematurely or may not be present at all.
In some infants, the head circumference may remain the same or else the increase would not be age appropriate.
Apart from the above symptoms, infants who are suffering from Craniosynostosis along with any other congenital syndromes could suffer from seizures, blindness, developmental delays, as well as mental retardation to a varying degree. However, pressure effects due to Craniosynostosis are rare and could be avoided following early interventions.
What treatment options are available for infants suffering from Craniosynostosis?
When diagnosed of having Craniosynostosis, the doctors would look into pressure effects and other clinical manifestations before deciding on any interventions. In most instances, they would be happy to avoid any interventions although frequent follow-ups and head measurements would be taken to ascertain any growth failures and other clinical manifestations. If decided to intervene, the only available options would be a surgery to relieve the fused cranial sutures or else to create a suture line by incising the skull bone.
There are two different surgical procedures adapted in correcting Craniosynostosis and in one such method, the surgeons would create a incision on the surface of the skull bone and would re-position the skull bones to correct any asymmetry. At times, the re-positioned skull bones would be held in place using screws and plates, which will get absorbed over time.
In the second surgical approach, endoscopic techniques are used to perform a minimally invasive surgery through a small incision, which enable surgeons to insert an endoscopic device, which can release the fused suture enabling it to be more flexible.
Apart from correcting the Craniosynostosis, some children would also require further surgeries to correct other facial abnormalities and at times, secondary surgeries would be needed to correct any recurrences of the same.
References:
Craniosynostosis information page. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/craniosynostosis/craniosynostosis.htm. Accessed April 05, 2011
Kinsman SL, Johnston MV (2007). Craniosynostosis section of Congenital anomalies of the central nervous system. In RM Kliegman et al., eds., Nelson Textbook of Pediatrics, 18th edition.
