Craniosynostosis

A craniosynostosis is a developmental anomaly which occurs as a consequence of abnormal and non-physiological sutural fusion. In a newborn, the membranous bones of the cranial vault are separated by the intervening sutures. Such arrangement enables the infant’s skull to pass more easily through the birth canal and allows the compensatory growth of skull during the brain growth.

When one or more sutures are prematurely closed, the compensatory growth starts perpendicular to the patent sutures since the brain still grows and expands in the direction of lower resistance. The result is an abnormally shaped skull and also, in more severe cases, increased intracranial pressure (ICP), as well as sensory, respiratory and neurological dysfunctions During infancy and childhood, the skull bones  expand to accommodate the growing brain.

Since it is a disease of infants sometimes it may be difficult to assess early in course if skull shape is normal or abnormal.

The most common presentation is with an unusual head shape in the first year of life the head may be long and narrow or triangular at the front broad and flattened or skewed.

Such children may have problems associated with the airway, feeding, eye protection and raised intracranial pressure.

Surgery for these children is done after taking into account various factors like treating hydrocephalus first, then may be staged remodelling of skull shape and in certain cases cranio facial advancement.  It is a challenging surgery due to the young age of the patient and sometimes long duration and postoperative critical care plays a very crucial role in this whole process.