Craniosynostosis is an abnormal fusion of one or more of the growth plates of the skull. It is relatively common, and can occur in as many as 1/1800 live births. It can be non-syndromic, meaning it occurs as an isolated event, or can be part of spectrum of deformities known as a syndrome. Non-syndromic is far more common, and is thought to occur possibly due to pressure placed on the skull in utero, though no one really knows exactly.
The growth of the skull is restricted perpendicular to the fused plate which can lead to a deformed head shape and increased pressure on the brain. The orbit (eye socket) and face may also be impacted as a result.
The metopic growth plate (suture) lies between the forehead bones and is now thought to be the most common craniosynostosis. It normally fuses around age 8 months, but early fusion can lead to a narrow but pointy forehead with closely set eyes (hypotelorism). Surgery involves expanding the narrow forehead and the top part of the eye sockets (supraorbital rim)
The sagittal suture runs longitudinally in the middle of the skull, and craniosynotosis can cause a head shape that is very long and narrow. The forehead and the back of the head (occiput) often bulge. If caught early (before the head shape is too deformed), removal of the fused suture and helmet therapy can lead to good outcomes. If late, typically two surgeries are needed to shorten and widen the skull, one for the back part and one for the front part.
Fusion of the unicornal suture produces a characteristic head shape that is trapezoidal. The forehead and eye socket on the affected side slope back, the back of the head flattens, and the other side often sticks out. The bones of the nose may slope towards the affected side and the chin often deviates to the opposite side. Surgery involves reshaping the affected bones.
The rarest of the craniosynostosis, A fused lambdoid suture causes a flattened back of the head with a large bulge behind the ear (mastoid).
If 2 or more growth plates are fused the head shape can be severely deformed and place extreme pressure on the developing brain. Many syndromic craniosynostoses are multi-sutural and surgery is aimed at first relieving the pressure (by expanding the vault) and then by reshaping the bones.
Suture release and helmet
This technique involves removal of the fused growth plate which will allow the brain to expand and then placement of a helmet for four to six months to help shape the formation of the developing skull. When identified early, this technique works great for sagital synostosis and may even be performed endoscopically (minimally invasive)
Cranial vault remodeling
The gold standard and most widely accepted way for treating craniosynostosis. The bones of the skull can be reshaped and pressure relieved using an approach through a bicoronal incision hidden in your child’s hairline. Using the latest technology of resorbable plates, screws and sutures, the abnormal bones are contoured and repositioned to recreate the normal anatomy. Infants recover incredibly well from this procedure. This surgery is often performed between 6-12 months.
Cranial vault distraction
A newer method of relieving pressure, typically in multi-suture synostosis involves lengthening the skull by applying devices known as distractors. Two small devices are placed on the bone edges which are slowly turned daily leading to the formation of new bone and expansion of the skull removing pressure on the developing brain.