Isolated (Non-Syndromic) Craniosynostosis: Suture-specific surgeries
Sagittal Suture Craniosynostosis (Scaphocephaly)
Ideally, the diagnosis is made shortly after delivery. For patients less than 4 months of age an extended strip craniectomy with postoperative helmet therapy is the treatment of choice. This approach provides good correction in head shape, with a shorter operation and hospitalization and lower risk for blood transfusion when compared to open cranial vault procedures. After surgery, we place the baby in a custom-fit molding helmet. This helmet fits up against the forehead and back of the head and does not apply pressure. Instead, it resists growth in these directions. The skull then grows to a more normal shape because the brain takes the path of least resistance and widens pushing the bones as it expands.
Patients with sagittal suture craniosynostosis (scaphocephaly) diagnosed older than four months of age are too old for extended strip craniectomy surgery. This is because after 4 months of age the skull bones quickly become too thick and stiff for sufficient reshaping with the helmet. As surgeons, we must customize our surgical plan to each child’s skull deformity.
Most often, we will use an open cranial vault remodeling for these patients. Often this is limited to reconstructing the back two-thirds of the skull in one operation. The full forehead seen in most of these patients tends to correct on its own once the back is reconstructed. Occasionally, in severe cases, separate operations are needed to correct the front and the back of the skull. These surgeries are done in stages with a period of 3-6 months between procedures to allow for recovery between the procedures.
There are many surgeries that have been developed to treat patients with sagittal suture craniosynososis. In general, the goals of surgery are to overcorrect the head shape to allow for a normal skull shape once fully grown. Occasionally cranial distraction can be used in these patients.
Single (Unilateral) Coronal Suture Craniosynostosis
There has been increased interest in using extended strip craniectomy with post-operative helmeting in these patients. We feel that this approach will often undercorrect the abnormal contour of the eye socket and forehead when compared to the degree of improvement seen in open cranial vault procedures. We will however use this approach occasionally. For the majority of our patients we use fronto-orbital advancement to increase the volume inside the skull and overcorrect the position of the forehead and eye socket, placing these bones as far forward as possible. The plan is to overcorrect the position of the forehead and eye socket as much as possible because the bones bones will not move foreward because of the closed coronal suture. So we overcorrect and allow the child to grow into the correction so they will achieve a normal skull shape as they approach completion fo the skulls growth which is over 90% grown by age 5 years old.
Metopic Suture Craniosynostosis (Trigonocephaly)
Like unilateral coronal craniosynostosis, there has been increased interest in using extended strip craniectomy with post-operative helmeting in these patients. We feel that this approach may undercorrect the abnormal contour of the eye sockets and forehead when compared to the degree of improvement seen in open cranial vault procedures. In general, we use a fronto-orbital advancement tailored to a patient’s deformity. This approach allows widening and reshaping of the forehead and moving the upper part of the eye sockets and temple areas to correct the “pinched” appearance to the forehead.. Bone from the back of the skull is fixed into position to widen the forehead using absorbable plates to allow for this reshaping. Surgery overcorrects the width of the forehead so the patient will “grow into” his or her forehead. Again, the goal is to achieve a normal dimension to the forehead when the patient is fully grown.
These patients need surgery to overcorrect the affected side. This is best done by swapping the bones from the back of the head using an open cranial vault procedure. This, along with reshaping of the middle portion of the skull, provides a durable reconstruction that rarely requires any more surgeries.