The first surgical treatment of craniosynostosis was undertaken by Lannelongue in 1892, and involved the correction of a sagittal synostosis. Since then, multiple procedures have been used for the treatment of this condition, ranging from simple suturectomies(removal of fused sutures without skull remodeling to extensive cranial vault reconstruction. The type of surgery done for craniosynostosis is unique to the surgeon performing the repair and unique to the which suture is fused. I encourage parents to thoroughly research all their options before deciding which surgery is best for your child. Second and third opinions from different craniofacial surgeons is recommended if you are unsure of the first consult.
Endoscopic Surgery:
In 1998, Dr. Jimenez and Dr. Barone pioneered the endoscopic surgery for Craniosynostosis. This surgery is the least invasive approach. For this surgery to be the most successful it is advised that surgery be performed on an affected infant before four months old and no later than six months old. Surgeons will utilize an endoscope through two small incisions measuring about 1.5 inches each at the top of the head. A segment of bone is removed near the fused bone and in a few other places which releases the fusion. No plating or reshaping is performed. Instead, in the first week after surgery, a custom molding helmet is made, which the patient wears for up to a year. The pro's of the endoscopic surgery are:
- Decreased operative time (1.5 hours or less vs. usually 5 or more hours)
- There is usually no need for a blood transfusion, and much decreased blood loss
- No plates or screws are needed
- Shortened hospital stay: patients generally go home on day 1 or day 2
- Smaller incisions which have significantly reduced scars
- Greatly reduced discomfort and swelling.
- Craniosynostosis must be caught at an early age for the best cosmetic results.
- travel to a qualified surgeon(few surgeons are qualified to perform the endoscopic procedure)
- a year of helmet wearing which will also require possible traveling for adjustments and checkups as the child grows out of the helmet.
- less favorable cosmetic results
- a higher possibility of secondary surgery later
- possibility of missed hydrocephelous when no CT scans are performed prior to surgery(it is stated that CT scans are not ordered in Saggital cases)
- Hyperostotic bone(Excessive or abnormal thickening or growth of bone tissue)
- leptomeningeal cyst which can develop if a dural laceration is not seen and repaired during surgery
- if a complication with bleeding should arise during surgery precious time is lost due to needing to open up the skull for repair.
Cranial Vault Reconstruction:
Cranial Vault Reconstruction also known as CVR or traditional surgery is the most common surgery performed. It is a complex surgery and will require the use of both a pediatric neurosurgeon and pediatric craniofacial surgeon. Some surgeons prefer to do either just the front of the skull(anterior CVR) or just the back of the skull(posterior CVR). While other surgeons might choose an overall CVR depending on the degree of reconstruction needed. In some cases a front orbit advancement(FOA) is performed to reconstruct the orbits around the eyes. FOA's are hardly ever performed without an accompanying CVR.
An incision is made in the scalp from ear to ear in either a zig-zag pattern or u-shape. The skull bones are then cut and removed by a pediatric neurosurgeon. A CVR should never be performed without the neurosurgeon who's job is to protect the duram that surrounds and protects the brain. A pediatric craniofacial surgeon then will reconstruct and piece the skull bones back together using plates, screws, and stitches. Most of these foreign objects are absorbable which means as the new bone grows the plates and screws will slowly disappear. The pro's of CVR Surgery are:
- Immediate and often lasting correction of the deformity without any visible or palpable bony defects
- Drastically reduced occurrences of secondary surgeries
- More qualified surgeons available to perform CVR surgery
- CVR is most successful when performed on children younger than one year of age but is highly successful when performed on even older children
- Any complications with the duram or blood loss can be immediately handled.
- Most successful treatment for multi-suture synostosis and severe cranial deformity.
- Blood loss with the need of blood transfusions(although this risk can be greatly reduced with the use of procreate shots and blood recycling procedures.)
- Highly invasive surgery that last 5 to 9 hours typically
- Plates and screws most often are needed
- 48 hour stay in PICU required followed by 3 or 4 more days in hospital
- Swelling and bruising
Strip Craniectomy:
Most commonly an incision is made across the scalp from ear to ear in a zig-zag or u-shaped pattern. However in some cases of Saggital the incision has been made from the front of the scalp to the back across the middle. A strip of bone is usually removed where 2 sutures connect and cuts are usually made down the sides of the skull to allow for natural reshaping as the brain grows. Sometimes smaller pieces of bone are removed and the skull is then allowed to be remolded as the brain grows and the open spots allow the natural growth to take place. Barrell Stave Craniectomy:
This too is an open procedure so an incision is typically made from ear to ear. Strips and pieces of bone are removed and repositioned with some gaps and in places where reconstruction is needed the bones are cut into smaller, flowering shapes that will move and conform with the brain as it grows. Bone sometimes can be stitched back together without need of plates and screws.
Spring-mediated surgery -
One of the newest methods of correction. Like the endoscopic surgery surgeons will utilize an endoscope through two small incisions measuring about 1.5 inches each at the top of the head. A segment of bone is removed near the fused bone and in a few other places which releases the fusion. Springs are then inserted in the open spaces to help ensure that the opening does not re-fuse. A second procedure will be required to remove the springs. As with any surgery all these procedures I have talked about have risk. It is important that parents do research, ask plenty of questions, and make sure you are completely comfortable in the surgical team they choose their your child.
Images above courtesy of eMedicine - Medical Reference
Overwhelmed?
Its completely normal to feel overwhelmed and frightened by the over-abundance of information presented to you during this whole experience. Not only are you dealing with a flurry of emotions, you also have huge decisions to make in a short amount of time. How everyone handles this whole process is unique to the individual. Know that you are NOT ALONE. Many other parents have been down the very path you are starting and are willing to help you by giving a shoulder to cry on, a friend to laugh with, and answers to your questions.
A Few of My Favorite Online Communities are:
CAPPSKidsJorge Posada Foundation
CranioKids
Faces
CCA Kids
more on our website are
How Do I Choose A Surgical Team?
For many families craniosynostosis post-surgical follow up last through adolescence. It is important to choose the Craniofacial Surgeon you feel is the best choice for your child. No pediatrician or insurance company should be allowed to bully you from getting the best surgical outcome for your child. Insurance denials can be appealed and many organizations can help with travel cost and boarding during surgery if you need to travel out of state. That being said there are plenty of highly qualified craniofacial surgeons in your area and your country. You will find if you ask opinions many of us have our favorite craniofacial teams and tend to be biased after years of having them be part of our families.
Click here for Link to List of Surgeons compiled by FACES:
