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Craniosynostosis Meets Clotting Factor

November 18, 2009

In the minimally invasive approach, two tiny incisions give access to the sagittal suture.


A rounder skull, post surgery.

When Kristie Palmer discovered last spring that her newborn daughter’s head was misshaped and would require surgery to correct, she called a children’s hospital near her home in Pittsburgh. They couldn’t see her until the end of May. Then Palmer learned that Hopkins pediatric neurosurgeon Edward Ahn and pediatric craniofacial surgeon Oliver Simmons specialized in treating the condition, called craniosynostosis. Afflicting 1 in every 2,000 newborns, it is caused by a premature closing of one or more of the sutures of the bones that make up the skull.

Ahn and Simmons take a minimally invasive approach, which would mean less blood loss than in the open procedure and only one or two days in the hospital instead of a longer intensive care stay – as well as potentially better outcomes in the shape of the skull. Palmer called and two days later 3-month-old Addison was being evaluated at Hopkins.

“Her head was changing so fast that a month seemed a long time to wait,” says Palmer. “Also, the hospital here didn’t offer the less-invasive operation.”

Palmer learned that had she waited another week or two, Addison might not have been eligible for the minimally-invasive procedure. The earlier the surgery after birth, Ahn explains, the better the cosmetic outcome. But neurosurgeons often delay the traditional, open operation until infants are at least six months of age because of anesthesia and bleeding risks. The minimally-invasive approach allows them to perform the procedure quickly, usually within one hour, and early, preferably when the infant is less than three months of age.

“The sooner you release the restriction that the craniosynostosis is causing,” says Ahn, “the sooner you allow the brain and skull to develop as they normally would.”

That restriction – the skull suture that has prematurely fused – is commonly the sagittal suture. In the minimally invasive approach, only two tiny incisions are made, allowing surgeons to see the suture with an endoscopic camera and to remove it without major dissection.

“Using the endoscope, we can create a space beneath the skull to remove the suture safely and without a lot of dissection,” says Ahn, noting that studies have shown that only 10 percent of patients undergoing the minimally invasive approach require blood transfusions. “In the open procedure, I would argue that 100 percent of patients receive transfusions.”

Addison was scheduled for her procedure but then another obstacle came up: Her blood screening pointed to a clotting disorder that proved to be Factor VII Deficiency. The extremely rare bleeding disorder – 1 in 500,000 – was confirmed by pediatric hematologist Shirley Reddoch. “We knew that the [bleeding] risk was increased with this identified factor deficiency, and this procedure, even though it was minimally invasive, was not without some bleeding risk of its own,” says Reddoch.

Reddoch recommended scheduled infusions of a coagulation product for a specific factor replacement – Recombinant Factor VIIa (rFVIIa) – beginning just prior to the start of the procedure and ultimately continued for a period of 72 hours, allowing Addison’s blood to clot normally during and after the operation. Addison did receive one red blood cell transfusion following the surgery, but there was no need for further infusion. The plan worked.

“The interesting thing,” says Ahn, “is that with the help of our hematologists we were able to offer minimally invasive cranial surgery on a baby with a clotting disorder.”

For more information, call 410-502-7700.