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It happens to one out of every 2,000 babies -- the bones in their skull fuse together, causing a pointed head. To relieve the pressure on a growing brain, surgeons have to operate. Now doctors have a way of fixing the condition with less blood loss, smaller scars and faster recovery for the smallest patients.He's not yet 2, but Jack Truitt is learning a lot about family traditions. He recently had surgery on his skull, just like his dad."When it happened to me, I was 3 months old," said Steve Truitt, Jack's dad.Both had craniosynostosis. Bones in the skull fuse together, causing babies heads to be shaped like footballs. Steve has a lasting reminder of his operation across his head, but baby Jack will have to rely on his parents and pictures to learn about his surgery."The difference between my scar and his scar is night and day," Truitt said.Surgeons took a minimally invasive approach for Jack's operation. Through two small incisions they removed the fused piece of bone at the top of the skull. It relieves the pressure on the brain and allows it to grow normally. And the natural shape of the head is restored.Compared to traditional surgery, the newer approach has a much smaller incision and less blood loss. Most babies don't need a blood transfusion, and they're in the hospital one day compared to five to seven."We have many infants who go into the surgery and never require a transfusion whatsoever. They come in the morning of surgery and leave the next day without ever receiving anything," said Dr. Edward Ahn, a pediatric neurosurgeon at Johns Hopkins Children's Center in Baltimore."I'd say within a couple of weeks you could see his head go from a football shape to a basketball shape," Truitt said.Jack had to wear a helmet after surgery to protect his head, but now he's a typical baby.In order for babies to qualify for the minimally invasive approach, surgeons must operate before three months of age.
BACKGROUND: Craniosynostosis is a congenital defect that causes one or more sutures on a babys head to close earlier than normal. Sutures are connections that separate each individual skull bone. The early closing of a suture leads to an abnormally shaped head, sometimes shaped like a football rather than round. Doctors dont fully understand what causes craniosynostosis, but a persons genes may play a role. However, most cases occur in families with no history of the condition, and children with the defect are otherwise healthy and have normal intelligence.There are different types of craniosynostosis. Sagittal synostosis is the most common, and it affects the main suture on the very top of the head. The early closing forces the head to grow with a long and narrow shape, instead of wide. Babies tend to have a broad forehead, and its more common in boys than girls. Frontal plagiocephaly is the next most common form of craniosynostosis, which is the closure of one side of the suture that runs from ear to ear on the top of the head. This type is more common in girls. Metopic synostosis is the rarest form, which impacts the suture close to the forehead. The childs head shape may be described as trigonocephaly, and the deformity may range from mild to severe.
DIAGNOSIS: Symptoms of craniosynostosis include the absence of the normal feeling of a soft spot on the newborns skull, an unusual head shape and slow or no increase in the head size over time as the baby grows. In order to diagnose craniosynostosis, a doctor will feel the infant's head and perform a physical exam. A neurological exam also helps in diagnosis. Doctors often measure the width of the infants head, X-ray the skull or use CT scans to get a clearer look at the problem. Then they determine if surgery is necessary.Doctors say craniosynostosis affects one in every 2,000 newborns. At Johns Hopkins and other hospitals, surgeons are taking a minimally invasive approach when appropriate to save the child from long recovery. The minimally invasive approach involves two inch-long incisions -- instead of a 10-inch incision -- across the top of the skull. It means less blood loss than the open procedure, less chance of a blood transfusion and less time in the hospital: one or two days compared to five to seven. In the minimally invasive approach, surgeons use an endoscopic camera to see the suture and then remove it without major dissection. Doctors say about 10 percent of babies undergoing the minimally invasive approach require blood transfusions, but in the open procedure, nearly 100 percent of patients receive transfusions. Surgeons say the earlier the surgery after birth, the better the cosmetic outcome. However, neurosurgeons often delay the traditional, open operation until infants are at least 6 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 earlier, preferably when the baby is younger than 3 months old.