Craniosynostosis: Adaya’s Story
Mandy knew something wasn’t right as soon she laid eyes on her new baby girl.
“I’m a labor and delivery nurse and since she was born breach by c-section, I was expecting to see an oblong (football-shaped) head.”
Most of her medical staff at the Key West hospital where baby Adaya was born assured Mandy the head would return to a more normal shape over time.
“One of my nurse coworkers, who shared my concerns, did some research and said, ‘Mandy, I don’t think your baby has breach head. I think she has craniosynostosis.’”
Even as a registered nurse, Mandy admits she didn’t know much about craniosynostosis, a condition in which the skull sutures close early and can cause problems with normal brain and skull growth. Premature closure of the sutures may also cause pressure inside the head to increase and the skull or facial bones to grow in an abnormal shape. This condition requires surgical treatment to correct the head shape and increase space for the brain to grow.
As soon as she was discharged from the hospital, Mandy decided to get another medical opinion.
This time the pediatrician told her that she couldn’t tell for sure if it was craniosynostosis but offered to give her a referral to get a skull X-ray. Mandy knew from her research that was not the ideal way to diagnose craniosynostosis. So, as a nurse and a mom, she knew she would have to travel outside of Key West to find a specialist.
By coincidence, the pediatrician who provided the second opinion often travels to Johns Hopkins All Children’s Hospital in St. Petersburg, Florida, because she has a child with special medical needs. She also did some of her medical training there. So, she called George Jallo, M.D., medical director of the Johns Hopkins All Children's Institute for Brain Protection Sciences and asked him to see Mandy and Adaya.
Mandy quickly learned of Jallo’s reputation as a respected physician in the field of craniosynostosis. She got an appointment with Jallo when Adaya was just 3 weeks old. “I figured if something needs to be addressed, I’d rather do it now instead of playing the waiting game and wondering,” Mandy says.
“What was unique about this case was the diagnosis was made very early in life,” Jallo says. “This provided us with several options for the treatment of craniosynostosis. If you wait until babies are 6-7 months or older you can’t do the minimally invasive surgery that we were able to do in this case.
Through the hospital’s Head Shape Clinic, Jallo says they try to see these patients as soon as possible, so they can get them in the minimally invasive program. Procedures are performed as a team effort with both neurosurgeons and reconstructive plastic/craniofacial surgeons.
The Head Shape Clinic under Matt Smyth, M.D., is organized by the Divisions of Neurosurgery, Plastic Surgery and Rehabilitative Medicine. The physicians are experienced in diagnosing and treating infants and children of all ages with abnormal head shapes related to a wide variety of conditions, most commonly positional plagiocephaly (flat head), but also craniosynostosis and craniofacial syndromes.
It was hard to turn over a 9-week-old to the surgical staff, but before I knew it, Dr. Jallo popped his head in the waiting area and told me they were all done and Adaya did just fine, Mandy says. It could not have been more than 45 minutes. It was amazing.Mandy, Adaya's Mom
“I admit, I cried a lot because I didn’t know what to expect,” Mandy says. “Dr. Jallo put me at ease when he said, ‘This is a bone surgery not a brain surgery. We are doing minimally invasive endoscopic surgery that will be done within an hour. Go home and enjoy your newborn. She’s fine for right now. We will schedule her surgery when she is 9 weeks old.”
“This all took place during the height of COVID,” Mandy says. “I had to do this all alone. Just me and the baby. It was probably the hardest thing I had to do in my life.”
Right on schedule in May 2021, Adaya had her surgery.
“It was hard to turn over a 9-week-old to the surgical staff, but before I knew it, Dr. Jallo popped his head in the waiting area and told me they were all done and Adaya did just fine,” Mandy says. “It could not have been more than 45 minutes. It was amazing. I was surprised Adaya didn’t need any medications except for some Tylenol. And as promised, we went home the next day.”
As part of the minimally invasive surgery protocol, Adaya needed to have helmet therapy, a type of treatment in which a baby is fitted with a special medically designed helmet to correct the shape of the skull. She had to wear this helmet 23 hours a day for almost a year, which created challenges for Mandy and Adaya’s brother, Trae. Every three weeks, Mandy and Adaya would faithfully make the 400-mile drive from Key West to Johns Hopkins All Children’s so doctors could adjust the helmet and measure her head.
“We see craniosynostosis in about 1 out of every 2,000 children,” says Alex Rottgers, M.D., chief of the Division of Plastic and Reconstructive Surgery at Johns Hopkins All Children’s. “In 10-20 percent of the cases, it can result in an increased pressure of the brain, which impacts neurological growth and development.
“It’s important that we identify and treat craniosynostosis in an appropriate time frame,” Rottgers says. “Drs. Jallo, Smyth and I work on these types of cases as a team. I’m the skull surgeon and Dr. Smyth or Jallo are the brain surgeons. It’s the brain surgeon’s job to make sure the brain is healthy and protected and my job as the skull surgeon is to give the baby a larger and more normally shaped skull. This improves the appearance and gives the brain space to grow.”
“We were not doing this type of surgery 15-20 years ago,” Jallo says. “Thanks to the advancements in medical technology, we try to see these babies in our Head Shape Clinic as soon as possible so they can be candidates for this program.
Rottgers believes the team also is doing a better job of educating pediatricians about craniosynostosis, so parents will not have to deal with the confusion and uncertainty that Mandy experienced.
“Mandy being diligent and asking questions allowed us to have the option to perform the minimally invasive procedure using helmet therapy,” Rottgers said. “If we had not seen her until 6-7 months of age, we would have been able to successfully treat her, but it would have been a very different and more complex surgical procedure.
“Always go with your gut,” Mandy says. “If it looks like something is wrong and you can see that the head shape is not right, it’s OK to get a second opinion.”
Today, 16-month-old Adaya, is a happy fun-loving silly little girl,” Mandy says. “Looking at her and watching her play, you could never tell she had craniosynostosis.”