Babies’ heads often look funny after birth. They become misshapen during delivery, only to return to a nice, round shape a week or two later. Yet, sometimes they don’t. That’s when you have to consider craniosynostosis, a birth defect that afflicts approximately 1 in every 2,000 babies. It’s one of the most common conditions treated at the Cleft and Craniofacial Center at Stanford Medicine Children’s Health.
“Immediately after Talia was born, I noticed a ridge down the middle of her forehead,” says her mother, Karina Sanchez. “We knew babies’ heads can be odd at birth, so we didn’t think anything of it. We thought the ridge would go away on its own, but it got more prominent instead.”
Karina works in the medical field, so her antennas went up. She mentioned it to her Central Valley pediatrician, who performed an X-ray and computer tomography (CT) scan—a test that helps doctors diagnose bone, joint, and internal problems. She confirmed craniosynostosis at 2 months of age and recommended that Talia be screened for a genetic syndrome.
The genetic test, scheduled at 6 months, ruled out a syndrome, but the geneticist recommended surgery. It was then that her pediatrician referred Talia to Stanford Medicine Children’s Health.
“When our pediatrician recommended Stanford Children’s, we were relieved. It gave us confidence that she would be in the best hands,” Karina says.
When the craniosynostosis team saw Talia, they confirmed that surgery was indeed needed. With craniosynostosis, a suture (or sutures) in the skull close early and restrict skull growth in the area, which puts pressure on the brain.
“Talia’s story is a very common and classic craniosynostosis story,” says Rohit Khosla, MD, a plastic and reconstructive surgeon and surgical director with the Cleft and Craniofacial Center at Stanford Medicine Children’s Health. “Her metopic suture, which extends down the middle of the forehead toward the nose, was fused, causing triangulation of her forehead and narrowing of the upper portion of her eye sockets.”
The metopic suture is one of six major cranial sutures that connect five large bones of the skull, which come together like large puzzle pieces. In most cases of craniosynostosis, just one suture is fused, but sometimes multiple sutures are fused, demanding a total remodeling of the skull. Luckily, Talia had just one.
While surgery improves the shape of the head, it is about much more than appearance. There are serious medical reasons to have surgery for craniosynostosis.
“Left untreated, her deformity would have gotten worse, as her brain would further expand to the back of her head to compensate for lack of space up front,” Dr. Khosla says. “The long-standing pressure on the front of her brain could have led to developmental delays, chronic headaches, chronic vomiting, and vision changes.”
The pediatric craniosynostosis program is one of just a handful in California. It serves as a referral center for the Bay Area—meaning that area doctors trust it to provide high-quality, advanced care. While craniosynostosis seems rare to pediatricians and the general public, it is commonly seen at Stanford Medicine Children’s Health. The team performs the corrective surgery as often as once or twice a week.
“These can be big operations, but they are very safe, and we get consistently good results,” Dr. Khosla adds.
The craniosynostosis team is made up of multiple pediatric specialists—neurosurgeons, plastic surgeons, neuro-anesthesiologists, geneticists, and ophthalmologists—and each specialist weighs in to formulate the ideal plan for every child, along with the parents. In addition, the genetics team performs sophisticated testing for gene mutations, staying ahead of the curve on newly discovered mutations that relate to the condition.
“We really appreciated how the doctors included us in their huddle and helped us make our decision as informed parents,” says Henry Sanchez, Talia’s father. “The overall care and support from start to finish was second to none.”
At 9 months old, Talia underwent surgery to open her fused suture and shape the forehead bones. It involved all hands on deck, with the neurosurgeon expertly cutting and removing the bone and protecting the brain while the plastic surgeon shaped the bone with special instruments. They then put the puzzle of Talia’s skull back together with absorbable plates and screws.
“It’s critical to have a team that works together regularly because it takes several hands working together in close quarters to do it precisely and safely,” says Kelly Mahaney, MD, pediatric neurosurgeon and interim chief of pediatric neurosurgery at Stanford Medicine Children’s Health.
Talia’s surgery was a complete success. There was a remarkable difference in the appearance of her forehead immediately after surgery.
“For me, all sense of time stopped during the surgery,” Henry says. “The team was really good at letting us know how she was doing, and very quickly we had her back in our sights.”
Before the surgery, the Sanchezes learned all they could about craniosynostosis. They joined a Facebook group of parents, which really helped them get a firsthand account of what to expect.
“Our advice to parents is plug yourself into a support group. Craniosynostosis is scary, so it helps to be supported by others,” Karina says. “And trust the doctors. They do this every day,” Henry adds.
Social services at Stanford Medicine Children’s Health set up the couple at the Ronald McDonald House so they could be near Talia at all times before and after the surgery.
Catching craniosynostosis early is best. When babies are young, doctors can perform a simpler, less-invasive procedure called endoscopy. Yet a referral at any age is important for maintaining a child’s healthy development.
“Dr. Mahaney said it was getting snug in there,” Henry says. “If we hadn’t acted when we did, there would have been pressure on her brain. She sat up and crawled right away after surgery, so I feel like the surgery did help her brain.”
It has been six months since the surgery, and 15-month-old Talia is taking on the world. She’s mimicking her parents, blowing kisses, waving, and eating everything in sight. Her joy of life, along with her wild, thick hair that covers her surgery scar, is helping her parents close the chapter on craniosynostosis.
Learn more about craniosynostosis care at Stanford Medicine Children’s Health and see before-and-after pictures of Talia under the heading “Metopic craniosynostosis.”
