Snapshot Reveals Rare Skull Condition in Toddler

Quanisha Williams loves taking pictures of her children and placing them in a scrapbook at each birthday. While looking through snapshots from her youngest son’s first year, more came up than just good memories. She noticed an obvious change in his head shape over time.

“When Nazir was born, he had a perfectly round head. By his first birthday, he had what looked like two knots on the side of his head, giving it a peanut shape,” says Quanisha.

She asked Nazir’s pediatrician about it at a well-child visit, but the doctor wasn’t concerned. Luckily, Quanisha had a chance to see another pediatrician, who noticed his head shape right away and ordered a CT scan. The CT scan showed that the cranial sutures in Nazir’s skull had closed too early, typical of a rare and sometimes serious condition called craniosynostosis. The doctor suspected that it might be causing delays in Nazir’s growth.

The bones that make up a baby’s skull are connected with sutures—fibrous material that allows the skull to enlarge evenly to accommodate a growing brain. While the metopic suture, which extends down the middle of the forehead toward the nose, typically closes during the first year of life, the remaining cranial sutures stay open to accommodate skull growth throughout childhood. All of the cranial sutures should be open (not fused) at birth.

Six major cranial sutures connect five large bones that come together at the top and sides of the skull. With craniosynostosis, babies can have a single suture that closes early, or multiple sutures that close early, restricting skull growth in the area of the prematurely closed suture and putting pressure on the brain.

“Nazir is unique in that he was diagnosed later than usual. Craniosynostosis commonly starts when a baby is in utero, so doctors often notice a misshapen head right at birth or in the first few weeks of life,” says Rohit Khosla, MD, a craniofacial surgeon who worked in tandem with Kelly Mahaney, MD, a pediatric neurosurgeon, to perform surgery on Nazir’s skull.

Nazir’s perfectly round head at birth defied the usual pattern, making doctors speculate that he might have had a rare form of craniosynostosis that shows up later. Craniosynostosis can cause babies to be irritable, cry in a high-pitched way, act sleepy, have difficulty feeding, develop seizures, have bulging eyes, experience problems with vision, experience projectile vomiting, and experience developmental delays. Yet Nazir never showed any of these signs. He has always been a happy, energetic boy who never hesitates to share his beautiful smile.

“When I got the news, I thought I was going to drop. But then I got to work googling and learning all about it. I saw babies with the same head shape as my baby, and I read about the surgery to fix it,” Quanisha says.

That’s when the mom of five from Madera, California, contacted Stanford Children’s Health. She met with Dr. Mahaney and Dr. Khosla, who are part of the multidisciplinary craniosynostosis care team. The craniosynostosis program at Stanford Medicine Children’s Health is one of a handful of centers across the country that bring together a multidisciplinary team of experts—neurosurgeons, plastic and reconstructive surgeons, geneticists, and even ophthalmologists—to work together to provide holistic care for children with complex needs.

“Nazir had multiple sutures that closed early. His craniosynostosis was unique in that it involved several growth plates, not just one, requiring two surgeries,” Dr. Mahaney says. “It really helped to have a mom who was so well informed and able to understand the complexities of his needs.”

To perform Nazir’s staged surgeries, Dr. Mahaney literally took Nazir’s skull apart while carefully protecting the covering of his brain. Dr. Khosla then reshaped Nazir’s skull bones and attached them into a normal shape with absorbable plates and screws. Bone grafts created space for Nazir’s growing brain that naturally filled in as he grew.

“It’s most common for a baby to have one area that’s fused, not several. Nazir basically had a total skull remodeling, which is very unusual,” Dr. Khosla says. “He is thriving and developing well, and we couldn’t be more pleased with the results.”

Approximately one in every 2,000 newborns develops craniosynostosis. When it is diagnosed early, ideally during the first month or so of life, surgery for craniosynostosis can be simpler and less invasive, if it involves only one prematurely fused suture. When it is left untreated, pressure can damage the optic nerve that connects the brain to the eyeballs. Luckily, Nazir’s vision is fine and has not been affected.

“The first time I walked into Stanford Children’s, it felt warm and welcoming. I thought, ‘OK, I can trust you guys with my baby,’” Quanisha says.

Her large family took turns showing up at the hospital to be with Nazir and Quanisha while he underwent each of his two surgeries. Doctors say he handled the surgeries like a champ. He went into surgery with a smile and came out with a smile.

“I completely trusted the doctors and everyone who cared for Nazir. He was in good hands,” Quanisha says. “Plus, the team was so energetic and friendly, down to the librarian and the front desk person.”

Dr. Mahaney encourages parents who notice changes in their child’s head shape to see their pediatrician and, if needed, seek more care. Getting care early is important to avoid developmental delays and to test for syndromes that are related to craniosynostosis.

“Craniosynostosis is a condition that’s rare for pediatricians to encounter in their day-to-day practice, but common for pediatric neurosurgeons. A pediatrician might see only a handful of children with craniosynostosis in their lifetime—making it easy to miss—while we see it every week,” Dr. Mahaney says. “I encourage parents to seek a second opinion from us if they are concerned.”

Today, Nazir is back to his bouncy, active self. He loves playing cars and trucks, but his favorite activity is basketball. At three and a half years old, he already knows how to dribble. Dr. Khosla appreciates Nazir’s happy, outgoing personality. Despite what he’s been through, Nazir is always smiling during follow-up appointments, eager to climb up on Dr. Khosla’s lap.

“His head is once again that beautiful round shape. We couldn’t be happier with the results, and we are so grateful for all the great care we received,” Quanisha says.