Hugo and Nicole’s infant daughter, Priscella, came to the Johns Hopkins All Children’s Head Shape Clinic because of a tightening of the neck muscles that cause a child to tilt her head to one side. Specialists in the clinic commonly see the condition, which is known as torticollis.
“Priscella needed physical therapy, and her family was educated on how to perform the proper neck stretches at home,” says Alex Rottgers, M.D., chief of pediatric plastic surgery at Johns Hopkins All Children’s Hospital. “In her case, however, torticollis was the minor problem.”
The specialists in the Head Shape Clinic immediately recognized a less common problem. Priscella had craniosynostosis, an abnormal fusion of cranial sutures that can cause eye and brain injuries, learning deficits, blindness and seizures due to restricted brain growth.
“They knew as soon as they saw her,” Nicole says. “They did a CT scan to confirm the diagnosis and scheduled her for surgery three months later.”
A Pressing Issue
Priscella, then 6 months old, was put on a regimen of iron supplements in preparation for the surgery. In the meantime, Hugo and Nicole brought her to Johns Hopkins All Children’s biweekly for physical therapy sessions, which helped resolve her neck stiffness.
“Communication with providers was strong during every visit,” Hugo says. “It’s a parent’s worst nightmare when a child has to go through surgery, but it was comforting to know that our child was in good hands.”
Johns Hopkins All Children’s has been performing surgery for craniosynostosis for many decades, including minimally invasive techniques for more than 15 years.
“Priscella is the poster child for why we have the Head Shape Clinic. The clinic is designed to provide a streamlined service for families of children with positional head shape deformities, but it also provides us with an opportunity to ensure that complex cases such as hers don’t go undiagnosed and untreated. We aren’t just providing helmets for flat heads. We’re providing a full evaluation and specialized care for each patient. ”
Alex Rottgers, M.D. Chief of pediatric plastic surgery at Johns Hopkins All Children’s
Minimally invasive surgeries typically allow for smaller incisions, and shorter procedures with less need for blood transfusions and a shorter hospital stay, but they require the use of an orthotic helmet for many months after the procedure to mold the growth of the infant’s head. For some parents, traditional open surgery may be preferred if they are not able or do not wish to use a helmet for their child. Regardless of preference, minimally invasive surgery is only available for children younger than 3 to 4 months of age.
“In the past, pediatricians were educated to observe infants suspected of having craniosynostosis for a while to see if the head shape would normalize on its own,” says Jerry Tuite, M.D., pediatric neurosurgeon at Johns Hopkins All Children’s. “When traditional open procedures are performed, that paradigm works well because there is no urgency to perform the surgery in the first few month of life. However, that period of observation can limit options now that minimally invasive techniques are available. Because minimally invasive surgery is only possible in infants, we now prefer to see the babies as soon as possible after birth, so we can explore all the possible surgical options. That need for prompt referral was one of our motivations for starting the Head Shape Clinic. We are more than happy to see any baby with an unusual head shape early on if craniosynostosis is suspected.”
Because Priscella was 6 months old when she was diagnosed with craniosynostosis, Tuite and Rottgers recommended she undergo open surgery three months after her initial appointment.
Priscella’s procedure was scheduled for Feb. 14.
Both a neurosurgeon and a plastic surgeon specially trained in craniofacial surgery are required to perform a fronto-orbital advancement, the procedure that Priscella was slated to receive on Valentine’s Day.
“We work as a team to remodel the bones of the skull and upper part of the face to provide more space for the brain to grow and to protect the eyes,” Rottgers says. “We also improve the shape of the bones of the skull to improve symmetry, function and appearance.”
Priscella’s surgery took five hours to complete and was performed by Rottgers and Tuite. The procedure was successful, and Priscella was discharged three days later. She returned for regular postoperative visits and will continue to receive longitudinal care from Johns Hopkins All Children’s Cleft and Craniofacial Center, which will monitor her facial growth and intracranial pressure.
“We will give her all the specialties she needs from now until she is an adult,” Rottgers says. “The majority of children with abnormal head shapes have changes to their skull that resulted from sleeping habits, intrauterine constriction, and torticollis. These children don’t require surgery, but our Head Shape Clinic focuses on improving care for these patients. Children are seen by a craniofacial surgeon or pediatric neurosurgeon to diagnose the problem and educate the family.
"In every Head Shape Clinic, we also have physical therapists and orthotists, who make helmets to treat children with the most severe positional head shape differences," Rottgers continues. "We designed the clinic to have all the resources these patients need. It’s very rewarding to reassure these families their child does not need surgery and that we can provide them immediately with the services they need to treat the abnormal head shape. When we identify patients like Priscella, with craniosynostosis, the Head Shape Clinic is a gateway into our Cleft and Craniofacial Center, where children receive multidisciplinary team care for the rest of their childhood.”
Priscella resumed her physical therapy for torticollis shortly after her surgery and is now down to one session a week. Instead of focusing solely on neck stretches and exercises, Hugo and Nicole are teaching their daughter how to crawl and eventually walk—normal milestones for any baby and parents.
“I still don’t believe my child looks the way she does,” Hugo says. “The doctors and staff are miracle workers. They did wonders for my daughter.”
Head Shape Clinic at a Glance
Primary specialties involved: Pediatric neurosurgery, plastic surgery and orthotics
Clinic availability and locations: The clinic is held regularly at four Outpatient Care locations: Main Campus at 601 Fifth St. South in St. Petersburg; 12220 Bruce B. Downs Blvd. in Tampa; East Lake at 3850 Tampa Road in Palm Harbor; and 4550 Colonial Blvd. in Fort Myers.
Primary treatments available: We address multiple types of head shape deformities through the use of individualized treatment plans that may include physical therapy, surgery, orthotics and more.
Referral required? Yes, a child’s primary care provider must refer him or her.
For more information: Patients or referring physicians should call 727-767-8181.