Neurology & Neurosurgery

The Cleft and Craniofacial Center at Children’s of Alabama is one of the busiest in the country, with some of the most experienced physicians and support staff. From cleft palate to craniosynostosis (a condition in which the skull fuses too early) and complex tumor surgeries, the center draws patients from the entire Southeast region and beyond. It is a truly multidisciplinary group with neurologists, neurosurgeons, plastic surgeons, and a craniofacial pediatrician. 

It also offers state-of-the art therapies, including a new type of endoscopic surgery for craniosynostosis in infants as young as three months that is only performed in a few centers in the U.S. “The typical procedure is an endoscopic release of the craniosynostosis followed by post-operative helmet therapy,” said neurosurgeon James M. Johnston, M.D. “Helmet therapy works well, but kids have to wear it for 23 hours a day, and that can be a lot of work for families, especially when they live far from Birmingham,” he said. In addition, the Alabama Medicaid program, which covers most of these children, does not pay for the helmets, which can cost thousands of dollars (and children often need more than one). This puts tremendous financial strain on many families.  

So Dr. Johnston, joined by neurosurgeon Curtis J. Rozzelle, M.D., and plastic surgeons Rene’ P. Myers, M.D., and John Grant, M.D., brought spring-mediated cranioplasty, which was developed at Wake Forest University, to Children’s. It starts with the same endoscopic craniectomy used for children who would require helmets. Only in this procedure, the plastic surgeon steps in and inserts custom-made springs into the bony defect created by the surgery. The springs work to expand the skull over several months to correct the abnormal head shape and ensure appropriate cranial volume for brain growth. A few months later, the plastic surgeon removes the springs during a same-day surgery.  

“What’s nice is that there’s no need for a helmet,” Dr. Johnston said. Plus, studies show the procedure is just as safe and effective as cranioplasties requiring helmets.¹ It’s also covered by all health insurance. “So, we’re able to do it for all children,” he said.  

A similar procedure using cranial distractors like those used to lengthen femurs is used for skull expansion, explained Dr. Grant. This technology is used in older children who need more intracranial volume but who are beyond the age at which the skull can form new bone to fill in surgically created soft spots. By “stretching” the bones of the skull more slowly, he said, the child’s body adjusts by making bone to fill in the growing gap. 

Regardless of the procedure used, early referrals are critical for these babies, said Dr. Rozzelle. “If we can see them by 2 months of age, that gives us plenty of time to get whatever preoperative assessments we need and get them on the schedule so that either the spring or endoscopic craniectomy with subsequent molding helmet is a viable option,” he said. Older babies cannot be treated endoscopically and require standard open surgery, which may lead to more blood loss and longer hospital stays.² 

Yet the craniofacial clinic still sometimes sees babies 6 months or older who never received a diagnosis or whose pediatrician didn’t refer them to Children’s. “That’s frustrating,” Dr. Rozzelle said. 

Nonetheless, said Dr. Myers, “Since we are comfortable with all of the techniques, we can tailor a plan to the individual child. No one is exactly the same.” 

¹ Arko L, Swanson JW, Fierst TM, et al. Spring-mediated sagittal craniosynostosis treatment at the Children’s Hospital of Philadelphia: technical notes and literature review. Neurosurg Focus. 2015 May;38(5):E7

² Hashim PW, Patel A, Yang JF, et al. The effects of whole-vault cranioplasty versus strip craniectomy on long-term neuropsychological outcomes in sagittal craniosynostosis. Plast Reconstr Surg 134:491–501, 2014.

If you’re going to conduct surgery on the brains of children with severe epilepsy, you better know what type they have, where they have it, and how it affects function.  

That’s where functional imaging comes in, including single-photon emission computerized tomography (SPECT), functional MRI (fMRI), positron emission tomography (PET), and magnetoencephalography (MEG). Most neurosurgical centers have one or two; but Children’s of Alabama has them all.  

“This is important,” said pediatric neurosurgeon Jeffrey P. Blount, M.D., “because there is never perfect alignment between the studies.” With multiple studies, however, comes greater certainty about the brain regions the disease impacts, which provides greater certainty about which parts to remove during surgery. Agreement between the scans is called “concordance,” and it is the central concept in epilepsy localization, said Dr. Blount.  

Most patients who require epilepsy surgery also require an invasive monitoring system prior to surgery, said neurosurgeon Curtis J. Rozzelle, M.D. In the past, he explained, that required an open cranial exposure to place electrodes on the surface of the brain and, sometimes, within the brain. 

But with newer techniques, particularly stereoelectroencephalography (SEEG), a minimally invasive surgical procedure used to precisely find the areas of the brain where seizures originate, surgeons can place an array of depth electrodes without performing a craniotomy. Instead, each electrode is placed robotically through a tiny hole drilled in the skull using a robotic stereotactic approach. “That relies very heavily on high-resolution scans,” Dr. Rozelle said, including fusing CT and MRI images, to put the electrodes in without damaging a critical part of the brain. 

“Mostly what we’re trying to avoid is hitting blood vessels with the depth electrodes while getting an array of electrodes that will cover the area of interest,” Dr. Rozelle said. The functional imaging studies are critical in establishing the target zones. Plus, since MEG and fMRI are based on magnetic field fluctuations, the MEG images can be mapped onto the MRI scan in three dimensions. The older technique, in which electrodes were placed on the surface of the brain, only provided a two-dimensional image. 

The child spends several days with the implanted electrodes to capture data about the seizures, which a neurologist then analyzes to identify the exact area of the brain that requires treatment. That surgery itself also relies heavily on high-resolution imaging. A laser ablation, for instance, is performed in the MRI scanner. A larger-volume surgery that requires open resection also relies on imaging because the surgical target looks the same as the normal brain. “To help us ensure that we hit the target, we can map the neurologist analysis into a navigation system that directs us to the right area,” Dr. Rozzelle said. “That ensures that we remove the tissue we need to take out and keep everything else intact.” 

Neurosurgeons at Children’s perform about 50 cranial epilepsy procedures a year, of which about 30 require the invasive monitoring. 

“We are very fortunate to work in a center where we have so much high-quality functional imaging available on a single campus,” said Dr. Blount. 

Children’s of Alabama neurosurgeon Brandon Rocque, M.D.

It’s not surprising that kids with brain tumors and their parents experience a significant amount of stress and psychological distress during the acute post-diagnosis period. It even has a name: pediatric medical traumatic stress. As Children’s of Alabama neurosurgeon Brandon Rocque, M.D., studied this phenomenon a few years ago, it occurred to him that it would almost certainly apply to children with hydrocephalus. 

“We know that just encountering doctors or the medical system can be traumatic for children,” said Dr. Rocque. “For children, just coming to the hospital can be traumatic enough to trigger post-traumatic stress disorder,” or PTSD. 

Numerous factors contribute to stress, particularly the perceived threat to the child’s life. “Even if there isn’t a threat, the child perceives it as such,” Dr. Rocque said. Add to that separation from their parents, uncertainty about the outcome, and the unpredictability of a serious medical condition. “That describes hydrocephalus extremely well,” he said, because these children are treated with shunts that could become blocked at any time requiring additional medical interventions.  

Symptoms of shunt failure can vary widely. Some children simply have a mild headache; other patients can become extremely sick and be in danger of death within a couple of hours. By age 10, “the average child [with hydrocephalus] has had at least two shunt replacements. This is always hanging over the families,” Dr. Rocque said, putting them and their children at high risk for PTSD. 

To test his hypothesis, Dr. Rocque introduced a screening survey into the hydrocephalus clinic to screen for PTSD as well as anxiety, depression, fatigue and resilience. “We found that, overall, the kids with hydrocephalus are doing pretty well. But the parents are not doing so well,” he said. About one in five parents met the diagnostic criteria for PTSD based on their symptoms. More than half attributed it to their child’s condition.  

So why aren’t the kids as affected? One reason, Dr. Rocque said, is that the children don’t know anything different. They’ve lived their entire lives with the condition and the shunts. “But for parents, there was always something new and the risk that something bad is going to happen to their child,” he said.  

Not all the kids surveyed were fine, however. “Some had issues with PTSD, and those were the ones coming to the hospital more. Those whose shunts weren’t behaving well,” Dr. Rocque said. “We need to be aware that these kids have a higher risk for PTSD.”  

They also found that the children and their patients tested exhibited very little resilience, which can help protect against PTSD.  

A survey conducted in conjunction with the Hydrocephalus Association confirmed their findings.  

Dr. Rocque and his team are now working with the association to develop a program to help reduce the risk of PTSD in patients and their families and with a psychologist who is also the mother of an adult with hydrocephalus to develop a tool to help build resilience in patients and their families.  

“This is the first time anyone has really focused on the psychological comorbidities of this condition,” Dr. Rocque said. “I think it has the potential to have a big effect in our population.” 

Pediatric neurointerventional radiology is a small but growing specialty, one increasingly in use given the growing number of endovascular procedures performed in children with neurovascular conditions. “It’s a niche specialty,” says Jesse Jones, M.D., Children’s of Alabama Chief of Neurointervention. “A lot of doctors don’t know about it—let alone patients.”  

Dr. Jones is part of the hospital’s vascular anomalies team, one of the largest pediatric vascular anomalies programs in the Southeast and the only one in Alabama. He works with an interdisciplinary team of experts specializing in the diagnosis, treatment and ongoing care of all vascular anomalies and is part of the team’s monthly clinic. 

On the adult side, neurointerventional radiologists spend a lot of time removing blood clots from stroke patients. But stroke is rarer in children. The hospital’s neurosurgeons and neurologists more often call on Dr. Jones to evaluate congenital anomalies, including vein of Galen malformation or arteriovenous malformations (AVM), as well as inflammatory disorders like vasculitis or obliterative vasculopathy. “It’s when a child presents with dangerous or unusual neurovascular findings and the team is trying to characterize it and plan future treatment that I come in,” he said. 

Dr. Jones, who completed a residency and two fellowships, uses minimally invasive techniques to diagnose and treat numerous neurovascular conditions, including stroke and AVM, but also aneurysms, and lympho-vascular proliferations of the head and neck. The beauty of his approach is that it helps avoid open incisions, reducing the risk of complications and enabling kids to go home sooner. 

His interest in pediatric medicine started with his grandfather, who was a pediatrician. “I looked up to the work he did treating children,” Dr. Jones said. “Working with adults can get frustrating because many conditions they have could have been avoided with lifestyle changes. But in children, they bear no responsibility.” 

Dr. Jones also knew he wanted to do something with the brain. “I’m fascinated with how the brain works,” he said. “It’s a miraculous organ and even after all these years of study still a bit of an enigma.” Being involved in a neuroscience-related field and interacting with other specialists who study the brain is intellectually stimulating, he said. “It’s the best of both worlds: I get to use my hands as an interventional radiologist and work with the brain too.” 

And, of course, work with children.