Antonio R. Porras, Ph.D., a staff scientist in the Sheikh Zayed Institute for Pediatric Surgical Innovation at Children’s National Health System, has received the prestigious Pathway to Independence Award from the National Institutes of Health (NIH). This award funds Dr. Porras’ research for the next five years, enabling him to develop two bone growth models that will better inform clinicians treating patients with craniosynostosis and help to optimize outcomes. Also referred to as the K99/R00 grant, this NIH award is for researchers who are either in the postdoctoral/residency period or who are early career investigators. It is designed to transition them from mentored positions to independent, tenure-track or equivalent faculty positions so that they can launch competitive research careers.
Marius George Linguraru, D.Phil., M.A., M.Sc., a principal investigator in the Sheikh Zayed Institute for Pediatric Surgical Innovation, is Dr. Porras’ primary mentor on this research project along with co-mentors Robert Keating, M.D., division chief of neurosurgery at Children’s National, and Maximilian Muenke, M.D., chief in the Medical Genetics Branch at the National Human Genome Research Institute.
Dr. Porras has taken a research interest in craniosynostosis, the early fusion of one or more cranial sutures that may lead to craniofacial malformations and brain growth constraints during childhood. With this NIH K99/R00 award, Dr. Porras will employ his expertise in computer science, biomedical engineering, quantitative imaging and statistical modeling to create a personalized computational predictive model of cranial bone growth for subjects without cranial pathology and for patients with craniosynostosis. Dr. Porras will also quantify the coupled growth patterns of the cranial bones and the brain using an existing brain growth model.
Affecting one in 2,100 to 2,500 live births, craniosynostosis complications can result in elevated intra-cranial pressure and subsequent impaired brain growth. While treatable, optimal outcomes are stymied by subjectivity in the evaluation of cranial malformations and prediction of cranial bone development. There are currently no personalized clinical tools available to predict healthy or pathological cranial growth and no objective techniques to optimize the long-term outcome of treatment for patients with craniosynostosis.
Children’s National in Washington, D.C., is the nation’s No. 6 children’s hospital and, for the third year in a row, its neonatology program is No.1 among all children’s hospitals providing newborn intensive care, according to the U.S. News Best Children’s Hospitals annual rankings for 2019-20.
This is also the third year in a row that Children’s National has been in the top 10 of these national rankings. It is the ninth straight year it has ranked in all 10 specialty services, with five specialty service areas ranked among the top 10.
“I’m proud that our rankings continue to cement our standing as among the best children’s hospitals in the nation,” says Kurt Newman, M.D., President and CEO for Children’s National. “In addition to these service lines, today’s recognition honors countless specialists and support staff who provide unparalleled, multidisciplinary patient care. Quality care is a function of every team member performing their role well, so I credit every member of the Children’s National team for this continued high performance.”
The annual rankings recognize the nation’s top 50 pediatric facilities based on a scoring system developed by U.S. News. The top 10 scorers are awarded a distinction called the Honor Roll.
“The top 10 pediatric centers on this year’s Best Children’s Hospitals Honor Roll deliver outstanding care across a range of specialties and deserve to be nationally recognized,” says Ben Harder, chief of health analysis at U.S. News. “According to our analysis, these Honor Roll hospitals provide state-of-the-art medical expertise to children with rare or complex conditions. Their rankings reflect U.S. News’ assessment of their commitment to providing high-quality, compassionate care to young patients and their families day in and day out.”
The bulk of the score for each specialty is based on quality and outcomes data. The process also includes a survey of relevant specialists across the country, who are asked to list hospitals they believe provide the best care for patients with challenging conditions.
Below are links to the five specialty services that U.S. News ranked in the top 10 nationally:
- Neonatology (No. 1), led by Division Chief Billie Lou Short, M.D.
- Neurology and Neurosurgery (No. 5), led by Division Chiefs Roger J. Packer, M.D., and Robert F. Keating, M.D.
- Nephrology (No. 6), led by Division Chief Marva Moxey-Mims, M.D., FASN
- Cancer (No. 9), led by Division Chief Jeffrey S. Dome, M.D., Ph.D., and
- Pulmonology and lung surgery (No.9), led by Division Chief Anastassios Koumbourlis, M.D., MPH
About one in 26 people will be diagnosed with epilepsy in their lifetime. That adds up to about 3.4 million people in the U.S., or about 1 percent of the population nationwide. This condition can have huge consequences on quality of life, affecting whether children will learn well in school, eventually drive a car, hold down a job or even survive into adulthood.
For most of those that develop epilepsy, medications can keep seizures in check. However, for about a third of patients, this strategy doesn’t work, says Chima Oluigbo, M.D., an attending neurosurgeon at Children’s National Health System. That’s when he and his team offer a surgical fix.
Epilepsy surgery has come a long way, Dr. Oluigbo explains. When he first began practicing in the early 2000s, most surgeries were open, he says – they involved making a long incision in the scalp that can span half a foot or more. After drilling out a window of skull that can be as long as five inches, surgeons had to dig through healthy brain to find the abnormal tissue and remove it.
Each part of this “maximally invasive” procedure can be traumatic on a patient, Dr. Oluigbo says. That leads to significant pain after the procedure, extended hospital stays of at least a week followed by a long recovery. There are also significant risks for neurological complications including stroke, weakness, paralysis, speech problems and more.
However, open surgery isn’t the only option for epilepsy surgery anymore. Several new minimally invasive alternatives are now available to patients and the most promising, Dr. Oluigbo says, is called Visualase. He and his team are the only surgeons in the region who perform this procedure.
In Visualase surgeries, Dr. Oluigbo and his colleagues start by making a tiny incision, about 5 millimeters, on the scalp. Through this opening, they bore an even tinier hole into the skull and thread a needle inside that’s about 1.6 millimeters wide. “The brain barely notices that it’s there,” he says.
The tip of this wire holds a laser. Once this tip is placed directly at the seizure foci – the cluster of nerve cells responsible for generating a seizure – the patient is placed in an intraoperative magnetic resonance imaging (MRI) device. There, after checking the tip’s precise placement, the surgeons turn the laser on. Heat from the laser eradicates the foci, which the surgeons can see in real time using MRI thermography technology. The margins of the destroyed tissue are well-defined, largely sparing healthy tissue.
After the wire is removed, the incision is closed with a single stitch, and patients go home the next day. The majority of patients are seizure free, with rates as high as 90 percent for some types of epilepsy, Dr. Oluigbo says. Although seizure-free rates are also high for open procedures, he adds, Visualase spares them many of open surgeries’ painful and difficult consequences.
“Having done both open surgeries and Visualase,” Dr. Oluigbo says, “I can tell you the difference is night and day.”
Although open procedures will still be necessary for some patients with particularly large foci that are close to the surface, Dr. Oluigbo says that Visualase is ideal for treating medication-resistant cases in which the foci are buried deep within the brain. A typical example is a condition called hypothalamic hamartoma, in which tumors on the hypothalamus lead to gelastic seizures, an unusual seizure type characterized by uncontrollable laughing. He also uses Visualase for another condition called tuberous sclerosis, in which waxy growths called tubers develop in the brain, and for cancerous and benign brain tumors.
It’s gratifying to be able to help these children become seizure-free for the rest of their lives, says Dr. Oluigbo – even more so with the numerous updates he receives from families telling him how much this procedure has improved their children’s lifestyle.
“Visualase has completely changed the way that we approach these patients,” Dr. Oluigbo says. “It’s extraordinary to see the effects that this one procedure can have on the quality of life for patients here at Children’s National.”
If a child’s refractory seizures – seizures that don’t respond to medication – are originating in a part of the brain that is central to function (for example, impacting memory or verbal skills) the standard next step – surgical resection – is not an option for seizure reduction or relief. In most cases, these children are followed, more medications are tried, and other strategies attempted, but few viable options exist to ease their symptoms.
It’s possible that the next generation of implantable neurostimulators, which act as a type of pacemaker for the brain, might make a difference for some children previously left with no answers. Children’s National neurosurgeon Chima Oluigbo, M.D., in collaboration with the Comprehensive Pediatric Epilepsy Program at Children’s National, is looking at how these devices might be used to reduce or eliminate refractory seizures in pediatric patients. One example of this type of device is the RNS System.
“The RNS has been FDA approved for adults since 2013,” says Dr. Oluigbo, who recently implanted a NeuroPace RNS in the first pediatric patient at Children’s National, and one of the first young patients in the country. “The safety and efficacy data in the adult population, now gathered from a cohort of more than 800 adults, is showing positive outcomes so far. That allows pediatric neurosurgeons to consider an off-label use of this device for patients under the approved usage age of 18, when no other treatments exist.”
The RNS operates differently from previous neuro pacemaker-style devices. It is a “closed-loop” system that doesn’t require external activation once a seizure has started. Instead, the precise location of seizure origination is identified via functional magnetic resonance imaging (fMRI). Leads are then placed at the seizure site via surgery, and once activated, the RNS monitors and self-activates when pre-seizure electrical impulses are detected. The device responds by emitting a series of its own electrical impulses to interrupt and reset the brain’s seizure activity. The RNS system’s ability to continuously monitor the patient also allows physicians to get an inside look at the ongoing brain function of these young patients.
“Children’s National is one of the first places to apply the use of this device in children, because we are one of the few locations on the East coast with the multi-disciplinary expertise to implement it safely and effectively,” says Dr. Oluigbo. “Our clinical epilepsy team has been imaging and treating children with epilepsy for almost 30 years. With one of the oldest neurosurgical programs in country and our technological capabilities, Children’s National becomes the perfect location to explore how technology like this can improve the quality of life for our patients, many of whom have previously been told there is nothing more we can do to help.”
De-personalized data from patients who receive the NeuroPace RNS will be shared with the company in the hopes that the data will assist the FDA in assessing the appropriateness of extending the age range of approval from 18 and above to 12 and above.
“Our hope is to contribute to the body of data about this device and determine if it will improve the lives of our younger patients the way it has already been done for adults,” Dr. Oluigbo concludes. “Kids’ brains may respond differently, however, sharing our patients’ experiences and outcomes will help us identify whether or not this is a viable and promising option for more children with refractory epilepsy.”
Children’s National rose in rankings to become the nation’s Top 5 children’s hospital according to the 2018-19 Best Children’s Hospitals Honor Roll released June 26, 2018, by U.S. News & World Report. Additionally, for the second straight year, Children’s Neonatology division led by Billie Lou Short, M.D., ranked No. 1 among 50 neonatal intensive care units ranked across the nation.
Children’s National also ranked in the Top 10 in six additional services:
- Neurology and Neurosurgery (No. 5), led by Roger Packer, M.D., and Robert Keating, M.D.
- Nephrology (No.6), led by Marva Moxey-Mims, M.D., FASN
- Cancer (No. 7), led by Jeffrey Dome, M.D., Ph.D.
- Orthopedics (No. 8), led by Matthew Oetgen, M.D.
- Pulmonary (No. 9), led by Anastassios Koumbourlis, M.D., M.P.H., and
- Diabetes and Endocrinology (a tie for No. 10), led by Fran Cogen, M.D., acting division co-chief
For the eighth year running, Children’s National ranked in all 10 specialty services, which underscores its unwavering commitment to excellence, continuous quality improvement and unmatched pediatric expertise throughout the organization.
“It’s a distinct honor for Children’s physicians, nurses and employees to be recognized as the nation’s Top 5 pediatric hospital. Children’s National provides the nation’s best care for kids and our dedicated physicians, neonatologists, surgeons, neuroscientists and other specialists, nurses and other clinical support teams are the reason why,” says Kurt Newman, M.D., Children’s President and CEO. “All of the Children’s staff is committed to ensuring that our kids and families enjoy the very best health outcomes today and for the rest of their lives.”
The excellence of Children’s care is made possible by our research insights and clinical innovations. In addition to being named to the U.S. News Honor Roll, a distinction awarded to just 10 children’s centers around the nation, Children’s National is a two-time Magnet® designated hospital for excellence in nursing and is a Leapfrog Group Top Hospital. Children’s ranks seventh among pediatric hospitals in funding from the National Institutes of Health, with a combined $40 million in direct and indirect funding, and transfers the latest research insights from the bench to patients’ bedsides.
“The 10 pediatric centers on this year’s Best Children’s Hospitals Honor Roll deliver exceptional care across a range of specialties and deserve to be highlighted,” says Ben Harder, chief of health analysis at U.S. News. “Day after day, these hospitals provide state-of-the-art medical expertise to children with complex conditions. Their U.S. News’ rankings reflect their commitment to providing high-quality care.”
The 12th annual rankings recognize the top 50 pediatric facilities across the U.S. in 10 pediatric specialties: cancer, cardiology and heart surgery, diabetes and endocrinology, gastroenterology and gastrointestinal surgery, neonatology, nephrology, neurology and neurosurgery, orthopedics, pulmonology and urology. Hospitals received points for being ranked in a specialty, and higher-ranking hospitals receive more points. The Best Children’s Hospitals Honor Roll recognizes the 10 hospitals that received the most points overall.
This year’s rankings will be published in the U.S. News & World Report’s “Best Hospitals 2019” guidebook, available for purchase in late September.
While epilepsy affects people of all ages, the unique way it manifests in infants, children and adolescents can be attributed in part to the complexities of the growing and developing brain. Researchers from the Children’s National Comprehensive Pediatric Epilepsy Program brought their expertise on the challenges of understanding and treating epilepsy in children to the recent American Epilepsy Society Annual Meeting, the largest professional gathering on epilepsy in the world.
“We aim to build the evidence base for treatments that are effective specifically for children with epilepsy,” says William D. Gaillard, M.D., chief of Child Neurology, Epilepsy and Neurophysiology, and director of the Comprehensive Pediatric Epilepsy Program. “We have learned much from studies in adult populations but technologies like functional MRI allow us to get in-depth understanding, often in non-invasive ways, of precisely how epilepsy is impacting a child.”
Dr. Gaillard was also recently elected to serve as the Second Vice President of the American Epilepsy Society. “The AES is the largest multidisciplinary professional and scientific society dedicated to the understanding, treatment and eradication of epilepsy and associated disorders, and I am honored to serve as the new Second Vice President,” he said.
The team’s presentations and poster sessions focused on several key areas in pediatric epilepsy:
Better ways to see, measure and quantify activity and changes in the brain for children with epilepsy before, during and after surgery
- Novel applications of fMRI for children with epilepsy
- Evaluation of an fMRI tool that tracks verbal and visual memory in children with epilepsy – one of the first to capture memory functions in this population of children using noninvasive fMRI;
- Early study of the use of “resting-state” fMRI to map language skills before epilepsy surgery – an important first step toward noninvasively evaluating children who are too young or neurologically impaired to follow tasks in traditional MRI studies;
- A study of whether intraoperative MRI, i.e. imaging during neurosurgery, allows for more complete removal of abnormal brain tissue associated with focal cortical dysplasia in children, which is a common cause of intractable epilepsy;
- A preliminary case review of existing data to see if arterial spin labeling MRI, which measures blood flow to the brain, has potential to identify blood flow changes in specific locations of the brain where seizures occur;
- An analysis of language laterality – the dominant side of the brain controlling language – questioning the true reasons that the brains of children with epilepsy have differences in the hemisphere that predominantly controls language;
- A review of some common assessments of language and working memory that are used pre- and post-operatively to gauge the impacts of pediatric epilepsy surgery. The study found that using multiple assessments, and studying results individually rather than as a group average, resulted in a more complete picture of the outcomes of surgery on these areas of brain function;
- A preliminary study examining whether continuous EEG monitoring of neonates with hypoxic ischemic encephalopathy, or lack of oxygen to the brain, can be a reliable predictor of neurodevelopmental outcomes while the infant is undergoing therapeutic hypothermia.
The molecular, genetic and biological factors that contribute to onset and severity of pediatric epilepsy
- A retrospective study of young patients with malformations in cortical development that are important causes of childhood epilepsy;
- Investigation of a simple saliva test to effectively identify the presence of two common viral infections, human herpesvirus-6B and Epstein-Barr virus, that may be contributors to onset of epilepsy in otherwise normally functioning brains;
- A preliminary review of the possible relationship between febrile infection-related epilepsy syndrome and the co-occurrence of another neuro-inflammatory condition – hemophagocytic lymphohistiocytosis.
Madison Berl, Ph.D., director of research in the Division of Pediatric Neuropsychology, and a pediatric neuropsychologist in the Comprehensive Pediatric Epilepsy Program, adds, “In order to expand our understanding of causes, impacts and outcomes, the range of research is broad given the complexity of epilepsy. This is the only way we can contribute to the goal of providing our colleagues and the families they serve with better resources to make informed decisions about how best to assess and treat pediatric epilepsy.”
There was no effective treatment for uncontrolled, difficult, and sometime painful movements associated with movement disorders. That is, before the development of deep brain stimulation (DBS) techniques.
Children’s National Health System is one of only two children’s hospitals with fully integrated DBS programs. Chima Oluigbo, M.D., who leads the pioneering Deep Brain Stimulation Program within Children’s Division of Neurosurgery, is one of few pediatric deep brain stimulation experts in North America and cross-trained in pediatric and functional neurosurgery.
Dr. Oluigbo says the effects of DBS are often dramatic: 90 percent of children with primary dystonia show up to 90 percent symptom improvement.
A 6-year-old boy with dystonia so severe that his body curved like a “C” was one of the first patients to undergo the procedure at Children’s National. Six weeks later, he gained the ability to sit straight and to control his hands and legs. He also was able to smile, an improvement that brought particular joy to his parents.
Inside the brain with movement disorders
Patients with movement disorders experience difficulties due to neurological dysfunction that impact the speed, fluency, quality, and ease in which they move. In these cases, neurons in the brain’s motor circuits misfire. Through the use of DBS, neurosurgeons can synchronize neuronal firing and accomplish the previously impossible: restoring muscle control to patients with these disorders.
Movement disorders are common in children. “It’s not just numbers, it’s also about impact. Think about the potential of a child who is very intelligent and can contribute to society. When that child is not able to contribute because he or she is disabled by a movement disorder, the lost potential is very significant. It has an impact,” Dr. Oluigbo says.
What is deep brain stimulation?
DBS uses an implantable device to send continuous, low-level electrical impulses to areas deep within the brain. The impulses prevent the brain from firing abnormal signals that are linked to movement disorders and seizures. When a child is considered to be a candidate for the technique, here’s what happens next:
- Imaging: Magnetic resonance imaging (MRI) helps pinpoint the area of brain tissue responsible for movement disorders and informs the treatment plans.
- Neurotransmitter implant procedure: Using minimally invasive neurosurgery techniques, doctors access the brain through a tiny incision in the child’s skull and place thin, insulated wires (leads) in the area of brain tissue responsible for the condition.
- Pulse generator implant procedure: The pulse generator (neurostimulator) is a battery-operated device that sends low-level electrical impulses to the leads. During a separate procedure, the pulse generator is implanted near the child’s collarbone. Leads are threaded under the child’s skin to connect with the pulse generator.
- Stimulation treatments: Once the leads and pulse generator are connected, the child receives a continuous stream of electrical impulses. Impulses are generated by the neurostimulator, travel through the leads, and end up in the deep tissue of the brain. Here, they block abnormal signals that are linked to the child’s movement disorder.
- Follow-up care: The child will likely need deep brain stimulation throughout his or her lifetime to make sure the device is working correctly and to adjust the neurotransmitter settings to meet his or her changing needs.
Deep brain stimulation at Children’s National
Children’s National is currently conducting clinical trials seeking to expand the use of this procedure to patients with cerebral palsy, one of the most common dystonias. The effective use of deep brain stimulation requires ongoing attention from a multidisciplinary team (from neuropsychology to rehabilitation medicine), giving seamless care under one roof.
There is evidence to suggest that this technique could be used to aid people with memory disorders, patients in minimally conscious states, and patients with incurable epilepsies.
Pediatric surgeons, physicians, and scientists from around the world are meeting in the nation’s capital Oct. 8 to 11 , for the 5th World Congress of the World Federation of Associations of Pediatric Surgeons (WOFAPS) hosted by The Sheikh Zayed Institute for Pediatric Surgical Innovation at Children’s National Health System. It’s the first time that the meeting will be in North America. This year’s theme is “re-imagining children’s surgery through global innovation and integration.”
The 5th WOFAPS congress includes many scientific and research plenary sessions by pediatric surgical experts from around the world. Eleven Children’s National and Sheikh Zayed Institute surgeons and physicians are participating in panels covering different topics and areas of expertise including:
- Minimally Invasive Surgery: Current State of Endoscopic & Minimally Invasive Bariatric Surgery
- The Current Standards of Management & Controversies in Pediatric Tumors: Neuroblastoma & Wilms Tumor
- Per Oral Endoscopic Myotomy (POEM) Techniques for Pediatric Achalasia: Approach, Techniques & Setting up Program
- Hot Topics in Pediatric Urology: Controversies & Advances