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Monday, September 26, 2011
Advances Fall 2011
CT scan showing deep brain stimulation used for a severe movement disorder in a child.
Pediatric neurosurgery focuses on the surgical treatment of a wide range of brain and spine disorders, many of which remain incompletely understood. The pathophysiology of these disorders is especially complex among pediatric patients because factors such as the location of an injury or abnormality occurring in the brain during a particular stage of the child’s development can greatly influence outcomes. Advances in basic science research on the developing brain, along with technologic advances in surgical and imaging techniques, have opened new opportunities for the treatment of pediatric neurosurgical disorders. Because some of the most promising technologic advances have arisen from disorders commonly encountered in the adult population, an environment in which aspects of collaborative research and clinical care coexist across the age spectrum provides fertile ground for the application of new techniques to the care of children.
MassGeneral Hospital for Children (MGHfC) is a leader in the evaluation and surgical treatment of children with pediatric nervous system disorders, including epilepsy, brain tumors, traumatic brain injuries, spasticity, severe movement disorders, hydrocephalus, and congenital anomalies of the spine and brain. The overall goal of MGHfC’s Pediatric Neurosurgery Program is to first preserve and then improve brain function.
Pediatric neurosurgeons and their colleagues in many specialties at MGHfC care for some of the most severely ill and injured brain and spinal cord patients in the United States and from around the world. Through this unique expertise, they are working to advance surgical treatment options and overall care of children with nervous system problems at all levels of severity.
Brain Injury Research Leads to Better Surgical Planning
Ann-Christine Duhaime, MD, director of pediatric neurosurgery at MGHfC, leads a research team to study certain aspects of brain function and injury in children. This research is focused on learning how the pediatric brain responds to trauma, including the brain’s ability to repair itself and recover function, at different stages of development from infancy through adolescence. Although the team is examining brain function following mechanical trauma, the findings can be translated to trauma of any kind, including that induced by surgical interventions.
Dr. Duhaime’s research has led to a greater understanding of the pediatric brain response to certain types of injury and of the circumstances that optimize recovery. Based on this research, MGHfC’s Pediatric Neurosurgical Program, in conjunction with other specialists, including those in critical care, neurology, radiology, and trauma, is continuously adapting and improving clinical practices. For example, new radiology protocols detect problems early in a patient’s course that can direct interventions, including surgical treatments, to limit brain swelling and compression of vital structures, thus reducing the chance of additional injury.
Continuous Brain Monitoring Improves Detection, Understanding of Seizures
Researchers at MGHfC are closely studying the incidence and effects of subclinical seizures in pediatric patients with serious brain disorders. The researchers have found that many pediatric patients, especially in the early years of life, are prone to this type of subtle seizure activity, which is undetectable without brain monitoring devices. Dr. Duhaime’s neurosurgical team is working closely with Sarah Murphy, MD, pediatric intensivist at MGHfC, to provide continuous brain monitoring of patients with various neurosurgical and neurological conditions in the pediatric intensive care unit (PICU). Under the direction of Dr. Murphy, the PICU secured funding for a continuous EEG monitoring system, which can, using electrodes, track cerebral function and pinpoint these seizures.
Seizures are especially dangerous to young children with neurological problems because continuous or frequent seizures may be particularly toxic to the developing brain. Understanding the prevalence of otherwise undetectable seizure activity may change treatment options for many young patients.
Intraoperative photograph of a hemispheric disconnection in which multiple pathways in the brain are severed to prevent spread of seizures to healthy tissue.
While continuous EEG monitoring is used in many pediatric centers to assess children with known epilepsy, MGHfC is among only a few centers in the country to offer this type of continuous EEG monitoring in the PICU setting for a wide variety of acute illnesses. The PICU is currently developing guidelines for the monitoring system, and the PICU staff has been trained to read algorithms provided by the system at the bedside so action can be taken immediately if a seizure occurs, even if it can’t be detected by observing the patient. In-depth evaluation of the data obtained from monitoring is conducted by a team of specially trained pediatric neurologists at Massachusetts General Hospital. The multidisciplinary team of specialists will study the results of continuous EEG monitoring in a wide PICU population to identify how frequently seizures occur among different diagnoses in the pediatric population, as well as how seizures affect outcomes. Once the frequency is established, the team will track how interventions, such as seizure medications, can benefit these patients and which of these interventions achieve the most favorable response.
Leader in Deep Brain Stimulation for Severe Movement Disorders
Deep brain stimulation (DBS)—a method by which abnormal activity in brain circuitry can be modulated through surgically placed electrodes—is a treatment modality that has been widely used in the adult population for Parkinson’s disease and other movement disorders. More recently, this approach has been used to treat a range of other conditions in adults, including epilepsy, depression, obsessive-compulsive disorder, and Tourette’s syndrome. The modality is adjustable and can be increased, decreased, or even discontinued if needed, which is highly desirable in contrast to older, irreversible surgical techniques in which lesions were made in brain circuits in an effort to restore a more normal balance in movement disorders and other brain conditions. Because of well-established positive outcomes and safety profiles in adults, DBS techniques are now being applied to a wider range of conditions, and DBS is now FDA-approved for certain pediatric movement disorders, including some types of dystonia.
DBS surgery in the pediatric population is still fairly rare in the United States. However, Mass General has extensive experience with this treatment for a variety of adult conditions, and, for this reason, it has become a referral center for pediatric patients with severe, life-threatening movement disorders. Some of these children, who have not responded to efforts to control their dangerous movements, have been transferred to MGHfC from intensive care units at other centers, where continuous sedation has been necessary to prevent ongoing muscle breakdown. To effectively treat these rare patients, MGHfC has gathered a team of multidisciplinary specialists who are experts in DBS in the adult and pediatric populations. The team, which includes specialists from adult and pediatric specialties, including Emad Eskandar, MD, director of the Adult Stereotactic and Functional Neurosurgery Program, and other collaborators from neuroradiology, neurology, and psychiatry, is one of the first multidisciplinary groups in the country focused on DBS for children with severe movement disorders, and is using the experience it has gained to extend DBS treatments to children with other disorders. These include other movement disorders, such as chorea and ballismus, as well as more behavior-related conditions, in which the technology may be beneficial.
Advancing Surgical Techniques for Epilepsy
As part of their mission to preserve and improve brain function, the pediatric neurosurgery and neurology teams at MGHfC are advancing surgical techniques for children with epilepsy. Epileptic seizures are especially detrimental in children because they can interfere with the brain’s ability to learn and grow.
The neuroradiology team is able to use highly specialized magnetic resonance imaging (MRI) to track where seizures arise from abnormally formed regions of the brain. Together with advanced electroencephalography techniques utilized by the child neurology specialists, the team can track how seizures spread. This approach employs diffusion tensor imaging, an MRI technique that shows connections in various brain regions, and magnetoencephalography, an imaging method that maps brain activity. Once the path of seizure activity is understood, MGHfC physicians can decide the surgery best suited to stop seizures in a particular patient.
Hemispheric disconnection, one technique used by neurosurgeons at MGHfC to stop epileptic seizures, involves surgically disconnecting areas of the brain that allow the seizures to spread. By effectively stopping the seizures, the brain has an opportunity to develop and grow. Disconnection procedures are not commonly performed in the United States; MGHfC physicians not only perform these complicated surgeries, but they are working to advance these surgical techniques by using a personalized combination of anatomic, functional, and electroencephalographic data.
Improving Outcomes with Intraoperative Monitoring
To perform such complex brain procedures and protect brain function during surgery, Dr. Duhaime established a new team of collaborators to provide sophisticated intraoperative neurophysiologic monitoring. The team employs various electrophysiological methods to monitor brain and nerve function during surgery. Monitoring helps detect the potential for intraoperative injury in real time, allowing physicians to make immediate corrections.
A team of experts from an experienced pediatric intraoperative monitoring group along with experts in adult intraoperative monitoring at Mass General are preparing to study new approaches in intraoperative monitoring in children. While these collaborations focus on ways to improve the capacity for pediatric intraoperative monitoring of neurologic functions, advances may also improve monitoring in adults. The team plans to work with engineers to adapt the technology in ways that will improve the safety and efficacy of monitoring in children, and to study its effect on outcomes from surgery.
Implications for the Future
Research into the surgical treatment and care of children with neurological problems is, in many ways, still in its infancy. However, experts at MGHfC are helping to gain a greater understanding of how the pediatric brain regains function as well as to develop better surgical and medical treatment options. By studying new treatment modalities with the most severe patients, this knowledge can be adapted to benefit patients with neurological problems of all severities.
Massachusetts General Hospital for Children
MassGeneral Hospital for Children (MGHfC) is a full-service, family-centered, pediatric “hospital within a hospital.” MGHfC provides the entire spectrum of pediatric care—from primary care to a broad range of specialty and subspecialty pediatric services. With more than 250 physicians representing more than 60 pediatric specialties and subspecialties, MGHfC has the expertise and experience—as well as the state-of-the-art facilities and technologies—to provide exceptional care for infants, children, and adolescents, including those who are critically ill and/or have rare disorders. MGHfC provides patient care services at its main campus in Boston, as well as at a number of community locations.
To refer a patient, please call the MGHfC Access and New Appointment Center, Monday through Friday, 8 am to 5 pm, at 888-MGHfC11 (888-644-3211), or visit the MassGeneral Hospital for Children website
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