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Sandi K. Lam, MD, FAANS
Director of Pediatric Cerebrovascular Neurosurgery Program
Co-Director of Craniofacial Surgery Program
Texas Children’s Hospital

“She is going to write about this in her college application,” her father states while she nonchalantly takes a selfie in our clinic room. Two years ago, my patient was undergoing surgery to remove an arteriovenous malformation (AVM) in the brain after a large bleed left her in a coma. Today, she is a self-assured high school athlete recruited by several universities. I marvel at the remarkable strength of this patient and her family, and at how far we have come in neurosurgery. 

Though they are relatively rare, pediatric vascular anomalies profoundly affect our patients’ lives. Children with neurovascular diseases pose particular challenges in diagnosis and treatment. Children are not “just little adults,” and vascular pathology in the pediatric population is distinctly different from adults, which requires a tailored approach to diagnosis and management. Just consider these challenges, to name only a few in dealing with this patient population:

  • The brain and body of a child or adolescent are still actively developing and are subject to adverse effects from both disease as well as treatment.
  • A child’s physiology is dynamic and requires specific considerations for medications, fluid management, limits in contrast (X-ray dye) loads and radiation exposure, and repeated anesthesia exposures.
  • Many commercially available medical devices and instruments have not been specifically developed for, tested in, or approved for use in pediatric patients.

Care teams must partner with families to determine treatment options, the timing of intervention, and goals of care. It is not possible to overstate the gravity, but also the privilege of caring for these children. The goal is to balance a happy childhood where “kids can be kids,” while simultaneously pursuing treatments with durable outcomes for the years ahead.

Despite their youth, children have remarkable resilience. Surgeries from which an adult may take weeks to recover may have a 10-year old requesting chicken nuggets the next morning. Kids are often the ones encouraging their parents, making jokes with the nurses, and pushing our team forward with their unwavering spirit. We owe it to them to match their staunch optimism in the face of disheartening circumstances. They deserve our best and brightest minds pursuing innovations in neuroimaging technology, endovascular therapies, radiosurgery techniques, medical targets and operative procedures.

Neurosurgery Blog has chronicled progress in vascular neurosurgery, from clinical advancements allowing treatment of increasing numbers of patients to accounts from the forefront of research and innovation, to inspirational personal experiences. Pediatric vascular diseases are relatively rare compared to adults, making them a challenge for individual researchers to study.  There have been remarkable recent advances thanks to collaboration between clinicians, basic science researchers, as well as patient advocates.

The story of success in the management of pediatric neurovascular syndromes is one of both re-engineering the care rendered these patients as well as technological advances in the clinic and the operating room. Multidisciplinary care teams focused on managing these diseases have produced improvements in diagnosis and management. These teams are particularly attuned to syndromes which are associated with congenital anomalies of other organ systems, such as the heart, kidneys, and skin, and how these abnormalities fit into the approach and treatment of children with neurovascular diseases. Continuing innovation in endovascular approaches has led to progress in the treatment of many of these diseases, such as vein of Galen malformations, a particularly challenging disease. The result is improved outcomes through risk stratification and appropriateness and timing of treatment for these patients.

Clinical progress has been accelerated thanks to the breakthroughs in understanding the biologic underpinning of these diseases. Basic science knowledge of pediatric neurovascular diseases has increased considerably in recent years:

  • Many specific genetic mutations have been identified, some known to cause inherited syndromes while others are responsible for sporadic malformations.
  • Potential for translational applications is exciting. For example, we know certain genetic syndromes are associated with pediatric arteriovenous malformations and that the recurrence rate is higher in these children than adults.
  • The diagnosis, treatment, and outcomes of moyamoya disease (an idiopathic neurovascular occlusive disease which causes strokes in children) continue to improve as laboratory work sheds light on the disease’s causes. Clinical trials identify the most effective neurosurgical interventions.
  • Cavernous malformations in children may occur at a lower incidence rate than adults, but also may have a more aggressive growth pattern and clinical behavior. The familial form of the disease initially spurred people to band together, with the development and growth of strong advocacy platforms with medical/research partnerships such as the Angioma Alliance.

Advocacy by both patients and physicians has also led to great progress. Access to centers with these multidisciplinary teams is essential for patients. On April 18, the Advancing Care for Exceptional (ACE) Kids Act was signed into law by the president as part of H.R. 1839, specifically addressing children with complex medical conditions requiring specialized care. These patients account for a disproportionate percentage of Medicaid spending on children. The ACE Kids Act addresses existing challenges, including the provision and coordination of care across multiple providers and services, and access to out-of-state care. The ACE Kids Act will also expand access to patient-centered, pediatric-focused coordinated care models tailored for medically complex children, ensuring these patients have access to the care they need

While innumerable physicians, nurses, researchers, and other staff have been instrumental in improving the care and management of patients with cerebrovascular diseases, we especially recognize the many pediatric neurovascular specialists who have moved this field forward through their dedicated endeavors, spirit of inquiry, and effective knowledge dissemination, including:  R. Michael Scott, MD, FAANS(L); Edward Robert Smith, MD, FAANS; Pierre Lasjaunias, MD; and Alejandro Berenstein, MD, among many others. Our understanding and treatment of pediatric neurovascular pathology has come so far, and we need to continue the push forward to find better treatments for these diseases.

Meanwhile, my patient has her work cut out for her, just trying to teach me how to take a selfie!

Editor’s Note: We encourage everyone to join the conversation online by using the hashtag #VascularNeurosurgery.

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