There are many rare problems that effect the blood vessels in the brain of newborns, infants and growing children. While very frightening and potentially lethal, many if not most of these conditions can be successfully treated. Unlike the majority of problems children can face that require a neurosurgeon, these problems can be quite complex and are often best treated at very select centers that can provide the varied support staff needed to deal with such complicated issues. These include in addition to pediatric neurosurgeons, endovascular neurosurgeons and radiologists, neurologists and pediatric intensivists.
I will try to outline several of the more “common” of these rare conditions in broad terms, as well as the role of the different specialists involved. In no specific order they include: Aneurysms, Arteriovenous malformations (AVM), and Moyamoya.
While these conditions are quite different, there are some similarities as related to how they present to the parent or pediatrician. Since these lesions involve the blood supply to the brain, when they become apparent can be very striking. This is to say that the symptoms do not come on gradually but rather abruptly. Certainly if a vascular lesion was to bleed or stop blood to an area of the brain the symptoms would be quite rapid. For smaller bleeds the symptoms may be simply acute onset of headaches with or without changes in sensorium or function. For larger bleeds, one might expect marked changes in sensorium and function including even coma or profound stupor. In addition to changes in sensorium or function, sometimes these problems can lead to seizures as their first sign of trouble. For other types of lesions, such as with the AVMs or Moyamoya you may see a slow change in a childs function over weeks to months.
Once a vascular event is suspected an emergency evaluation is required to provide the child with the best chance of successful outcome. Often the first step is a CT scan or MRI scan to look for blood or a stroke in the brain. If a vascular lesion is found it will usually lead to the child having a study to look at the blood vessels of the brain, known as an angiogram or arteriogram. The timing of the study is quite dependent on many factors including the probable diagnosis, the childs level of function and the availability of the studies and specialists. In most centers in the US it is not the neurologist or neurosurgeon who performs the study but rather a radiologist. In addition to having a radiologist and anesthesiologist who are comfortable in treating infants and children with complicated problems, it is quite helpful to have an endovascular neurosurgeon at the center to help in treating the child. The endovascular neurosurgeon is a highly skilled physician who not only can perform the angiogram studies, but when indicated may be able to treat the problem by injecting varying materials in the blood vessels to help stop the source of bleeding. This treatment called embolization, may be a permanent cure or it may make it easier for the surgeon to then treat the problem by decreasing the risks of bleeding or stroke during surgery.
In addition to surgery and embolization, some vascular lesions may be able to be treated wholly or in part with a type of focused x-rays such as the gamma knife or linear accelerator. These x-ray treatments essentially burn away the vascular lesions but unlike the surgical treatments, these treatments require upwards of two years to take maximum effect and may leave a child unprotected from further problems during the time period. While this may not seem desirable, some lesions can not be completely treated with surgery and or embolization and this may represent the best alternative under certain circumstances.
What becomes clear from this discussion, is that for most situations, vascular lesions in children require specialized centers with pediatric and vascular neurosurgeons who have the latest technologies and specialists required to offer a complete evaluation and treatment plan for these rare and often complicated lesions.
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