Chiari malformation (CM) is a malformation of the back part of the brain where the space for the brain stem and cerebellum is insufficient causing downward displacement of these tissues through the opening at the base of the skull called the foramen magnum. Treatment is reserved only for patients who develop problems directly related to the Chiari malformation. There are countless problems that can arise from a CM, but the most common are neck pain, visual disturbances, change in pitch/volume of the voice or cry, difficulty with swallowing (especially liquids), worsening of arm/leg strength, sleep apnea or syringomyelia (fluid build-up within the spinal cord).
Epilepsy is defined as having two or more unprovoked seizures. Seizures are sudden changes in the electrical activity of the brain. They cause changes in behavior, sensations, awareness and motor function. Epilepsy is now divided into four basic types: Generalized, Focal, Generalized and Focal, unknown if generalized or focal.
1% of the US population suffers from epilepsy: 3 million adults and 470,000 children.
It is the 2nd most common neurologic disorder affecting over 1 million patients in the US.
Anti-epileptic medications continue to be first line treatment for epilepsy. 2/3 of patients with epilepsy are well controlled with medications alone.
30% of patients are not well controlled with anti-epileptic medications. Despite the many new medications being introduced over the last 25 years this number has remained stable. Early consideration for surgical options is important when medications fail to provide seizure control.
Surgical treatment for epilepsy:
When focal epilepsy becomes refractory to medication management then surgical options should be considered early. Surgical options for medical refractory focal refractory epilepsy can provide a 50-80% chance of seizure freedom. Alternatively patient who have failed multiple medications have less than a 10% chance at good seizure control.
Localization is the key: For patients who have epilepsy that is not well controlled on medication localization is important to long term management. Multiple surgical options are available for treating epilepsy and the choice of procedure is often determined by the location of the seizures. Long term inpatient video EEG monitoring and Stereoelectroencephalography (sEEG) monitoring is available for targeting seizure origins. These services are available for both adults and children at Oishei Children’s Hospital of Buffalo.
RNS- Brain-responsive neurostimulation: This new technology records the electrical activity of the brain and can recognize the early brain activity seen in seizures. When a seizure is detected the RNS will stimulate the brain and suppress the seizure activity. Patients report a 75% reduction in seizure activity at 9 years (RNS website)
Anterior Temporal Lobectomy– Surgically the skull is opened and a portion of the Temporal lobe is removed. Although this is an invasive operation an anterior temporal lobectomy can provide a good chance to cure epilepsy.
Laser ablation– This minimally invasive surgery has less recovery and less pain. During surgery a laser probe is inserted through a small hole in the skull. The probe is advanced and guided using MRI to its target. In real-time the laser is utilized to remove the seizure focus without damage to the surrounding tissue.
VNS – Vagal Nerve Stimulation provides electrical impulses to the brain by utilizing the vagus nerve. These mild impulses are routinely delivered by an implantable device in the chest wall. Stimulation can additionally be triggered with the use of a magnet that the patient wears on the wrist. Vagal Nerve Stimulators decrease the overall number of seizures that patients experience and can prevent seizures from occurring.
DBS – Deep brain stimulation: Approved for the treatment of focal epilepsy that is not well controlled with medications when other surgical options are not appropriate. This therapy uses a surgically placed electrode in the brain to stop brain signals that cause seizures.
How do I become a patient: Contact Dr. Jonathan Riley at 716-218-1000 for epilepsy consultation.
Links to include: John R. Oishei Children’s Hospital https://www.ochbuffalo.org/care-treatment/epilepsy
Epilepsy Foundation https://www.epilepsy.com/
Hydrocephalus is defined by excessive cerebrospinal fluid (CSF), as a consequence of imbalance between the production and absorption of CSF. Most commonly it is absorption of CSF that is the problem. When this imbalance occurs, the ventricles become enlarged as the fluid builds up. An analogous situation is like a sink with a faucet that is always on at a constant rate. As long as the drain at the bottom of the sink is open, there is no build-up of fluid. If the drain starts to get plugged then fluid will start to well up within the sink.
The way in which children with hydrocephalus are affected is dependent upon their age at the time of diagnosis. In children less than two years, the most common way it is detected is an abnormally rapid head growth. Rather than following a normal growth curve on a head circumference chart, these children start to cross curves and often ultimately exceed the curve for the 95th percentile. This occurs since the young child’s skull bones are not yet fused. Thus as the fluid builds up, it separates the bones and causes the skull to expand too quickly. Less frequently, children in this age group develop vomiting, excessive sleepiness, unexplained irritability, loss of development milestones or even developmental delay, downward deviation of their eyes and seizures. When examined, the soft spot tends to be full and the gaps between the skull bones are often split wider than normal. Children older than five years have skull bones that are less likely to expand as fluid builds up thus they tend to show signs of raised pressures within the brain itself. This typically causes them to complain of headaches, nausea with or without vomiting, listlessness or lethargy, unexplained irritability, loss of developmental milestones, change in behavior, change in school performance and rarely seizures. Medical examination of these children can range from normal to showing pressure induced changes in the eye nerves (papilledema), problems with eye movements, gait disorders and/or problems with cognition. Children between two to five may have a mixture of clinical findings that fall somewhere between the older and young age groups.
Several studies can be helpful in establishing the diagnosis of hydrocephalus, including ultrasound, computed tomography (CT) scan and magnetic resonance imaging (MRI). Ultrasounds are only useful in the very young children that still have open soft spots. This study is very good at looking at the size of the ventricles but doesn’t provide as much detail as a CT or MRI does about the structure of the brain tissue itself. A CT is sort of the middle-tiered study that gives both good views of the ventricles and brain tissue and can potentially identify sites of blockage within the brain. Sometimes the addition of intravenous contrast (dye) can add further information. The advantage of CT is that the study itself is quick so that sedation is not needed and thus can be scheduled in relatively short order. The most elaborate way of imaging the brain is MRI and this is certainly the best study to look at specific sites of blockage within the brain. A MRI, however, does require sedation or anesthesia if the child cannot hold still for at least 30 to 45 minutes. As a consequence it cannot be as easily scheduled as a CT scan. Thus, patients may have at least two of these studies done during the course of their evaluation.
Unfortunately, there is not a reliable, long-term non-surgical treatment for hydrocephalus. There are some medications available that might reduce production of CSF but they often cause significant side effects and are not effective over the long-term. There are two forms of surgical treatments that can be employed; the first is a ventricular shunt and the second is an endoscopic third ventriculostomy. The former can be used on all patients with hydrocephalus, whereas the latter is reserved for only those with non-communicating hydrocephalus.
A shunt is a device made of silastic that is placed under the skin and runs from the head to the belly (peritoneum), chest (pleura) or heart (atrial). Every shunt has three parts; the catheter that goes into the brain, the valve, and the tubing that goes to one of the three above mentioned distal sites. The shunt serves as a separate pathway to drain CSF in the brain that will ultimately be absorbed by the lining of the belly cavity, chest cavity or directly into the heart. The valve serves as a regulator of CSF flow through the shunt system. Surgery for placement of a shunt requires an incision in the head and over the site for the distal catheter (belly, chest or around the neck or collarbone). The surgery takes about one hour and children are usually discharged the next day. It is important to note these shunts are self-sufficient and do not require that you do anything to keep them working. You will see parts or all of the shunt as it bulges underneath the skin, particularly evident in the neck. This should not be of concern.
An alternative treatment for non-communicating hydrocephalus is a surgery known as endoscopic third ventriculostomy (ETV). In these cases the blockage of CSF flow occurs beyond the third ventricle and presumes that the ability to absorb CSF back into the bloodstream is normal. Thus, if a communication between the ventricle upstream from the site of the blockage and the subarachnoid space can be made, the hydrocephalus could be effectively treated. This is done by placing an endoscope into the lateral ventricle, and then under direct vision the tip of the device is guided into the third ventricle. The fluid within the ventricle can escape through the hole into the subarachnoid space and ultimately get absorbed back into the bloodstream through the normal pathways. In properly selected patients, about 80% can be effectively “cured” of the hydrocephalus by an ETV. The remaining 20% will need to be treated with a shunt.
MD FACS FAANS
Minimally Invasive Brain Endoscopy
Spine & Skull Base Disorders
Minimally Invasive Brain Endoscopy
Publications | August 14, 2020View all Publications
In the News | January 23, 2017UBNS in the News
The KL2 Research Career Development Program supports state-of-the-art clinical and translational research and provides young investigators with the knowledge and skills necessary to perform high-impact research and succeed in today’s highly competitive research environment.Continue to news article