There is an array of successful and promising treatments for epilepsy according to Bauer. "If we know a part of the brain is abnormal and that that is where the seizures are coming from, then we can do a craniotomy and cut out part of the brain. If we aren't sure of the location of the seizures we can take the bone off the brain and put in electrodes to monitor for seizures, pinpointing exactly where they are coming from. Then we would do a second surgery to cut out that part of the brain and remove the electrodes. That can be very successful."
Other procedures include hemispherectomy: "We cut the connections between one hemisphere and the rest of the brain. For kids who are severely disable, with one part of their brain very functional and the other not, this can be a lifesaving procedure." There is also the vagal nerve stimulator—where an electrode is wrapped around the vagus nerve in patients when the location of the epilepsy seizures cannot be pinpointed—and electrical impulses are sent to the nerve into the brain which can decrease the number of seizures a patient is having. "These procedures can often make them seizure free or lessen the occurrences," says Bauer. "That's a huge benefit because then they can potentially drive and work and learn in school and do things that are difficult when you are having multiple seizures each day."
Bauer also cares for children suffering from spasticity, such as cerebral palsy. Procedures—like putting in a baclofen pump—have shown almost immediate results in helping to decrease the tone (or tightness) in children's arms and legs, so that they can walk and better function. "I recently had a young patient, around ten-years old. She had spasticity in her arms and legs and wasn't able to pour a glass of milk for herself. She could walk, but it was very difficult. She had taken oral baclofen, but even the lowest dose caused toxicity. We put in the pump and immediately she could make the smooth motion to pour a glass of milk without spilling. With therapy she continues to improve. Now she gets around with more control, or for longer distances, on her Segway©.
The baclofen pump is a device that is implanted in the abdomen and goes into the spinal column. It infuses a highly concentrated solution—baclofen—directly into the spinal column, which can greatly decrease spasticity. "When you take the pill orally," Bauer explains, "very little of it goes to the brain and most of it causes side effects, so a lot of people can't take a very high does because they get overcome with side effects. With the pump we can bathe the nerve roots and spinal cord in the drug and then the patients can get great improvement in spasticity. It's a very good procedure for some patients.
"This young patient benefitted so much I wish we could have gotten to her earlier and identified the issue," Bauer says. "I was a little upset at that, because the earlier spasticity is treated the better outcomes are long-term. They can start physical therapy sooner to overcome those barriers."
Children with spina bifida, or myelomeningocele, are born with their spinal cord open to the air. Other children with more subtle spina bifida occulta only have subtle or no markings on their back, that it may not be recognized until they start having symptoms. Those symptoms can include: back pain, orthopaedic deformities, weakness in legs or feet, or incontinence. "Sometimes," according to Bauer, "imaging will show a tethered spinal cord requiring surgical detethering." Spinal tethering is when the spinal cord becomes attached to the spinal column, which in some cases, but not all, may limit mobility and blood flow. Detethering is the process of removing the lesions or connections that have formed.
At Dartmouth-Hitchcock, Bauer has been pioneering the technique of laser-assisted detethering of spinal cords. "In children with spinal dysraphism—spinal developmental abnormalities—we use a handheld laser to more quickly and safely perform the procedure," explains Bauer. "Detethering has been done at D-H for quite a while, but not with the laser. The laser makes it safer and more precise. It can both cut and melt away fat, so I'm able to melt away fat and do neuro-monitoring during the procedure, while stimulating to ensure I don't cut good nerve tissue. That's more difficult with other techniques. Laser-assisted detethering also adds speed to the operation, so less anesthesia needs to be used and recovery time is improved."
In the area of hydrocephalus—a buildup of fluid inside the skull that leads to brain swelling—a condition that often presents with spina bifida, Bauer says, Dartmouth-Hitchcock is also doing endoscopic third ventriculostomy/choroid plexus cauterization. "It's a fairly new procedure used to treat increased pressure in the head. We were able to coagulate the part of the brain that produces spinal fluid to decrease production—which increases the efficacy of the procedure. For the right patients, this means that that child wouldn't need a permanently implanted shunt," which has been the standard method of treatment and often need to be replaced within a few years.