Penn State Health Neurosurgery Tackles Rare Diseases

By Cari Wade Gervin
Thursday, May 7, 2020
Specialty: 

Advanced cerebrovascular and endovascular surgical techniques and technology for complex cases bring patients in central Pennsylvania new hope.

“A puff of smoke.” That’s the translation from Japanese of the word Moyamoya, which is also the name of a rare cerebrovascular disease. The blocked arteries at the base of the brain give the appearance of a puff of smoke on an angiogram, but that charming description belies the danger of the progressive disease that can first present as a series of strokes or transient ischemic attacks. Successful treatment of Moyamoya disease ultimately requires cerebral revascularization with direct cerebral bypass — a surgery that Ephraim W. Church, MD, assistant professor of Neurosurgery, Radiology and Neurology and director of the Cerebral Revascularization Program at Penn State Health Milton S. Hershey Medical Center, specializes in.

“It’s a microsurgery that I find both challenging and rewarding,” Dr. Church says. “And it’s a very effective surgery — we can bring the stroke risk from about 10% per year down to approximately 5% for the remainder of the patient’s life, and these patients can go on to live normal, healthy lives.”

Dr. Church and his team at Penn State Health Neurosurgery have been using the latest microsurgery technology to conduct the bypass surgeries — around two times a month to treat Moyamoya — numbers that Dr. Church says are increasing. The surgery uses a microscope to magnify 1-millimeter vessels 20 times. Dr. Church then uses a suture that’s about a third of the diameter of a human hair to connect a healthy vessel directly to a vessel in the brain, bypassing the narrowed or occluded vessel.

“It’s a microscopic, reconstructive surgery to create a route for blood around the blockage,” Dr. Church says. “It requires good microsurgical technique, intense concentration and creative thinking.”

A Spectrum of Treatment

Penn State Health Neurosurgery is just one of a few programs in the country that performs the direct bypass surgery, which is also used to treat carotid and intracranial stenosis, occlusion from other etiologies like atherosclerotic disease and complex brain aneurysm reconstruction, in which an aneurysm can’t be treated with traditional techniques.

Dr. Church, who completed his residency at Penn State Health, returned to the hospital last fall, after a fellowship at Stanford University Medical Center, to create and lead the Cerebral Revascularization Program.

“It’s a wonderfully collegial department,” Dr. Church says. “And it offered the opportunity to build something new that we didn’t have before. This was something we didn’t really have in this region.”

And, true to Penn State Health’s commitment to providing a multidisciplinary care model, the Cerebral Revascularization Program isn’t the only advanced neurosurgery offered by the department.

“We have a leading-edge Endovascular Neurosurgery Program, which is treating cerebrovascular disease through the vessels using catheters,” Dr. Church says. “We also have neurosurgeons who are doing tremendous work in our Movement Disorders and Functional Neurosurgery Program, which includes the placement of electrodes in the brain to treat various conditions. And we have neurosurgeons who focus on and use the latest robotic techniques to treat spine conditions in the Penn State Health Spine Center.”

“One of the goals as we continue to work on these conditions is to develop a strong research and education program aimed at better understanding how to take care of these patients and improving our microsurgical and bypass approaches. We also look forward to training the next generation of cerebrovascular neurosurgeons.”
— Ephraim W. Church, MD, director of the Cerebral Revascularization Program at Penn State Health Milton S. Hershey Medical Center

Strength in Collaboration

The opportunity to collaborate with other specialists, both inside and outside of the department, allows Dr. Church and colleagues to create strategic and personalized treatment plans for each patient.

“Brain tumors can be quite vascular and require cerebral revascularization,” he says. “So if you’re dealing with a tumor that’s quite vascular, one of our Skull Base Surgery Center colleagues may ask one of the vascular neurosurgeons to embolize the tumor to reduce the blood flow to the tumor so that it can be removed safely. Or if the tumor involves an important vessel in the brain, we might look at bypassing that vessel, so the diseased vessel can be removed along with the tumor, but the patient won’t have a stroke.”

As the Cerebral Revascularization Program grows, Dr. Church hopes it will become a resource for other providers to obtain information and research on Moyamoya and other similar conditions — an endeavor that proved worthwhile when the Program received a call from a neurologist about a patient in her 20s, whom he thought was having strokes caused by a carotid dissection.

“When the neurologist described the imaging, I suspected she might have Moyamoya,” Dr. Church says. “We transferred her to our center, and she went on to have a successful bypass operation. She has had no further strokes. She’s doing wonderfully.”

Through increasing awareness for referring physicians, Dr. Church aims to ultimately improve patient outcomes.

“Many times, patients can go years before they’re diagnosed correctly with Moyamoya and then receive the treatment they need to prevent additional strokes,” Dr. Church says. “It is very common for young people with Moyamoya to present with stroke, but the Moyamoya diagnosis is not recognized. They might go for years and develop additional strokes and disabilities before they get to our center and receive the treatment they need to prevent additional strokes. So, part of the goal of our Cerebral Revascularization Program is to educate physicians and patients about Moyamoya and other similar conditions, so we can be proactive about getting patients the care they need.”


For more information about Penn State Health Neurosurgery, visit hmc.pennstatehealth.org/neurosurgery. To refer a patient, call 717-531-3828.