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Home of Sidney Kimmel Medical College

Abnormal Blood Vessels
in the Brain, Once Unreachable,
Can Now Be Treated

An expertly performed neurosurgery ends brain bleeds and reverses neurologic deficits for a 25-year-old Bucks County patient.

On June 30, Christian Czmar of Bucks County turned 26 years old. It was an especially happy birthday, free from the threat of future brain damage due to spontaneous intracerebral hemorrhage (ICH), thanks to the highly successful brain surgery he underwent at Jefferson Hospital for Neuroscience (JHN).

Christian’s celebration was a far cry from his anything-but-happy 23rd birthday in 2018. He awoke that morning with a severe headache – his initial symptom of what turned out to be spontaneous ICH.

Spontaneous ICH – also known as a brain bleed – occurs when blood vessels in the brain rupture on their own, rather than because of an injury to the head, resulting in a stroke.

Headshot of Dr. Christopher Farrell

Dr. Christopher Farrell

“Stroke is a general, widespread term for any sort of vascular injury to the brain,” says Jefferson neurosurgeon Dr. Christopher Farrell. “The most common type is ischemic stroke, where a lack of blood flow to a portion of the brain causes the cells in that area to die.

“ICH is the second most-common type of stroke. High blood pressure is what usually causes blood vessels to rupture spontaneously, particularly in older adults. But in patients under 50, the source of rupture is often a structural lesion, such as a brain tumor, arteriovenous malformation or, in Christian’s case, a cavernous malformation.”

Troubling Days

Christian’s father, Don, clearly remembers his son having a hard time focusing on anything that was happening or said to him on that awful birthday three years ago. Even after taking some aspirin and a nap, Christian’s headache persisted and he was unable to hold down the little he ate.

“I was getting concerned that Christian might’ve had some sort of concussion,” says Don, a nurse manager in the ICU at Jefferson Bucks Hospital. “We really didn’t know what was going on. But we decided to sit tight overnight.”

The next day was even more troubling.

When Christian woke up, he could speak only gibberish. His pupils were somewhat dilated, and he seemed to be looking right through me. Those were major red flags for me. So, my wife, Jill, and I took him to the ER at Jefferson Bucks, where he was given a CT scan. – Don Czmar

The scan revealed that Christian’s cerebral aqueduct, an area of the brain that drains cerebral spinal fluid through the body, was obstructed. This caused the ventricles, or hollow spaces where the fluid originates in his brain, to become noticeably enlarged – a condition called hydrocephalus; this extra fluid puts great pressure on the brain. As soon as the radiologist noted the enlarged ventricles, says Don, Christian was immediately transferred to JHN and placed under the care of Dr. Farrell, who specializes in skull base surgery.

Cavernous Malformation

“A cavernous malformation is a collection of abnormal blood vessels that patients are often born with, although they can sometimes be acquired later in life,” explains Dr. Farrell. “What makes these blood vessels abnormal is that they don’t have the usual walls of muscle and tissue that keep them intact. Their thin walls make them prone to rupturing over time,  and they tend to bleed. More than 50% of patients with cavernous malformations develop hydrocephalus. After their initial bleed, cavernous malformations are prone to continued bleeding, causing damage to the surrounding brain and neurologic deficits like diminished vision and seizures.”

Unfortunately, it can be very difficult to remove cavernous malformations because they often originate in deep parts of the brain that are difficult to access surgically. Doing so has traditionally required a craniotomy – a procedure in which the neurosurgeon opens up a large portion of the skull and goes through the patient’s brain to access and remove the cause of the bleed. In attempting this, neurosurgeons have often done more harm to the brain than good.

Christian’s cavernous malformation was located at the very back part of the midbrain, which is the top of the brainstem. This area controls the up-and-down movement of the eyes. The aqueduct, a very narrow channel where cerebral spinal fluid drains, also runs through that area.  Because Christian’s brain bleed was small, it did not affect his vertical gaze center, but it did block the aqueduct so that the cerebral spinal fluid couldn’t flow.

Draining the Fluid Endoscopically

Rather than a craniotomy, Dr. Farrell used a minimally invasive procedure called an endoscopic third ventriculostomy. He made a small incision in Christian’s skull and inserted the endoscope, a thin, tube-like instrument with a small camera. This provided Dr. Farrell with a detailed image of the third ventricle of Christian’s brain. With a tiny instrument inserted in the camera’s side port, he made a tiny hole in this ventricle, through which the excess cerebral spinal fluid drained into another area of the brain and was absorbed.

The procedure was a success – with the fluid pressure relieved, so were all of Christian’s troubling symptoms, and he was able to return home just three days after arriving at Jefferson.  “Everything was fine,” says Don. “Christian needed to rest and build up his activity tolerance, but he no longer had any impairment or visual issues.”

“I had to get MRIs every six months so that Dr. Farrell could keep tabs on my cavernous malformation,” adds Christian. “Apparently, I had some small bleeds, but none that I noticed or that Dr. Farrell felt required treatment.”

Cross section of a brain stock illustration

A “Whoa!” Moment

But on January 30 of this year, Christian awoke with more nausea, extremely blurred vision and an inability to gaze upward with his eyes – a condition known as Paranaud’s syndrome. Don and Jill contacted Dr. Farrell’s office and were told to bring Christian in immediately.

“They did an MRI, which showed that Christian’s cavernous malformation was bleeding significantly,” recalls Don. “The double vision was what concerned us most, but during his examination, when we first noticed his inability to look up – that, for me, was a ‘Whoa!’ moment.”

Weighing the Pros and Cons

Christian was kept in the hospital for three days and put on a steroid regimen. Although the bleeding stopped, his double vision persisted, and he was given an eyepatch for temporary help. Dr. Farrell advised Christian and his parents that, given that bleeding had occurred multiple times, more bleeding would likely follow and could cause serious damage.

“The brainstem has traditionally been considered surgically inaccessible – it’s very difficult to reach,” explains Dr. Farrell. “But thanks to experience and clinical trials, our neurosurgeons have the ability to access the brainstem and other formerly ‘unreachable’ places without damaging surrounding areas. However, with each case, we still have to determine whether or not we can safely remove the cavernous malformation once we get into the brainstem – surgical damage could potentially be worse than the malformation. We have to weigh that risk.

“In Christian’s case, we thought that we could access and remove the malformation safely. I did not think that we could accomplish recovery of the damage that had already been done by Christian’s hemorrhage, such as restoring his vertical gaze. But I did think that with the surgical approach we planned, we could avoid any more injury for the rest of his life. Otherwise, the malformation could have continued to bleed and do more and more damage.

Dr. Farrell met with Christian and his parents to explain the pros and cons of having surgery to remove the malformation.

“As a highly experienced nurse manager, Christian’s dad is incredibly savvy about the medical issues of big, challenging surgery,” Dr. Farrell notes.  “Because his hemorrhage proved that it was going to continue to rupture and cause more and more potentially life-long problems for Christian, he and his family opted for surgery.”

Even Better Outcome Than Anticipated

On February 16, Dr. Farrell performed a difficult, eight-hour procedure that did not require him to go through the brain itself and risk further damage. Instead, he was able to go over Christian’s cerebellum and under his occipital lobes to reach the brainstem and enter it through a small opening to successfully remove the malformation.

The procedure was an even greater success than Dr. Farrell anticipated. The next day, when he awoke, Christian’s upward gaze was completely restored. Since then, he has regained about 90% of his vision.

“I still have some residual double vision, especially if I read for a long period of time,” says Christian, “but it’s minor. Overall, I’m feeling much better.”

“When he first got home,” adds Don, “Christian was on modified bedrest. He wasn’t allowed to bend over from the waist. He had a lifting restriction of five pounds for a good two and a half months. He still tires easily but he’s gradually regaining his activity tolerance. His main problem now is some post-surgical anxiety about future problems, even though Dr. Farrell completely removed the malformation, so he’s now seeing a neuropsychiatrist that Dr. Farrell referred us to.”

“Christian did extremely well,” says Dr. Farrell. “His case is one of several successes over the past few years that demonstrate the advanced ability of Jefferson neurosurgeons to go into areas of the brain once considered unreachable, thanks to our experience and breakthroughs we’ve made through our innovative clinical trials.”

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