Patient Treated for Rare Skull Base Aneurysm

Rebecca Anderson enjoys reading, gardening and riding with her boyfriend on his Harley. A self-described small-town girl, she grew up in Spickard, Missouri, population 250, and now lives in Princeton, Missouri, just a bit larger at 1,100. The local hospitals provide the best possible care and service, but when unusual or complex needs emerge, it can take some time and extra help to diagnose and treat them.

For that reason, Rebecca is thankful for The University of Kansas Health System. Its main campus is about 2½ hours from her home, but fortunately, she was able to get there quickly enough when she needed to.

In early 2017, Rebecca, 40, experienced significant ear pain. She thought it was another event in her history of sinus problems. Her primary care provider agreed, but also noticed Rebecca's tonsils seemed swollen. Rebecca was prescribed steroids and antibiotics and took the whole course, but felt no better.

"Then, as I sat down to eat one day, I suddenly felt really weird," Rebecca said. "I knew something wasn’t right. I called my mom and said I had to go to the ER."

There, Rebecca had a laryngoscopy, which provided a close-up view of her larynx and throat. The procedure revealed the source of Rebecca’s discomfort. She had a carotid artery aneurysm – a bulge in the wall of one of the main blood vessels that carries blood to the brain, neck and face.

"The aneurysm was so big, it was pushing my tonsils upward," Rebecca said. "I soon became very hoarse and all but lost my voice."

Rebecca’s local ER doctor contacted The University of Kansas Health System. The doctor wanted to transfer Rebecca immediately, but there were no ambulances available to take her. Rebecca and her boyfriend, Jay Stevens, hopped in their car and began to drive.

"It was a very scary ride," Rebecca said. "I kept thinking this could explode at any minute. It was very stressful."

Upon arrival, Rebecca was promptly admitted. Evaluation confirmed that the giant aneurysm extended to the base of her skull. In addition, Rebecca informed her care team that many years earlier, she had had an aneurysm in her basilar artery, which supplies blood to the brain stem. She’d been sent out of state for treatment, and the aneurysm had been coiled, closed off to blood flow.

"The location of Rebecca’s current aneurysm combined with her history set up a very complex situation," said vascular surgeon Kirk Hance, MD. "We were unable to address the current aneurysm via the neck and needed to perform further evaluation to better understand how her prior treatment would influence our team’s decision-making for her best outcome."

The team performed an angiogram to evaluate the status of the four main arteries that serve as the brain’s critical suppliers of oxygenated blood. The test showed that Rebecca’s two vertebral arteries were no longer carrying blood to her brain, leaving the two internal carotid arteries to fulfill that crucial function. And of these two remaining arteries, one contained the aneurysm.

"We had the option to coil and occlude the skull base aneurysm, eliminating the potential for it to rupture, but that would leave Rebecca with a single carotid artery responsible for her brain’s total blood supply," said Dr. Hance. "We needed to understand the impact that strategy would have on her functional capabilities and consider the risk of stroke before we could proceed."

A specialized test performed by a neurointerventional radiologist provided this important information. And Rebecca played an essential role herself. She remained awake and responsive to instructions during the balloon test occlusion – in which a small, flexible balloon was guided into the artery and inflated to block blood flow, simulating the blood flow process as it would occur without the affected artery. This allowed the care team to evaluate her brain function without the artery she might lose during surgery.

"I was like, 'You’ve got to be kidding me! I have to do what?'" Rebecca said. "That was the scariest part of this whole situation. They asked me questions and they had me do things like raise my arms. It was so strange, but the whole staff was really wonderful. It’s amazing what they can do."

Alan Reeves, MD, director of interventional neuroradiology, performed the balloon test occlusion.

"With the complexity of the aneurysm and the unusual nature, it was thought that the team could have to sacrifice the entire carotid artery," he said. "The balloon test occlusion simulates in a controlled fashion what would happen if that occurred, providing insight on how the brain would respond. If it didn’t respond well, it could leave the patient at risk for a life-threatening or life-altering stroke."

Once the balloon was placed and inflated in the artery, the care team interacted with Rebecca to evaluate the effects of the simulation.

"We assessed speech, grip strength, foot motion and facial droop," Dr. Reeves said. "We monitored these functions for about 20 minutes. Any signs of deterioration would indicate the brain was not receiving enough blood, and we would deflate and remove the balloon immediately."

Rebecca’s brain and body responded well to the simulation, indicating to her care team that sacrificing the artery would be a feasible option. However, neurosurgeon Paul Camarata, MD, one of few surgeons in the region skilled at this procedure, hoped that would not be necessary.

"Rebecca’s arteries were undoubtedly weak, as evidenced by her previous problems," Dr. Camarata said. "Sacrificing the artery with the aneurysm could very likely induce a future aneurysm in the remaining vessel. We did not want to leave her with just a single functioning artery, so decided to place a long artery from the carotid in the neck to the brain, effectively bypassing the diseased segment."

Rebecca’s complex surgery required the combined specialty expertise of a multidisciplinary care team. Rebecca’s narrow trachea raised concerns about the ease and safety of intubation, so otolaryngologist Shannon Kraft, MD, assessed her before surgery. Because the aneurysm involved the main artery to the brain, Dr. Camarata’s skills were critical. Dr. Hance removed the right cervical carotid aneurysm. Cardiothoracic surgeon Emmanuel Daon, MD, removed Rebecca’s right radial artery from her arm to apply as a graft. Dr. Reeves, in addition to the presurgical ateriogram and balloon test occlusion, performed an angiogram midprocedure to assess progress and reaffirm the strategy.

"Aneurysms can occur in patients of any age, but the location of Rebecca’s at her young age was quite rare," Dr. Hance said. "Most are not as large or extensive. This was truly a case in which the availability of a multidisciplinary team of specialists made every difference in achieving a positive outcome."

"These complex cases require multiple perspectives and points of view to assess, plan and address," added Dr. Reeves. "Each of us on a care team knows our own niche very well, but we also collaborate to apply our combined experience to solve complex health challenges. That’s what truly sets us apart and leads to the best outcomes for our patients."

Rebecca was hospitalized for a few days following the surgery. She continues to visit the health system for ongoing follow-up care, including seeing Dr. Kraft who has supported improvement of the vocal cord paralysis caused when the aneurysm obstructed the nerves in Rebecca’s throat, preventing the vocal cords' natural movement.

"While it has not completely returned to its normal state, Rebecca’s voice quality is much improved," Dr. Kraft said. "She is fully functional, and we will continue to support her conservatively. There are surgical options she may consider, but at this time, she does not want to undergo additional procedures unless absolutely necessary. Our multidisciplinary team remains available to her as she continues to progress."

Rebecca is doing well today and continuing to recover her strength.

"I'm still a little weak, but getting better every day," she said. "I think it’s really incredible how all these doctors came together. They are smart, talented people, each one the best at what they do, and they work together for the good of the patient. I could not ask for better care."

Rebecca is back at her books and back in her garden. And she’s eager to get back on a Harley as soon as her doctors approve.

Patient testimonial: As with all treatments, individual patient results vary. It is important to discuss your treatment options with your healthcare provider.