Loyola surgeon successfully performs operation on patient suffering from rare disorder

Loyola University Chicago Stritch School of MedicineMar 4 2014

Jarely Sanchez is an affectionate, energetic little girl who loves ballet.

"Everyone she meets, she connects with," said her father, Jose Angel Ulloa. "She's like a magnet."

But for more than a week, the three-year-old girl wasn't herself. She had been suffering severe headaches and nausea that caused her to throw up dozens of times. And then one morning she suddenly screamed out in pain and fainted. Her parents worried that something was terribly wrong.

Jarely was taken to the emergency room, where doctors ordered a CT scan. And when the scan showed there was bleeding on the surface of her brain, Jarely was transferred to Loyola University Medical Center.

The bleeding on Jarely's brain was caused by a complex and potentially devastating condition that is almost never seen in children. There are only a handful of descriptions in the medical literature.

"You could go through an entire career and not see this a single time," said Jarely's neurosurgeon, William W. Ashley Jr., MD, PhD.

Bleeding on the surface of the brain is called a subarachnoid hemorrhage. But strangely enough, there was nothing wrong with Jarely's brain. Ashley performed an angiogram procedure that uses dye to obtain real-time pictures within the blood vessels of her brain and spinal cord. After ruling out more common causes, Loyola physicians determined that the bleeding was caused by an extremely rare abnormality involving the blood vessels of her spinal cord in the middle of her back. This malformation is called a spinal arterial venous fistula (spinal AVF).

Normally, arteries and veins are separate, with arteries conveying high-pressure blood coming from the heart to the body's organs, and veins conveying low-pressure blood back to the heart. But in Jarely's case, a major artery in her spine was directly connected to a vein. Consequently, high-pressure blood coming from the heart was shooting through a hole (fistula) into the vein like a fire hose.

The veins, not made to withstand such pressure, expanded like a balloon, forming what is called a venous aneurysm. The increased back-pressure in the veins draining the spinal cord may have caused her bleeding. The aneurysm also put direct pressure on the spinal cord which, if not treated, could have led to paralysis in the legs.

Ashley is among a new generation of neurosurgeons who are trained to perform traditional open surgery, as well as less-invasive endovascular techniques that use catheters rather than scalpels. Jarely's parents opted for the less-invasive technique.

Ashley inserted a catheter (thin tube) in the femoral artery in Jarely's groin. He guided the catheter through her blood vessels up to the arteries in her spine.

Ashley guided the catheter to the precise location in the spinal AVF where the artery connected to the vein. He then injected a liquid polymer that hardened almost instantly to block blood flow from the artery to the vein.

The procedure was challenging in Jarely, because a child's blood vessels are smaller and thinner than those of an adult. "We have many more constraints with a child. But the procedure was a complete success," Ashley said, "This was a team effort at Loyola that involved a lot of caring doctors and staff."

Jarely's dad said that as soon as Jarely got out of surgery, "She looked 100 times better. We are very thankful. We consider Dr. Ashley and our daughter to be a miracle."

Ashley said it's unknown what caused the spinal AVF in Jarely. She may have been born with the condition, which then progressed over time.

Jarely will celebrate her fourth birthday on March 18, and is feeling great. "There is a good chance that the lesion is gone for good," Ashley said. "I expect that Jarely will be back dancing again soon."

Ashley is an assistant professor in the Departments of Neurological Surgery and Radiology of Loyola University Chicago Stritch School of Medicine. With his dual training in open and endovascular surgical techniques, Ashley can offer patients the safest and most effective treatment options for complex neurovascular diseases.