A Novel Approach to Brain Surgery

December 2011
 
Five-year-old Ava Menefee was suffering frightening stroke-like episodes: One side of her face would temporarily droop, or she'd lose all sensation in one hand. Although she had undergone surgery to fix the faulty blood flow to her brain that caused the episodes, the operation had failed.
 
Ava’s parents Scott and Chuan Menefee knew the problem was dire. The Los Banos, Calif., family had been told another brain surgery was not possible. But Ava's episodes, called transient ischemic attacks, would likely escalate to full-blown strokes and lasting brain damage unless repaired.
 
In June, the family's search for a second opinion led them to Gary Steinberg, MD, PhD, a neurosurgeon at Stanford University and Lucile Packard Children's Hospital, who offered a bold plan to fix Ava's brain. "When all else fails, you try this," he said.
 
Steinberg and colleague Sanjeev Dutta, MD, proposed stretching a network of blood vessels in the abdomen up to Ava's brain. They would leave the vessel network attached to its blood supply in the torso and tunnel the blood vessels under the skin along one side of Ava's neck to the surface of her brain.
 

Ava's surgeons, Gary Steinberg, MD, PhD and Sanjeev Dutta, MD

Hearing the plan, Scott and Chuan were relieved — and overwhelmed. The prospect of another brain surgery for Ava "was awful," Chuan said. And yet the family desperately wanted Ava to be able to return to her favorite activities – from playing softball to riding her horse – unimpeded by transient ischemic attacks or the threat of future strokes. "The only thing worse would have been to hear them say they couldn't do anything at all," Chuan said.
 
Steinberg is one of the world's foremost experts in surgical repair of moyamoya, the name of Ava's brain vascularization problem. Ava was born without some of the large blood vessels that normally supply the brain.  The Japanese word moyamoya translates as "puff of smoke," a description of the tiny, fragile clusters of blood vessels that the brain grows to try to compensate for the missing vessels.
 
In her first surgery at another hospital, Ava’s scalp arteries and a scalp muscle called the temporalis muscle had been grafted onto the surface of her brain in the hope that they would take root and replace the missing circulation, a method that works most of the time. But because it failed for Ava, there were no longer any scalp arteries or muscle to use in another attempt at repair.
 
“I've never quite seen this problem before,” Chuan recalled Steinberg telling them at their first visit. “But we can fix it."
 

The arrows that point to the white areas in Ava’s brain show some of the small strokes she suffered before surgery.

Ava's transient ischemic attacks had become so frequent that she couldn't attend school, and an MRI scan showed that she was beginning to experience small strokes as well. Sixty-five percent of symptomatic patients who do not receive surgical repair and have impaired blood flow to the brain suffer major strokes within five years of diagnosis. That high probability of stroke was the reason a second brain surgery was worth the risk.
 
To use blood vessels from Ava's abdomen, Steinberg brought Dutta onto the medical team. A specialist in minimally-invasive pediatric surgery, Dutta could use laparoscopic instruments to dissect Ava's omentum, a network of abdominal blood vessels found in a large flap of soft tissue near the stomach and liver, away from the organs it normally supplies.
 
"The approach we planned is very unusual," Dutta said. The omentum has been used for brain revascularization before, but, to the surgeons' knowledge, had never been stretched to supply both sides of the brain. In its normal role in the abdomen, the omentum quickly creates new blood vessels at sites of trauma or infection. "That ability is what we're exploiting in this situation," Dutta said.
 
In the operating room, Dutta made four tiny incisions, each less than an inch long, in Ava's abdomen. With assistance from pediatric surgeon Matias Bruzoni, MD, Dutta separated the omentum from other abdominal organs and then cut its parallel channels in a special pattern to elongate it. He tunneled the vessels up under Ava's skin, making one more small incision in her neck to help turn a corner in the tunnel. Steinberg performed a bilateral craniotomy and carefully laid the blood vessels on both hemispheres of her brain. The vessels are expected to grow new channels to supply blood into Ava's brain.
 
The unconventional approach worked. Now, almost three months after Ava's Sept. 15 surgery, her transient ischemic attacks have become much less frequent and severe, a good sign that new blood vessels are beginning to form in her brain. 
 
"Ava's prognosis is excellent for living a normal life," Steinberg said.
 
The uncommon procedure, and the teamwork it required between different surgical specialties, provide "a perfect example of the type of collaboration we can accomplish at Packard Children's," Dutta said. "This was a pretty unusual, heroic effort to help this child."
 
Ava showed her own heroic side in her recovery, quickly returning to kindergarten and reading, an especially important love for a young girl who wants to be a teacher when she grows up.
 
"She's the toughest, bravest person I know," Chuan said of her daughter, who’s looking forward to the holiday season. "Ava and the whole family are so thankful because this year, we know how close we came to having a very different life. We're just fortunate to have a facility like Packard Children's so close to us, with the kind of people that are there. We know we're in the very best hands."