Winter 2009

Dramatic Case Highlights Multidisciplinary AVM Treatment

Ricardo De Jesus was born at Hawaii's Tripler Army Medical Center with life-threatening heart failure and pulmonary hypertension. Subsequent imaging revealed a large vascular lesion in the right side of his brain. Acting aggressively to save the boy's life, Tripler's neonatology team had the infant airlifted to UCSF Benioff Children's Hospital, where neuroradiologist A. James Barkovich, M.D., identified the lesion as an arteriovenous malformation (AVM).

"The AVM was filling an entire half of the cranial vault, and there was little development of that side of the brain," says neurologist Heather Fullerton, M.D., director of the UCSF Pediatric Stroke and Cerebrovascular Disease Center. Based on the imaging studies and discussions with Barkovich, neurosurgeon Nalin Gupta, M.D., PhD, and interventional neuroradiologist Christopher Dowd, M.D., Fullerton spoke with the family about the prognosis and treatment options.

"We expected Ricardo to at least be disabled, perhaps severely, and we counseled the family to that effect, but they felt very strongly they wanted to go forward with treatment," says Fullerton. Surgery was not an option; it rarely is in infants with such large lesions, given the potential for excessive blood loss. So the team turned to embolization to save the boy's heart and lungs. In the case of De Jesus, however, embolization posed an unusual challenge.

"Usually, an AVM is a single connection," says Dowd, a pioneer in the embolization process. "In Ricardo's case, it was a very diffuse scenario and the target was much more elusive. We needed to be aggressive enough to diminish heart failure and allow continued survival, but we didn't want to cut off blood supply to the normal parts of the brain." In the end, Dowd needed to complete three separate embolizations of proximal branches before the heart failure and pulmonary hypertension were successfully reversed.

When the team last saw De Jesus in May 2008, he was 14 months old and progressing well. "The heart is not an issue," says Dowd. "There is some AVM there that won't impair neurological function, but it might require further treatment to reduce risk."

"He's off all cardiac medications," says Fullerton. "And he is doing remarkably well developmentally. In May, he had three words, receptive language, intact vision, and was scooting around the floor and walking with assistance." Except for annual visits, De Jesus is now under the care of his pediatrician in Monterey, California, where the family received compassionate reassignment by the Army.

From Imaging Through Embolization, Radiation
and Surgery

The De Jesus case is highly unusual, but it does highlight the value of experience and a multidisciplinary approach to all AVM treatment.

"Usually, if a child comes in with acute neurological problems and an X-ray turns up something that doesn't look quite right, we do a CT or MR scan, with the help of a pediatric anesthesiologist," says Barkovich. "The key is recognizing a potential vascular lesion. With a hemorrhage it's easy, of course, but in other cases it can be more difficult. Kids look a lot different than adults. Most have AV fistulas, while adults have true AVMs with a large nidus."

Once the group — which treats 25 to 30 pediatric AVMs each year — believes it is looking at an AVM, Dowd and his team perform a cerebral angiogram to view the various drains and feeders. "Generally, angiograms go through the femoral artery, but in infants we sometimes go through the umbilical artery because there is less long-term risk," says Dowd.

"We usually grade AVMs from 1 to 5," says Gupta. "If they are higher grade, we tend to be fairly aggressive with treatment because the cumulative risk of hemorrhaging over a child's lifetime is pretty high. And if it's already ruptured, that increases the risks of death and disability."

Often the treatment begins with an embolization during the angiogram. In that process, Dowd inserts medical-grade glue (cyanoacrylate) or platinum microcoils or material such as sponge particles through microcatheters. "The choice depends on the architecture of the connection between arteries and veins," says Dowd.

Except in the case of infants and very young children, treatment rarely ends at embolization. With children over the age of 5, the team usually employs a multimodal approach that might include embolization, surgery and radiation using a Gamma Knife. "If we can do surgery, that's the best option because if removal is complete, it is curative," says Gupta. He notes, however, that if the AVM is particularly large, he may not resect it because of the associated risks.

Many of these complex procedures take more than eight hours and occur in multiple locations such as the interventional radiology suite, the operating room and radiation oncology suite. This is all made possible by an expert team of pediatric anesthesiologists who are skilled in the care of the highest-risk children inside and outside of the operating room.

"What's right for these children is a little like a 3-D chess match," says Dowd. "There is no cookie-cutter approach because there are so many different parameters. That's why experience, expertise and access to technology are very significant for these cases."

For more information, contact Heather Fullerton, M.D., at (415) 353-3681.

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