Our patients’ stories: Yousef’s Vein of Galen surgery

Yousef-1Yousef Alrkhayes was just two days old when a doctor burst into his mother’s, Khadjad’s, hospital room with unsettling news. “[He] came into my room and said that Yousef has high pressure in his heart and they didn’t know why,” she recalls. After you are discharged, the doctor continued, don’t even go home—go straight to the main hospital.

In the four days it took Khadjah to recover enough to move with her son, Yousef made little progress. His heart was still under stress and no one could say why. As their doctor sent them on their way, he begged them to ask for an echocardiogram at the hospital.

Khadjah could tell from the sound of his voice that he was worried.

Trains for oxygen

Metro ShanghaiShanghai, China is home to the world’s largest network of metro stations in the world. The trains made 2.5 billion trips last year alone, covering 334 total miles of track and serving more than 8 million people every day.

It’s impressive, but nothing compared to the volume, distance and speed of blood moving through the human body. The average adult body has 6 quarts of blood, which circulates through the entire body three times every minute. Laid end to end, the total length of all the body’s arteries, capillaries and veins can reach up to 60,000 miles—enough to wrap around the earth twice.

Like metros, the vascular system is made up of different parts, each playing a specific role in transporting oxygen throughout the body. Arteries carry high-pressure, fast-moving oxygenated blood from the heart to the rest of the body. They feed into capillaries, which slow the flow of blood enough for surrounding tissue to absorb oxygen, decreasing the blood’s pressure at the same time. When it has no more oxygen to deliver, blood—by then low-pressure and slow moving—moves into the venous system, which carries it back to the lungs and heart to be re-oxygenated.

Like any metro system, the vascular system can fall victim to crippling congestion. Heavy traffic in areas only capable of supporting low traffic can cause delays and breakdowns.

That’s what happens when the body develops an Arteriovenous Malformation (AVM). The body never develops capillaries in specific areas, leaving high-pressure, fast-moving blood to flow directly into the veins. Without the capillaries, the blood doesn’t slow down enough to deposit oxygen to the surrounding tissue, which then signals to the heart that it needs more oxygen. The heart starts pumping harder and harder and harder, creating a vicious cycle.

“The hearts of children with AVMs are beating very rapidly, all the time,” Darren Orbach, MD, PhD, interventional radiologist at Boston Children’s Hospital explains. “It’s not even just the speed, but also the contractility; the heart increases its oomph with each squeeze.”

This supply and demand problem was the very cause of Yousef’s heart strain, but at the time, back in Kuwait, no one knew the problem was rooted in his brain.

It’s all in Yousef’s head

Yousef 4Khadjah was devastated when the clinician administering her son’s echo admitted he couldn’t tell what was wrong with Yousef’s heart. But her devastation turn to curiosity when he abruptly took his imaging equipment off her son’s chest and placed it on his head. The echo of his skull didn’t reveal anything, but it peaked his curiosity enough to schedule an ultrasound and a CT scan of Yousef’s head.

Five days later, the results of the CT scan confirmed the clinician’s theory: Yousef has an incredibly rare congenital AVM known as a Vein of Galen Malformation (VOGM). VOGMs affect a vein deep inside the brain, preventing capillaries from forming and straining the brain’s venous system.

“I was really surprised and I wanted to know everything about it,” Khadjah explains. “I started reading everything I could on the subject.”

VOGMs can be life threatening because they put strain on the developing brain, heart, kidneys and liver. And since it isn’t able to properly drain its internal venous system, the brain is at risk for permanent damage the longer the VOGM is left untreated.

A neurologist in their hometown of Kuwait City said he would be able to operate on Yousef at six months with a 40 percent chance of success—a prospect Yousef’s parents weren’t satisfied with. Because the malformation was so rare, they soon realized, they would need to find help outside of the country.

A French hospital told them that they would only feel comfortable operating after Yousef had reached his first birthday. A second hospital with the expertise they needed—this time in Britain—gave them a similar response. The third hospital, Boston Children’s, turned out to be the perfect fit.

Arm floaties for the brain

VOGMs, and AVMs in general, are a regular part of Orbach’s work. He treats as many as 15 cases like Yousef’s every year. That track record, and Yousef’s outstanding clinical exam, went far in reducing his family’s fears.

VeinofGalenI 1“There was never a clinical cardiac problem,” Orbach explains. “He wasn’t in heart failure even though they sensed he was in overload. Besides that, clinically, he looked fantastic.”

Not that long ago—as recently as the 1980s—VOGMs were often a death sentence. Treatment meant a neurosurgeon would have to try to access and then close off the connection between the arteries and veins in the brain. Severe bleeding and hemorrhaging were serious risks.

“The treatment was almost worse than the lesion back then,” Orbach explains. “They used to talk about morbidity and mortality of over 90 percent.”

But recent advances in materials, imaging technology and catheter-based techniques have greatly improved the odds. A year before he met Yousef, Orbach treated an AVM in a premature baby who weighed only four pounds, something he admits would not have been feasible even a few years ago.

Today, most surgeries to correct VOGMs involve inserting a catheter into a major artery—usually entering mid-thigh—and then snaking it around the heart and neck to reach the brain. Once there, a surgeon can inflate a small balloon attached to the outside of the catheter—think arm floaties for catheters—to close off the connection between the arteries and veins and then inject a glue to seal off the connection. Once the glue has set, it’s simply a matter of deflating the balloon and carefully removing the catheter.

20140611_VeinofGalenIR-33PreviewlargeEven with recent advances, guiding the catheter through a newborn’s twisty tangle of arteries and veins is no small feat. Yousef’s four-hour operation soon stretched into a fifth, sixth and even seventh hour.

“Obviously when the procedure took longer than 4 hours, it made us think,” Khadjah recalls. But seven hours after he was placed under anesthesia, Yousef emerged from the operating room with a major weight off his mind.

“During the procedure, we would go up to the nurses and staff and ask if everything was alright,” Khadjah recalls, “but even when it started taking so long, we weren’t worried. We trusted our doctor.”

 

 

To find out more about Vein of Galen Malformations and about Boston Children’s Hospital’s Cerebrovascular Surgery and Interventions Center, click here.