Treating the Tricky Spinal Vascular Malformation

Pediatrician
November 13, 2013

Meredith Carter

Following her treatment at
Johns Hopkins Children’s Center for a
large spinal vascular malformation, “Madalyn is moving perfectly,” says her mom, Meredith Carter. 

Born five and a half weeks early, Madalyn Carter was admitted to the NICU at a Washington, D.C. hospital, where neonatologists quickly noticed she wasn’t moving her arms. An MRI revealed the cause—a spinal vascular malformation (SVM) compressing the newborn’s spinal cord and cutting off circulation to her brain. Madalyn was at risk of suffering a severe stroke, doctors told her mother. Worse, they had no treatment to offer.

“We were devastated,” says Meredith Carter. “It was like a ticking time bomb from which she might end up paralyzed.”

Seemingly with nowhere to go, the Fulton, Md., mother remembered that Johns Hopkins pediatric neurosurgeon Ben Carson was a member of her church. She contacted Carson, and he referred Madalyn to pediatric neurosurgeon Rafael Tamargo and interventional neuro-radiologist Philippe Gailloud, who have deep experience in treating these rare but lethal lesions.

“Experience is important,” says Gailloud. “With it, you have seen these lesions before and you know what you have to deal with.”

“Most doctors, even neurologists and neurosurgeons,” says Tamargo, “will only see a handful of spinal vascular malformations in their entire careers.”

ADVERTISEMENT

Also critical to a good outcome, say Tamargo and Gailloud, is an early and accurate diagnosis. Undetected and untreated, these lesions can grow, bleed and even kill, says Gailloud, noting that dramatically increased pressure in the venous system can cause hypertensive cardiomyopathy.

“When the spinal cord circulation fights against such high venous pressure, everything starts to dilate, and the child gets weak and loses bowel and bladder function,” explains Gailloud. “These very big vascular malformations compress the spinal cord like a tumor, resulting in a bleed or stroke.”

Tamargo and Gailloud strive to see patients the day after every patient inquiry, but getting the right diagnosis can also be delayed by the challenge of imaging these conditions. Some spinal fistulas often aren’t visible on MRI, and the ones that show up on CT scan require a shrewd radiologist to spot them. Definitive diagnosis requires a spinal angiogram—a specialized imaging technique often eschewed by doctors. When this technique was invented in the late 1960s, Gailloud explains, it was blamed for spinal infarctions and other serious complications. Spinal angiograms have become much safer with decades of experience, and Gailloud and his colleagues perform two or three of these procedures each week. In a recent paper they described spinal angiogram outcomes on 350 patients.

“There were no significant complications,” Gailloud says. “It turns out to be a very safe procedure.”

Once they diagnose an SVM, Tamargo and Gailloud formulate an individualized treatment plan. Most patients can be treated endovascularly by embolizing their problematic blood vessels with coils or glue. For those whose anatomy or conditions don’t allow such treatment, surgery is the next best option. Madalyn’s case was even trickier. Her SVM—a large perimedullary venous fistula—was on the interior side of her spinal cord rather than on the back of the neck, making access through the femoral artery the only option. In such cases, says Gailloud, it’s important to stage the procedures.

“If you try to treat the whole thing in one session, the lesion may get so angry that it begins to swell and damage the spinal cord,” says Gailloud.

Madalyn underwent her first embolization at 9 months of age, her second at 15 months, both times with no complications. “Within a day of her first procedure she started to move her arm—it was incredible,” says Carter. “Now at 4 and a half years old, Madalyn is moving perfectly. You would never know anything was wrong.” 

For more information, call 410-614-1533.