As she grabbed her car keys and sprinted out of her office, Kimberly Almarode struggled to control the terror that surged through her body.
Her son’s preschool teacher had just called to say that Almarode’s 4-year-old son, Bentley, had fallen asleep in a classroom playhouse and teachers were having trouble rousing him.
“I just panicked,” recalled Almarode, 26, who lives in Stuarts Draft, Va., a town about 40 miles west of Charlottesville. She sped to the school to pick up Bentley, who lay on a cot in the nurse’s office. He opened his eyes when he saw her and said, “Mommy, I want to go to the doctor right now.”
During the previous two weeks, Almarode had grown increasingly worried about her previously healthy middle child. Bentley complained of frequent headaches that had worsened from bothersome to debilitating. “He went from a playful little boy to a zombie who couldn’t move off the floor,” she recalled.
But Almarode’s insistence that something serious was wrong with Bentley was dismissed by doctors who attributed his headaches, lethargy and episodic vomiting to a virus.
Three days after that March 2 call from the preschool, an optometrist had a distinctly different reaction. While examining Bentley for a possible vision problem, the eye doctor was so alarmed that he called the University of Virginia Medical Center to schedule an emergency brain scan and told Bentley’s father to take his son to Charlottesville immediately. What he saw, the optometrist told Curt Almarode, “was not normal in a child.”
“We expected to find a horrendous brain tumor,” recalled U-Va. neurosurgeon Kenneth Liu, who met the family soon after Bentley was admitted. The optometrist had detected a condition called papilledema, a swelling of the optic nerve caused by an accumulation of fluid that presses on the brain. Along with lethargy and vomiting, papilledema in a child is often a sign of pediatric brain cancer. Instead, Liu and his colleagues were surprised to discover a disorder unusual in children.
To treat it they used a new and potentially risky procedure, then waited anxiously to see if it would work. During a timeout in the operating room — a brief period during which the surgical team huddles to confirm important details before beginning an operation — Liu recalls feeling “scared [to death].”
“I remember very clearly saying, ‘This is not only a 4-year-old, [but] we’re doing things that have never been done, so let’s try not to screw it up,” the neurosurgeon said. “It was a fairly tense room.”
Liu’s gamble paid off. Six weeks after his surgery, Bentley went back to school, and he was soon playing T-ball, wearing a helmet to protect his head.
At first Almarode thought Bentley’s headaches might be an excuse to get out of doing something he didn’t want to do, such as putting his toys away, or a sign of routine illness. “We don’t overreact,” said Almarode, who with her husband also has a daughter, now 8, and a 3-year-old son. “If our kids get a boo-boo, we tell them to suck it up.” She gave Bentley an over-the-counter pain reliever and assumed he would get better.
But over the next week, he didn’t. His teacher called to say he was falling asleep in class. One night while the family was out to dinner, Bentley told his mother his head hurt terribly, then vomited all over the table.
A few nights later, she watched Bentley fall into a deep sleep, during which he began to shake and twitch uncontrollably. The next morning — Saturday, Feb. 28 — she took Bentley to the pediatrician who was covering for his regular doctor.
The pediatrician diagnosed Bentley with a virus. Almarode was skeptical and noted that he had no sign of a virus, such as a fever. She asked whether Bentley might be developing migraines, which she suffered from. The doctor seemed noncommittal but offered to put the child on Topomax, a potent anti-seizure drug used to prevent or treat migraines.
“I don’t think so,” Almarode remembers telling him. “I was on it, and it makes you feel like a zombie.” She told the doctor she wanted to discuss the medication with her husband first.
She took Bentley home and began watching him closely. The normally wiggly preschooler lay on the floor watching silently as his little brother played with his toys. That alone was a huge red flag. Bentley never let anyone — especially his brother — play with his toys.
By Monday morning he seemed a little better, so his parents took him to school. A few hours later, his teacher called.
At the emergency room where his mother took him straight from school, the doctor who checked him out didn’t seem concerned. He, too, told Almarode that Bentley had a virus. His recommendation: Take the little boy home and give him Tylenol and anti-nausea medicine. Almarode, who works as a scheduler in a radiology office, said she pushed for a CT scan of Bentley’s head, worried that he might have suffered an injury from a fall. When the scan came back — it was normal — she recalled that the doctor seemed peeved, particularly when she questioned how a virus could cause such symptoms.
“He told me nothing was wrong with him and advised me to take him to a pediatrician, which he was not,” she said. “He made me feel like an idiot and that I was overreacting.”
Once home, Almarode wondered if Bentley’s headaches might signal a problem with his vision. She made an appointment with an optometrist, who agreed to see Bentley three days later.
Almarode was at work when her husband called from the optometrist’s office. He tersely told her he was taking Bentley to Charlottesville. For the second time that week, Almarode grabbed her purse and ran out of her office. “I knew something was wrong,” she remembers telling her husband as she jumped into his car for the 45-minute drive. “I just needed someone to listen.”
One of the first tests U-Va. doctors performed was an MRI scan of Bentley’s brain. Liu said that instead of finding a large cancer, as they expected given his symptoms, the scan showed a Chiari malformation in which tissue from the cerebellum protrudes into the spinal canal.
But a Chiari shouldn’t cause high intracranial pressure, a life-threatening condition. A normal reading for a 4-year-old is about 10 mmHG, Liu said; Bentley’s pressure at times rose into the 50s. “Anything over 20 makes neurosurgeons really nervous,” he said.
After further testing, Liu suspected that the malformation was not the cause of Bentley’s problem but the result of it. His headaches, vomiting and stupor were caused by pseudotumor cerebri — essentially, a false brain tumor. “Patients behave like they have a brain tumor,” Liu said. “But the brain looks completely normal.”
Pseudotumor is most common in young obese women, but it sometimes occurs in children. If no underlying cause is found, as in Bentley’s case, the disorder is called idiopathic intracranial hypertension; it affects about 1 person in 100,000.
In many patients, Bentley among them, the problem results from narrowed or obstructed veins in the transverse sinuses, areas beneath the brain critical to the drainage of blood. In Bentley’s case said Liu, an assistant professor of neurosurgery, the Chiari appears to have developed as a result of the increased pressure on Bentley’s brain. Without treatment, he was at risk of a brain bleed or permanent blindness.
There are three ways to treat pseudotumor. The first, using medications to lower the rate of spinal fluid production, is not optimal, Liu said, because it doesn’t address the underlying cause. The second involves running a shunt from the brain to the belly to relieve pressure on the brain; shunts require replacement and can become blocked. The third and newest option, devised about a decade ago, involves using a metal stent to open the blocked veins, relieving the pressure and restoring blood flow.
“Stenting made the most sense to me — if it could be done safely,” said Liu, who specializes in treating blood vessel disorders. But, he noted, the procedure is new — it is not clear how long the stents will last — and the devices are designed for adults, not children. Most are threaded up the femoral vein in the groin and into the transverse sinuses.
Liu had a long conversation with the Almarodes during which he presented the options and said he thought that stenting posed the best chance for a good outcome. Bentley’s parents agreed.
On March 10, Liu and his colleagues tried for an hour to thread the stent from Bentley’s groin to his skull. Liu said the procedure had to be aborted when it was clear the stent could not be safely pushed into the sinus.
At that point, Liu said, he wondered whether the best approach might be to access the vein by drilling a tiny hole in the back of Bentley’s skull. Liu had used such an approach to treat blood vessel disorders and cancer, but never pseudotumor. Nor was he aware, he said, of other doctors who had done so.
“I wanted to make sure I wasn’t crazy,” he added, so he contacted nearly a dozen neurosurgeons in Charlottesville and around the country. Opinions were decidedly mixed. “Half of them said, ‘You’re crazy,’ and the other half said, ‘It makes sense.’ ”
“I was very open with the family,” Liu recalled, when presenting the novel approach. Among the risks were a brain bleed or a blood clot.
Kim Almarode said Liu sidestepped her question to him: What would you do if it was your child? She said she and her husband were supportive because they trusted the neurosurgeon who had used such an approach successfully in other cases. “It wasn’t new to him,” she said.
On March 12, Liu and his colleagues performed the procedure. Within 24 hours Bentley’s intracranial pressure began dropping. Three days after his operation, Bentley was well enough to go home.
Two weeks later, a follow-up MRI showed that the Chiari had begun to recede, something Liu said he has never seen. A recent MRI was normal, Almarode said, and Bentley appears to have fully recovered.
Almarode said the experience convinced her of the importance of trusting her instincts when it comes to her children’s health. “One of the worst parts of this,” she said, “was hearing over and over again that I was overreacting.”