Saturday, October 11, 2008

Brain surgery changes toddler's life in a positive way

Lily looks and acts like most toddlers. The 16-month-old grabs toys, smiles and babbles to her parents.

She loves crowds and basks in attention.

A speech therapist says Lily's speech is appropriate for her age and requires no special work, at least for now. Her vocabulary includes "dada," "no" and "all gone."

But Lily's parents, Lisa and Paul Rossignol, astound people by describing a surgical procedure Lily underwent in April.

Surgeons removed the right half of Lily's brain.

Doctors in Cleveland, Ohio, used the procedure, called a hemispherectomy, to control epileptic seizures that became more frequent and violent over time.

"They call them salaam seizures because it looks like she's bowing," said Lisa Rossignol, Lily's mom. "The body actually jackknifes."

The seizures came on gradually. When Lily was 2 or 3 months old, Lisa noticed that her daughter was lethargic and showed strange, rolling eye movement after a nap.

"I knew that something wasn't right," said Lisa, a 29-year-old advertising professional. "I was just sick about it."
Within weeks, Lily's seizures became more severe. On Nov. 3, Lisa drove her daughter to the emergency room with a series of massive seizures. "She could barely get a breath they were coming so fast," Lisa said. "I was screaming and driving down the highway."

Doctors say Lily suffered a stroke before she was born that left large cysts, or fluid-filled voids, in her brain. The damage led to a condition called infantile spasms, a dangerous type of epilepsy marked by debilitating and life-threatening seizures.

Epileptic seizures are much like electrical storms in the brain that interrupt both brain and body functions. Shortly before her surgery, doctors estimated that Lily experienced some 350 seizures a day, said Paul Rossignol, Lily's father.

"She pretty much was seizing in some form all day long," said Paul, a certified acupuncturist who has remained home much of the past year to care for Lily.

"She didn't babble much," he recalled. She rarely laughed or slept. "Before the surgery, we probably saw her laughing two or three times."

The frequent seizures so weakened Lily that she became susceptible to disease, said Dr. Doug Postels, a pediatric neurologist at Presbyterian Medical Group who diagnosed Lily with infantile spasms. Lily also experienced paralysis on the left side of her body, which is controlled by the right side of the brain.

"She probably would have died," Postels said. "She would have just kept seizing and seizing until she got pneumonia or something like that."

Postels referred the couple to Dr. Bruce Fisch, a University of New Mexico epileptologist.
Fisch said two-thirds of Lily's right hemisphere was damaged and that Lily needed a hemispherectomy.

The couple experienced some panic after leaving Fisch's office but remained focused on the job ahead. "I don't think we knew what we were getting into, really," Lisa said.

Epilepsy is caused by damage to the brain, Fisch said.

Hemispherectomy is one of several types of surgery used to relieve epileptic seizures by removing the source of the damage, he said. It is used in cases where the damage to the brain is widespread.

Lily underwent two operations at the Cleveland Clinic. The first, called a functional hemispherectomy, essentially disconnected the right hemisphere of Lily's brain. The four-hour surgery on April 4 left the brain intact but dysfunctional, Lisa said.

But Lily's seizures continued, prompting doctors to perform a second surgery on April 14. The two-hour surgery, called an anatomical hemispherectomy, physically removed the right half of her brain.
"That ended her seizures," Lily's mother recalled. "She came out of the second surgery smiling and talking."

Lily's personality emerged almost immediately after the second surgery, Paul said. "It really, really was like a miracle."

Because Lily was only 10 months old at the time of her hemispherectomy, she has a good shot at developing normal language and intellectual abilities, Fisch said.

"The younger you are, the more you can recover from any brain injury," said Fisch, who recommended treatment at the Cleveland Clinic.

The brain's ability to rewire itself in response to an injury is called plasticity. In very young people, the remaining half of the brain can take over the functions of both hemispheres, he said.

"It sounds extreme," Fisch said of hemispherectomies. "It is aggressive. It's a very extensive procedure, but, overall, the outcome is wonderful."
Postels said the void left by a hemispherectomy fills with spinal fluid. The remaining half of the brain seems to remain in place, he said.

The chief complication of the procedure is hydrocephalia, or "water on the brain," which typically can be corrected by a shunt, he said.

Postels also said he is optimistic about Lily's physical and intellectual development.

"Lily will probably never be a ballet dancer, but she probably will look pretty dang good in a couple years," he said.

Lily's parents are hopeful but realistic. They recognize that Lily could face problems with intellectual development. But, without the surgery, Lily faced a bleak future, if any. So far, she's doing well.
"Cognitively, she's doing pretty good," Paul said. "Her speech is good. She makes connections with people. She's talking a lot."


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