Radical surgery offers hope for baby racked by seizures
Dylan Catania was born with what his surgeon called “a big, bad brain†that left him racked with seizures and could lead to severe disability. Would severing half of it give him a chance at a normal life?
Dylan Catania was born with what his surgeon called "a big, bad brain" that left him racked with seizures and could lead to severe disability. Would severing half of it give him a chance at a normal life?
Times photographers Francine Orr and Don Kelsen spent months with Dylan Catania and his family, documenting his parents' decision to go forward with surgery and the long weeks of recovery. Their video and photos give readers an intimate look at the journey.
Glossary of Terms
Brainstem: The part of the brain continuous with the spinal cord. Includes a bundle of nerves connected to the spinal cord that allow the body and brain to communicate.
Cerebral cortex: An outer layer of gray matter surrounding the cerebrum. The cerebral cortex carries out most aspects of "higher," or "executive," brain function, such as planning, analysis, thought and memory.
Cerebral hemisphere: Half of the brain.
Corpus callosum: Wide, flat bundle of white matter connecting the two halves of the brain.
Electroencephalography (EEG): The recording of electrical activity using electrodes placed along the scalp to detect how neurons are firing within the brain.
Hemimegalencepahly: A rare disorder in which a baby is born with one-half of the brain enlarged and malformed, leading to epileptic seizures.
Hemispherectomy: Brain surgery to remove half or a portion of half of a patient's brain that is damaged. Often used to bring seizures under control.
Hippocampus: Greek for "seahorse," which it is shaped like. A bundle of nerves deep inside each hemisphere, this brain structure is involved in long-term memory and spatial navigation.
Internal capsule: Bundle of white matter in each hemisphere that connects the cortex and the brainstem.
Magnetic resonance imaging (MRI): Diagnostic imaging technique that uses a magnetic field and radio waves to produce highly detailed images of the body.
PET: Positron emission tomography, a specialized imaging technique that uses short-lived radioactive substances to produce three-dimensional colored images of those substances functioning within the body. PET scanning provides information about the body's chemistry not available through other procedures. Unlike CT (computerized tomography) or MRI (magnetic resonance imaging), techniques that look at anatomy or body form, PET studies metabolic activity or body function. PET has been used primarily in cardiology, neurology, and oncology.
Shunt: A tube that carries fluid from one body part to another. For instance, to relieve fluid buildup in the brain, surgeons can install a tube in the skull that drains into the abdominal cavity.
Thalamus: Two small, egg-shaped structures of gray matter in the front of the cerebral hemispheres that serve as way stations, relaying sensory information to and from the cortex.
Ventricle: A channel for cerebrospinal fluid in the brain.
Interview
Q&A with Dylan's Doctor
Dr. Raman Sankar is a professor and chief of pediatric neurology at Mattel Children's Hospital UCLA in Westwood. Sankar, who treated Dylan Catania, answered questions for the Los Angeles Times about Dylan's condition. Readers are invited to submit additional questions to [email protected].
Q: Why did you recommend that Dylan's parents agree to surgery to sever half of his brain?
A: Before surgery, Dylan was having as many as 50 to 100 seizures a day. For this condition, which I have seen for over 20 years at UCLA, this is the only treatment that gives you control over the seizures and gives you a chance at development. Some of the children don't do as well as Dylan.
Recovery of development after such a procedure is very highly dependent on the structural and functional integrity of the remaining hemisphere -- in this regard, thus far Dylan appears to be quite lucky.
Q: Do doctors know what causes hemimegalencephaly?
A: No. It's not an intrauterine infection or something that we can readily identify. We know it happens early around the time the neurons are multiplying and migrating to assume the normally specified positions. It happens early in conception. Everything we do is sort of controlled by genes, but this is a complex process that doesn't fit an inheritable pattern. We can't say it is inherited like diseases like cystic fibrosis or sickle cell anemia.
Q: How can a child live with half a brain?
A: To exist as a living thing, you don't need a whole lot of brain. You just need to look at insects and rodents. Animals that have a much smaller brain size can do amazing things. But they don't have language, they can't do art.
Much of the cerebral cortex, which supports our higher functions such as language, artistic sensibility, fine motor skills, etc., is not necessary for basic life support. These functions are in deeper structures such as the brainstem, to control breathing, circulation and so on.
The normal brain matures based on relevant sensory input. Normal hearing is necessary to organize the auditory cortex, and vision is necessary to mature the visual cortex, and so on. If one side is consistently producing seizures, which is electrical noise, that's not the stimulation you want for the good side of the brain -- you want it to hear the mother cooing at the baby and, say, Bach!
In essence, it becomes a deprivation if all your brain can perceive is seizures. And that's the reason we undertake the surgery early -- so that the structurally normal hemisphere, whether it's the right or left, can acquire all of these skills at a time in development where it can learn most effortlessly.
Q: What were the risks of surgery? The side effects?
A: There's a certain amount of risk in every surgery: You may die from anesthesia; you may never wake up. Every surgery involves a certain amount of exposure to infection, to bleeding. In brain surgery, unless you're in expert hands, the potential for a catastrophic stroke or bleeding is profound. Incomplete surgery can result in residual seizures later, thus compromising expected development.
We waited for Dylan to barely [weigh] 5 kilograms: 10 pounds -- not very big. There isn't an international standard for when you should do [the operations]. We've been doing this for a while and have an extraordinary team to support the neurosurgeon. We do a much larger number of the younger cases. But that is in part because of our culture.... We're not the type of place that will say we'll wait until you're 2 years old. That's a period of phenomenal development of the brain. It's a period of enormous brain plasticity, brain wiring, and we don't want all of that electrical noise shaping it.
Q: If the Catanias had not agreed to surgery, how would you have treated Dylan?
A: With lots of medicine. But then he would have the double jeopardy --frequent seizures, but also the potential toxicity of excessive medications on brain development.
Q: Is there the possibility of a less radical treatment in the future?
A: Not for this disorder. I cannot picture it right now. That would be nice as a science fiction fantasy -- like you have a switch that turns everything off. But for the foreseeable future, we will be offering this treatment. In this disorder, one entire hemisphere is in chaos. That's why we take such a radical approach. There are other conditions where we do a very, very selective surgery.
Q: Did the surgery work for Dylan? What is his prognosis now?
A: You never know fully, but I would say his prognosis is very good because we did it early. Post-surgically he's not having seizures and is making progress. We've done it long enough to say that it's very, very important that six months out from the surgery Dylan hasn't had any seizures.
We have high expectations for Dylan's overall outcome. That is partly because of the information we had even before the surgery -- that is, what we could discern by many modes of investigation (MRI, PET, EEG) suggested normal structural and functional attributes of his remaining hemisphere.
Even if the seizures start again when he is 3, it's not such a big deal because he has had the time to develop. Our expectation is that he is going to do well.
Dylan is making good eye contact, smiling responsively and visually tracking. Above all, he is not experiencing seizures. I have great hopes for him to become an inspiration for other families and to showcase what is sometimes possible even in the face of a very severe brain condition that threatens the future of a precious baby.
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Family's Blog
Dylan's sister and parents share their experiences throughout his treatment and surgery.
Research & Resources
Nonprofit organization helps patients and families before and after radical brain surgery.
Dylan Catania was born with what his surgeon called "a big, bad brain" that left him racked with seizures and could lead to severe disability. Would severing half of it give him a chance at a normal life?
The baby boy lay motionless at the center of the windowless operating room. The room gleamed under fluorescent lights that illuminated the wisps of blond hair lining his scalp.
Inside Dylan Catania's skull, nerves were misfiring.
The right half of his brain was larger than the left, and malformed. The condition, extremely rare, was causing as many as 100 epileptic seizures a day.
To save their son's mind, Dylan's parents had agreed to the unthinkable: an operation to sever half his brain.
In operating room seven at Mattel Children's Hospital UCLA in Westwood, an anesthesiologist lifted the nearly 3-month-old baby onto a bed of white sheep's wool spread across the stainless steel operating table and placed a mask over his nose and mouth. Within minutes, he was unconscious.
Dr. Gary Mathern, a pediatric neurosurgeon, scrubbed in. Under his arm, he carried a well-worn wooden case containing his surgical loupes, or magnifying glasses.
A nurse swabbed Dylan's head with an antiseptic and a local anesthetic, then turned him on his side, his pale pink head snug in a black U-shaped pillow. Over his right ear, medical residents had scrawled “SA yes†in black marker, for “surgical area.â€
Mathern drew a “T†across the right side of Dylan's scalp to guide his incision.
He and a nurse draped the baby with blue plastic, stapling the edges until all that showed was the 5-inch-long oval of scalp he planned to remove.
Dylan's body held just about enough blood to fill a soda can, about 14 ounces. He was expected to lose it all during surgery, to be replaced with blood given by his father.
Mathern would have to cut a careful path around the delicate blood vessels and arteries that fed Dylan's young brain, removing damaged tissue without harming the rest.
The surgeon raised his scalpel.
It was 9:15 a.m.
“Call Mom and Dad and tell them we're beginning.â€
***
Dylan Philip Catania was born Jan. 16. He weighed 6 pounds, 5 ounces, and measured 19 inches long.
His parents sent out birth announcements with a heart-shaped photo of their 9-year-old daughter, Bella, holding Dylan. The card said: “You have made my life complete.â€
The pregnancy had been a welcome surprise. Jeff and Ellen Catania had not expected to have another child. Ellen, 46, was a fundraiser for the Geffen Playhouse; Jeff, 59, a fundraiser for Children's Institute Inc., which helps abused and neglected children.
Ellen, like many older mothers, had a full range of prenatal tests. All the results were normal. So was the delivery by caesarean section.
They were preparing to take Dylan home from the hospital when he had his first seizure.
Jeff and Bella had carried their bags down to the family's gray minivan. Alone in the hospital room, Ellen held Dylan, 5 days old, staring at his tiny brown eyes. They looked strange, vacant. Then they rolled up into his head.
Panicked, Ellen pressed the nurse call button. She ran into the hall, clutching her son, calling for help.
The baby's eyes were twitching rhythmically.
A nurse summoned a neonatologist. Dylan was admitted to intensive care. Tests were ordered. He would not be going home.
Jeff, shocked and worried, also felt relieved: For now, the hospital was the safest place for his son.
Bella couldn't understand.
“Daddy, please don't do this,†she said. “Let's take him home.â€
Jeff took his daughter aside.
“This little baby cannot look out for himself,†Jeff said. “So we have to.â€
A month later, on Ellen's 47th birthday, the Catanias sat down with Dr. Mathern. It was time to make a decision.
“Parents have referred to this as the doom and gloom lecture,†the surgeon said briskly as they settled into an examining room at the hospital.
“It's not meant to frighten you,†he said.
The couple smiled nervously as Jeff fed their son a bottle. Mathern is an expert on Dylan's rare condition, known as hemimegalencephaly. No more than two dozen children with the condition are born each year in the U.S. It was not inherited, he said. It had nothing to do with their ages. Researchers believe it develops shortly after conception, as the fetal brain is forming. The cause is unknown.
Jeff and Ellen sat surrounded by anatomical posters of the human brain and illustrations of seizures. On a computer screen loomed the black and white MRI image of Dylan's brain. The right cortex was oversize and distorted. It was smooth where there should have been ridges. Landmark brain structures were out of place or missing.
Mathern explained the risks of not operating.
As with all newborns, Dylan's higher brain, or cerebral cortex, was still developing, creating millions of new nerve connections. Those vital connections make us who we are, allowing us to read, write, feel, remember and communicate.
The seizures were like noise in Dylan's brain, a blast of loud rock music. So far, the noise was confined to the right side. Eventually, it would probably spread to the left, disrupting nerve connections and lowering his IQ.
Even with medication, there was a 90% chance that Dylan would suffer so many seizures by the time he was a toddler that he would be mentally retarded, with an IQ below 50. He would be unable to talk or to recognize his parents.
The surgeon moved on to the risks of operating. The surgery, called a hemispherectomy, was radical. Only about a dozen were performed at UCLA each year. Not all were successful.
Even with advanced brain imaging, doctors cannot always isolate and remove from an infant's brain the part where seizures originate.
Since 1986 about 200 patients, most children, had undergone hemispherectomies at UCLA to treat various seizure disorders. One died, an 8-month-old baby boy with Dylan's condition who lost a massive amount of blood. For 70% to 80%, surgery succeeded in controlling their seizures.
After surgery, the left side of Dylan's brain would take over for the the right side, a process called neural plasticity. The younger the patient, the greater the plasticity. So doctors at UCLA recommend operating before a baby is 6 months old.
But even success came with a trade-off.
There are limits to plasticity. Dylan's left hand and foot would remain slightly paralyzed, and he would probably have a blind spot on his left side. He would need extensive physical therapy to keep up with his peers.
To the surgeon, a little paralysis was a small price to pay for the ability to think and communicate.
***
An image of the baby's brain, red and glistening, filled a monitor next to the operating table. Dylan's skull had been drilled open, flaps of skin and rubbery brain covering peeled back and secured with elastic bands, metal hooks and black plastic clips.
Portions of his frontal, central and temporal lobes were exposed, the part of his brain that would help him hear, speak and move.
On the brain's surface, Mathern had spread white postage stamp-size sheets of electrodes. They transmitted nerve impulses, converted into pulsing lines on the screen. The dozens of lines should have been frequent, high waves. Instead, they were long and low, a clear sign of a malfunctioning brain.
Mathern felt vindicated. They had made the right decision.
The surgeon touched the brain's surface with a gloved finger. It felt rigid, firmer than it should be. The ridges were distorted -- some small and tightly coiled, others so enlarged they crowded out other brain structures.
“You want to see a bad brain? That's a bad brain,†Mathern said to a resident.
Mathern slipped on his green surgical mask, grabbed his wooden case and extracted his surgical loupes, replacing his glasses with the magnifying lenses.
He grasped what looked like oversize tweezers, a cauterizing tool he used to burn away brain tissue near the Sylvian fissure, a dividing line down the side of the hemisphere. He headed straight for the middle cerebral artery.
If he severed and sealed the artery quickly, he could prevent potentially fatal blood loss. Dylan would suffer a mini-stroke, but the damage would be only to the portion of the brain about to be removed, 35% to 40% of the right hemisphere.
Mathern reached the artery, cut and sealed it.
Blood gushed momentarily. The resident bathed the area in saline solution, suctioned that away and squirted a surgical foam to staunch the bleeding.
Mathern cut parallel to the underlying ventricle, a channel for cerebrospinal fluid. As he worked, the resident suctioned up burned tissue.
By 2:40 p.m., they had cleared a “doughnut hole†along the ventricle surrounding a central block of tissue. Mathern undercut the block, severing the right brain's connection to the brainstem and body.
The inner edge of the baby's skull was exposed, a brilliant white.
Nurses wheeled over an operating microscope. Loupes were not powerful enough to probe the deep, cramped recesses of the brain the surgeon was about to delve into.
“We're doing the disconnection,†he said.
It was time to separate Dylan from what Mathern called the big, bad half of his brain.
***
The Catanias had nagging doubts after meeting with Mathern. That night, back at their house on a steep Beverly Glen hillside, they wondered if they were doing the right thing for their son.
“Even doing the surgery, there's no guarantee that it's going to work. That's crazy, isn't it?†Ellen said.
As they sat in the dining room, Dylan slept in his father's arms.
Jeff argued that surgery was the best option.
“As a parent, you have to make these decisions, however painful they may be. Parents do it every day,†he said.
Dylan's condition had worn them down. They had to monitor and document every seizure in a worn blue spiral notebook -- some lasting up to eight minutes, a few so severe they rushed him to the emergency room. They had to administer his three seizure medications, a powerful cocktail that included the narcotic Phenobarbital, which left Dylan groggy, his brown eyes perpetually half-closed. He was still sleeping in their room -- they had not had time to assemble his crib.
Ellen was breast-feeding every few hours. Dylan weighed 8 pounds, 10 ounces, and doctors wanted him to gain more than 4 pounds before his surgery in two months. Jeff had not functioned on so little sleep since his college days.
“I just worry that the surgery won't work, that he'll need a lot of care,†Ellen said. “Quite frankly, we're not young parents. We both work; we have to work. How are we going to do all we can for him?â€
Jeff reminded her that they had a strong network of family and friends. Ellen looked away. Dylan twitched, the start of another seizure.
The surgery was set for April 9, nearly three months after his birth.
Six days before the operation, Dylan was baptized.
Neither Ellen nor Jeff was particularly religious. The baptism was mostly a hedge against Ellen's greatest fear: that the surgery to save her son's life would kill him.
It was the day before Easter, and Westwood United Methodist Church was decked with fragrant lilies. Together with family and friends, the couple carried Dylan to the altar. He was dressed in a white, double-breasted satiny suit with matching cap and booties. He had not suffered a seizure all day, allowing his parents to relax and enjoy the celebration.
“May the Lord lift up the light of his countenance on you and give you peace,†the minister said, raising Dylan into a shaft of sunlight streaming through the stained glass windows.
***
Under the operating microscope, Dylan's brain looked like white jelly laced with fine red lines -- blood vessels.
In babies with hemimegalencephaly, the vessels are easily broken, or “friable.†Among them lurked a sleeping dragon, the anterior cerebral artery, a pulsing extension of the internal carotid artery that supplied blood to Dylan's brain.
“Use the artery like a freeway,†Mathern told the resident as their gloved hands moved beneath the microscope.
The two worked together delving deeper and deeper until they reached the base of the skull. There lay the control center that connected the two sides of Dylan's brain, a wide, flat bundle of white matter called the corpus callosum.
Removing the corpus callosum is considered the toughest part of the operation, the “deep disconnect.†If the surgeon leaves any of the tissue behind, it could continue to trigger seizures. And he must remove it without disturbing the nearby great cerebral vein, or vein of Galen.
Mathern acted quickly and deliberately, suctioning out the white matter to the edge of the vein of Galen and removing the right hippocampus, a brain structure full of neurons that plays a role in memory.
The surgeon paused to survey his work.
“From here to there he's disconnected.â€
Mathern had left much of the right side in place, where it would help with normal growth and skull formation. Nerves would continue to misfire in the remaining portion, but, severed from Dylan's body, they should no longer trigger seizures.
Blood filled the cavity Mathern had created. The monitors surrounding the operating table glowed with Dylan's vital signs, all stable.
It was 3:55 p.m.
The resident stitched the brain covering back together and fastened the missing oval of skull into place with titanium screws. He inserted a temporary drain and stapled shut the T-shaped incision. It was too soon to know for sure whether the operation would put an end to Dylan's seizures. In 20% to 30% of cases at UCLA, seizures persist even after surgery.
In coming days, Dylan's brain would swell and might require a second operation to install a permanent drain, or shunt.
But for the moment, they were done.
***
Mathern was still in his green scrubs when he found the Catanias in a corner of the waiting room, surrounded by half-empty coolers and gift baskets.
It was about 4:20 p.m., more than seven hours after he had started. The crowd of family and friends had grown so large, Mathern asked them to follow him out to a veranda, where sun streamed through magenta bougainvillea.
“This was a very severely abnormal case,†he said.
Mathern explained that without surgery, problems on the right side of Dylan's brain would have worsened. With time, his seizures would have intensified and he might have lost the use of the left side of his body. They had made the right decision.
“He's never going to know. To him, this is life.â€
***
Dylan was waking up.
He opened his eyes first, wider than before the surgery. He glanced to the left, then to the right. He cried, kicked both feet and waved his right arm in a circle as if conducting an invisible orchestra.
Days after the operation, his scar was still raw, but doctors said he was doing well. Inside his brain, the lining was irritated, but the nerves seemed to have stopped misfiring.
A nurse helped Jeff stabilize the drain in Dylan's head and cradle his son in his arms. Dylan looked up and into his father's brown eyes, mirror images of his own.
Jeff thought: “For the first time, he really sees me.â€
Yet his parents still froze at every hiccup, afraid it might be the start of a seizure.
“Slowly, I'm letting go of that anticipation,†Jeff said.
The Catanias had befriended a mother whose son had the same condition as Dylan. The 3-year-old had just survived his third brain operation.
She brought her laptop over, and Jeff and Ellen watched in one video as the boy's left eyelid twitched.
“This was his first seizure after the surgery,†the mother said.
“Fear of the return -- it never goes away, does it?†Ellen asked.
The other mother shook her head.
***
It has been 261 days since Dylan's surgery.
He is a cheerful baby. His eyes follow the action in a room. He holds his head up, smiles, flirts, rolls over on his own and is starting to sit up and eat solid food.
He is small for his age and, even with daily physical therapy, he is developmentally delayed. Doctors say his language skills are low to average, but the Catanias have decided not to worry about the usual milestones.
They celebrate small achievements, like when their son clasps his left hand, a side he rarely uses.
“We still don't know how this is going to play out for him,†Jeff said. “I'm hoping he's a risk-taker, that he is able to rationalize, to think.â€
So far, Dylan remains seizure-free.
Brainstem: The part of the brain continuous with the spinal cord. Includes a bundle of nerves connected to the spinal cord that allow the body and brain to communicate.
Cerebral cortex: An outer layer of gray matter surrounding the cerebrum. The cerebral cortex carries out most aspects of "higher," or "executive," brain function, such as planning, analysis, thought and memory.
Corpus callosum: Wide, flat bundle of white matter connecting the two halves of the brain.
Electroencephalography (EEG): The recording of electrical activity using electrodes placed along the scalp to detect how neurons are firing within the brain.
Hemimegalenchephaly: A rare disorder in which a baby is born with one-half of the brain enlarged and malformed, leading to epileptic seizures.
Hemispherectomy: Brain surgery to remove half or a portion of half of a patient's brain that is damaged. Often used to bring seizures under control.
Shunt: A tube that carries fluid from one body part to another. For instance, to relieve fluid buildup in the brain, surgeons can install a tube in the skull that drains into the abdominal cavity.
Ventricle: A channel for cerebrospinal fluid in the brain.
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