Keyvon still checks in with Dr. Sun regularly.
A Little More Space: Keyvon's Story
“All the girls think I’m cute now,” reports Keyvon, age 7. One year after his surgery at Children’s Hospital & Research Center Oakland, the girls’ assessment is indeed accurate. But the problem that prompted the unusual operation on his skull went far beyond aesthetics: Keyvon had a very rare life-threatening condition that had defied diagnosis before his arrival at Children’s.
Keyvon began suffering from debilitating headaches at age 5. “The pain came suddenly and was severe,” Keyvon’s grandmother, Jeanne Danielson, remembers. “In the middle of something, he would say ‘Oh-oh, here it comes’ and then he would lay down until he threw up. Then he would fall asleep. At their worst, the headaches happened every other day.”
As the headaches continued and intensified the family and the boy’s pediatrician wondered whether a vision problem might be the cause. An ophthalmologist didn’t find anything amiss. Then, because the headaches were coupled with unusually high blood pressure, Keyvon was referred to the Nephrology department at Children’s.
Kidney malfunction is often the source of high blood pressure in kids, but, in Keyvon’s case, all tests came back normal. However, nephrologist, Barbara Botelho, MD, noticed that the boy’s eyes were bulging slightly. Dr. Botelho referred Keyvon to pediatric neurosurgeon Peter Sun, MD, who named the source of Keyvon’s symptoms and referred him to plastic craniofacial surgeon Bryant Toth, M.D, at Children’s Hospital’s Comprehensive Craniofacial Center.
Keyvon had a rare form of a serious skull disorder called craniosyntosis. A child’s skull is composed of multiple bones separated by sutures, or openings, that allow for growth. In craniosyntosis, these “growth lines” fuse prematurely, usually before birth, causing the skull to expand in the direction of the remaining open sutures with the result of a noticeably abnormal head shape. Because of this strong visual symptom, most cases of craniosynostosis are easily diagnosed and corrective surgeries are typically done within the first year of the child’s life.
Atypically for the condition, Keyvon’s skull sutures did not close in utero but at some later undetermined time. His head, which for a while had been able to grow along with the rest of his body, appeared to be symmetrical—at first glance. But by the time he came to Children’s, all of Keyvon’s skull sutures had fused. His head size was small for his face. The skull was also heaping up creating a peak -like shape of the top of his head. His brain was developing and expanding, but his skull could not. The terrible headaches and (most likely) the high blood pressure were the result of pressure building in his head—pressure that could be lethal.
“It is very, very rare to see a person with a completely smooth, fused skull,” states Dr. Sun, who chairs Children’s Division of Neurosurgery. Although head growth stops in adulthood, the sutures almost never fuse, or smooth over, the way Keyvon’s did. Drs. Sun and Toth recommended surgery.
Keyvon, his grandmother, and his mother each absorbed the highly unusual diagnosis in different ways. Mom Kanani remembers bursting into tears but can barely recall the conversation that followed the initial news. Grandmother Jeanne was better prepared, but admits to having an urge to bolt out of the room. “I remember that Dr. Sun made a long pause between explaining what was happening to Keyvon and the treatment option,” Jeanne says. “I now realize he was giving Kanani time to digest what she was hearing, but for a split second I though he was taking a breath to tell us that there was nothing they could do. I wanted to run.” Keyvon said nothing, but later asked his grandma, “They are not going to cut my head, are they?”
She answered his question directly: “Just a little, to make some space for your brain.”
That’s a lot for a young boy to think about. A few days later Keyvon said, “I think they’re going to cut this much,” holding his thumb and index finger about an inch apart.
To that, Jeanne responded: “Honey, we trust the doctors completely to cut just as much as you need.”
In the surgical suite on May 23, 2002, Keyvon was in great spirits: he joked and clowned, talking to the nurses about which “tools” they were going to use on him; he pretended to be falling asleep, then opened one eye and, in a melodramatic tone, said “Pray for me.” His energy was undefeatable—and contagious. “We were almost rolling on the floor with laughter by the time he was wheeled away,” Jeanne remembers.
Dr. Toth made an incision that zigzagged from one ear to the other to ensure Keyvon’s scar would remain invisible even with a short haircut. Dr. Sun cut the skull into several pieces according to shape and size determined by Dr. Toth, and removed the bone from the membrane that covers the brain and separates it from the skull. Dr. Toth rotated the sides of Keyvon’s head to eliminate the “peak” that had started to form after the skull had fused. Reconstructing the skull, Dr. Toth used bone paste—a synthetic, porous material that serves as a net and allows the bone to grow through—to increase the space inside Keyvon’s head. To keep the skull pieces together, the surgeons also used plates made of a plastic polymer that the body slowly absorbs. At the end of the procedure, the surgeons had increased the circumference of Keyvon’s head by four centimeters.
During the six-hour surgery, the Neurosurgery department’s Sue Ditmeyer, RN and pediatric nurse practitioner, called the family every hour to keep them informed. “At one point I picked up the phone, and there was Sue’s voice saying ‘His skull is off and he’s doing great,’” Kanani marvels, remembering the incredible call with a smile.
After surgery, Keyvon’s headaches disappeared and his blood pressure dropped. After a brief post-op disappearance, his mischievous spirit returned. “The surgery has changed his life,” Jeanne says, “or more precisely given his life back to him. He used to say he hated his world and that he wanted to hurt himself. Now he tells everybody how girls think he’s cute.” Keyvon’s family voice few regrets: “We only wish we had met you all earlier.”