Five-year-old Will DeMauvise has never been stopped by his cerebral palsy—in family videos, he swims, throws a baseball and drives a toy car. He and his parents want the same thing for him: a full life with as much independence as possible.
Injections of botulinum toxin and phenol into Will’s legs helped relax his rigid, spastic muscle and increased his range of motion. But because the injections are very painful, he needed to go under general anesthesia each time. And the effect was short-lived.
“We had to do it every three to four months, two to three times a year,” says Will’s mother Marlee. “Every time he did it he had to go under. It was heartbreaking.”
Because his leg muscles were so tight, Will needed a walker to get around. His gait was unsteady and stiff-legged. Unable to take broad strides, he walked on his toes with a “scissoring” motion, crossing one leg tightly over the other.
“We were looking for a way to offer him more independence through something long-term,” says Marlee.
Serendipitously, she learned that Shenandoah Robinson, MD, a neurosurgeon at Boston Children’s, was offering such a solution—a one-time spinal operation called selective dorsal rhizotomy (SDR).
For muscles to work properly, they need to both contract and relax. But in children with spasticity, the muscles don’t relax. Instead, certain sensory nerves keep telling the motor nerves that the leg muscles need to be tighter. SDR breaks this harmful feedback loop by cutting some of these rogue nerves, allowing some “relax” signals to get through.
SDR isn’t for every child with spasticity. “The best candidates are kids whose spasticity affects mainly the legs, who are able to walk alone or with a walker or canes, and who are old enough to participate in the intensive physical therapy afterwards,” says Robinson. “That requires a certain level of thinking and cognition, and a certain amount of stamina and attitude.”
Robinson carefully evaluated Will and decided he qualified: He was bright, determined, and still young enough that SDR could help his muscles and limbs develop more normally, sparing him stigma at school and perhaps future operations.
Strange as it sounds, however, the operation was likely to make Will worse before making him better. Because his trunk muscles were weak, he had been compensating by relying on the strength in his taut legs to propel him forward, awkward as his gait was. What if he lost what little he had?
“We knew we were running a risk with Will and his low tone,” says Marlee. But although his Will’s core muscles were weak, Robinson felt he had enough trunk control and could work on strengthening his core after surgery.
Two months later, in a four-hour operation in March, Will lay on his stomach under general anesthesia, hooked up to a machine that monitored the electrical activity in his legs. Robinson made a cut about an inch long in his lower spine, removing a small section of bone to expose the nerve roots. Under a microscope, she carefully isolated the sensory nerves and stimulated each little rootlet in turn, guided by the monitor, cutting those that responded most abnormally.
When Will woke up, his pain closely controlled with anesthesia, he had noticeably less spasticity. Within less than a week, he was bearing weight on his legs. “He could lift his leg and climb the stairs—actually pull his leg high enough to the next stair,” says Marlee. “He takes wider steps and doesn’t scissor his legs anymore. He has a wider base to stand on.”
As predicted, Will was also more wobbly and weak than before. But intensive physical and occupational therapy, along with swimming and horseback riding, has started to turn that around.
“After the surgery, he realized very quickly that he could do more things with his legs—he could see the progress and kept working harder and harder,” says Robinson. “That’s great to see because that carries over to school and everything else.”
He entered kindergarten last fall full of determination. He’s doing well, though his progress has stalled a bit. “He had a growth spurt, which always sets these kids back a little,” says Robinson. The family also recently relocated to Arizona, and Will is adjusting to his new physical therapy team there.
“Will has a long road ahead of him,” says Marlee, “but his attitude makes all the difference.”