Multidisciplinary expertise, advanced rehabilitative and surgical protocols, and the use of state-of-the-art diagnostic technology enable Gillette Children’s Specialty Healthcare to provide the most up-to-date management of movement disorders and related spasticity and motor delay.
One of the most common causes of pediatric motor disability and spasticity is cerebral palsy, a disorder that affects an estimated 500,000 children in the U.S., according to the Nemours Foundation. Gillette is one of the top cerebral palsy treatment centers in the world, staffed by a team of neurologists, neurosurgeons, orthopedic surgeons and pediatric rehabilitation specialists who are uniquely equipped to partner with community pediatricians and offer comprehensive treatment strategies.
Multidisciplinary Evaluation of Motor Delays
While the most common pediatric movement disorder is cerebral palsy, motor delays may also stem from underlying metabolic disorders or be idiopathic in nature, according to Marcie Ward, MD, pediatric rehabilitation medicine specialist at Gillette.
Designed to reveal the root cause of a child’s motor dysfunction, the Motor Delay Diagnosis and Treatment Clinic at Gillette is a one-stop referral resource for the families of young patients who have delays in their gross or fine motor skills consistent with cerebral palsy. These delays may include missing age-related milestones, such as rolling over, walking and crawling, abnormal gait patterns, and left- or right-hand dominance that is noticeable before children reach 18 months of age. Children with hypotonia, those who were born prematurely and those with a history of perinatal asphyxia are also candidates for a cerebral palsy assessment.
During the Motor Delay Diagnosis and Treatment Clinic evaluation that follows referral, families have an opportunity to meet with a pediatric rehabilitation medicine specialist, neurologist and orthopedic surgeon, as well as with members of the physical, occupational and speech therapy teams, on the same day. This not only enhances convenience, but also enables families to benefit from the expertise of all providers who are typically responsible for making a cerebral palsy diagnosis and managing the condition.
“The neurologist conducts an in-depth review of the child’s birth history, as well as any previous diagnostic testing or imaging that’s been performed, to assess the etiology of the delay or impairment,” Dr. Ward says. “The physiatrist does many of the same things, but we also look at the child’s function and development, and how we can maximize and further improve development despite limitations, delays or neurological deficits. The orthopedic surgeons assess children’s bone and joint health to rule out abnormalities, such as hip dysplasia, that might contribute to walking delays or an abnormal gait.”
Gait analysis uses data to drive healthcare decisions.
The results from these analyses guide treatment decisions. The sooner children are evaluated and begin treatment, the better their long-term outcomes.
“Early intervention helps put a child on the right path to achieve his or her greatest developmental potential and can potentially prevent future medical co-morbidities or complications,” says Nicole Williams Doonan, MD, pediatric neurologist at Gillette. “The longer problems remain unaddressed, the harder it is for children to change course or catch up with their peers.”
Depending on the results of the evaluations, management strategies may include the use of orthotics and assistive devices, referrals to other specialists, and physical, occupational and speech therapy. Neurologists at Gillette also guide the medical management of epilepsy, autonomic dysfunction, movement disorders and headaches, as necessary.
A certified orthotist in Gillette Children’s Specialty Healthcare’s in-house lab customizes ankle foot orthoses for a patient.
If children live outside the region or otherwise have to travel away from their communities to receive specialized care at Gillette, the Motor Delay Diagnosis and Treatment Clinic team works with physicians in patients’ own communities to coordinate close-to-home services.
All-inclusive Care for Pediatric Spasticity
Roughly 80 percent of children with cerebral palsy have spastic cerebral palsy, according to the CDC. Children with this condition have increased muscle tone that contributes to rigidity and impaired movements.
Gillette offers multidisciplinary, comprehensive evaluation of spastic cerebral palsy, as well as multiple conservative and surgical treatment modalities aimed at managing muscle tone and resolving any underlying orthopedic bone and joint deformities that may contribute to movement irregularities.
For children identfied by their physicians as possibly having spasticity, the journey to evaluation and treatment begins at the James R. Gage Center for Gait and Motion Analysis, a center that has a worldwide reputation as a leader in assessing movement disorders. Here, physical therapists guide patients through a series of tests using advanced technology. During a walking test, for example, infrared cameras are used to capture pictures of the skeleton — particularly the relationship of the joints to one another. Therapists also administer plantar pressure testing to collect information that can be analyzed for a better understanding of gait patterns and the distribution of pressure under the feet. Gait analysis also includes a thorough physical exam as well as an electromyogram.
Data derived from these studies is used to inform decision-making about treatments and, specifically, whether or not a child is an appropriate candidate for selective dorsal rhizotomy.
“Once all of the data is collected, a certified gait interpreter will meet with the therapist and an engineer from the gait lab to go through the data and video and learn about patients and their history,” says Michael Healy, MD, pediatric orthopedic surgeon at Gillette. “From there, we do an interpretation and begin to form opinions around what the best course of treatment is going to be.”
“Some of the things we look for following the gait analysis include muscle overactivity and high-energy oxygen consumption that is at least two times the normal expenditure for a similarly aged peer,” adds Tom Novacheck, MD, Director of the James R. Gage Center for Gait and Motion Analysis and pediatric orthopedic surgeon at Gillette. “In terms of movement, we also look for muscle stiffness in the knee during stance and swing, as well as excessive plantar fasciitis during stance and swing.”
A Meeting of the Minds
Following the gait analysis, families come to the Spasticity Evaluation Clinic for an assessment. Like in the Motor Delay Diagnosis and Treatment Clinic, this multidisciplinary clinic gives families an opportunity to meet all of the physicians who may be involved in their child’s care at the same appointment. The providers involved in spasticity evaluation and treatment include pediatric rehabilitation medicine specialists, pediatric neurosurgeons and pediatric orthopedic surgeons.
As part of the evaluation, children may have MRI or CT scans, which enable neurosurgeons to look at the brain or spinal cord pathology contributing to spasticity. The pediatric rehabilitation medicine specialists also evaluate how spasticity impacts a child’s function, while the orthopedic surgery team analyzes how spasticity affects the child’s skeletal development.
Rehabilitation is an integral part of treatment plans.
“The multidisciplinary clinic gives parents a comprehensive view of what lies ahead,” Dr. Healy says. “We educate parents about rhizotomy and the four-to-six weeks of rehabilitation that follows. We also speak with parents about the orthopedic concerns causing difficult ambulation. Most of the children who are selected for rhizotomy are ambulatory, so we discuss any improvements in skeletal alignment that can be made after tone reduction to correct dysfunction. This comprehensive discussion gives families a realistic idea of what’s expected and allows us to better work with them on shared goal-setting.”
Available treatment modalities in addition to selective dorsal rhizotomy include physical therapy, onabotulinumtoxinA and phenol injections, medication, and the use of casting or orthotics. Children that have widespread high muscle tone in their arms and legs may also be candidates for placement of an intrathecal baclofen pump. This device delivers doses of baclofen directly into the intrathecal space, which allows physicians to lower the dose needed to reduce muscle tone and lessen the risk of systemic side effects.
A State-of-the-art Procedure
Selective dorsal rhizotomy is a complicated three- to four-hour surgery during which neurosurgeons identify and cut the sensory nerve roots contributing to the abnormal signals responsible for spasticity.
Michael Healy, MD, orthopedic surgeon, Mark Gormley Jr., MD, pediatrician and rehabilitation specialist, and Debbie Song, MD, neurosurgeon, develop a care plan with a patient and family.
“Rhizotomy entails unroofing bones from the back of the lower spine and isolating the nerve roots that carry information that may be affecting spasticity,” says Debbie Song, MD, pediatric neurosurgeon at Gillette. “On both the right and left side, we tease each of the dorsal roots into tiny rootlets and intraoperatively use electronic impulses to simulate the rootlets. The physiatry team helps us monitor the signals, and we cut the rootlets carrying the abnormal messages that contribute to spasticity.”
After surgery, children complete an intensive, four- to six-week inpatient rehabilitation program. This is followed by outpatient therapy that takes place five days a week for the first month or two after surgery.
“Initially, we restrict walking so children can regain their strength and gross motor skills,” says Angela Sinner, DO, pediatric rehabilitation medicine specialist at Gillette. “During inpatient rehabilitation, children spend time on their stomachs to build core strength and practice kneeling and sitting positions to strengthen underdeveloped muscles. Towards the end of the inpatient program, they perform supervised walking, and we work with children to adopt a heel-to-toe reciprocal gait pattern.”
One year after surgery, children have a postoperative gait analysis to evaluate the efficacy of the procedure.
Exploring Candidacy for Selective Dorsal Rhizotomy
Children who are candidates for selective dorsal rhizotomy are typically ambulatory and have spastic diplegia in which high muscle tone and stiffness most commonly occur in the legs. In recent years, however, indications for rhizotomy have expanded to include certain children with spastic quadriplegia who are not candidates for an intrathecal baclofen pump, and older children and teens who are ambulatory and have severe spasticity, according to Dr. Song.
Contraindications for selective dorsal rhizotomy include children who have cognitive disabilities that may prevent successful completion of a rehabilitative program, as well as children who have significant muscle weakness or dystonia.
“We typically advise against rhizotomy in cases where the procedure may make dystonia worse,” Dr. Song says. “Similarly, if children have muscle weakness, we counsel patients and their families that if we perform surgery and take away that tone, they’ll have weakness after surgery, which may complicate their postoperative recovery.”
Resolving Comorbid Orthopedic Deformities
Many children who are candidates for rhizotomy also have underlying bone and joint deformities that require surgical correction. In roughly 90 percent of these cases, selective dorsal rhizotomy is performed first, with orthopedic surgery following at least one year later. Procedures follow this order for several reasons.
After orthopedic surgery, children may have muscle spasms. Spasticity may worsen these spasms, so reducing spasticity prior to surgery can enhance patients’ comfort following orthopedic surgery, according to Dr. Novacheck. Once spasticity is treated, orthopedic surgeons may also need to adjust the surgical plan. For example, if muscle tone is reduced, surgery to resolve soft tissue contracture may no longer be necessary.
“If spasticity is managed prior to surgery, we can stay focused on bone deformity correction,” Dr. Novacheck says. “Orthopedic surgeons at institutions that don’t have tone management and rhizotomy programs have to do more surgery on the soft tissues of the legs.”
Exceptions to this treatment order include children who have hip dysplasia or other significant torsional deformities that may interfere with the ability to perform rehabilitation prior to rhizotomy. In these cases, orthopedic surgery may be performed prior to the rhizotomy procedure.
Gillette offers the full spectrum of pediatric orthopedic surgeries. Common procedures performed to resolve bone deformities in children with spasticity and cerebral palsy include femoral derotational osteotomy and reconstructive foot surgery.
If children have multiple deformities, orthopedic surgeons at Gillette perform single-event multilevel surgery (SEMLS) during which two teams of orthopedic surgeons work simultaneously to resolve all deformities at the same time. Gillette team members are innovators in this technique, which prevents the need for multiple, painful orthopedic surgeries and rehabilitation.
“SEMLS enables us to put children through one single surgery and rehabilitation period,” Dr. Healy says. “This enhances pain management, limits hospitalizations and leads to better long-term outcomes. This is also where gait analysis helps — we get a comprehensive picture of what we need to do so we can achieve the goal of addressing multiple concerns while performing only one surgery.”
To refer a patient to Gillette Children’s Specialty Healthcare for a spasticity or motor delay evaluation, call 651-325-2200.