The three-neurosurgeon team at Gillette Children’s Specialty Healthcare offers expert surgical care for brain and spinal cord disorders. Their success is founded on trusted and established partnerships with colleagues across a multitude of pediatric disciplines.
A shunt to drain cerebrospinal fluid is placed at Gillette Children’s Specialty Healthcare.
Pediatric neurosurgeons Patrick Graupman, MD, Peter Kim, MD, PhD, and Debbie Song, MD, FAANS, work to enhance health and quality of life for children with a variety of complex neurological conditions, including epilepsy, spasticity, dystonia, spina bifida, Chiari malformation, brachial plexus injury, hydrocephalus, traumatic brain injury (TBI), cerebral palsy, moyamoya disease and tethered spinal cord.
Assisting patients who have these complex disorders requires ongoing dialogue with clinicians from a wide range of specialties, including neurology, orthopedic surgery, and physical medicine and rehabilitation. That open, ongoing communication is a hallmark of Gillette’s neurosurgery program, and a true game changer for the patients and families these neurosurgeons help.
“Specialists can’t work in a bubble when treating children with brain and spinal cord conditions,” Dr. Song says. “For example, every child with spina bifida has a tethered spinal cord, and 30 to 40 percent of them will be symptomatic and require surgery to free the cord from surrounding tissues. Other providers play key roles in accumulating the information necessary for surgery, which imaging alone can’t do.”
To evaluate patients with spina bifida, the neurosurgery team refers young patients to the appropriate Gillette experts, such as physical therapists who administer manual muscle strength tests to help determine strength in the context of growth spurts and pediatric orthopedic surgeons who monitor for potential musculoskeletal concerns.
“Many patients with spina bifida have some degree of scoliosis,” Dr. Song says. “Not all of them will have clinical tethered spinal cord symptoms, but if the orthopedist notices scoliosis isn’t following the expected clinical trajectory, we get concerned. Bowel and bladder dysfunction are red flags as well and require the involvement of a pediatric urologist. The actions I take with my patients are often influenced by the findings of my colleagues who are orthopedists, urologists, or physical medicine and rehabilitation physicians.”
To facilitate multidisciplinary collaboration and communication, Gillette neurosurgeons team up with colleagues in other specialties to hold several disorder-specific clinics — most of which meet at least once a month. The clinics include:
- A complex movement disorders clinic comprised of a pediatric neurosurgeon, pediatric neurologist and pediatric rehabilitation medicine physician
- A spasticity management clinic that includes a pediatric neurosurgeon, pediatric orthopedic surgeon and pediatric rehabilitation medicine physician
- A spina bifida clinic with involvement from a pediatric neurosurgeon, pediatric neurologist, pediatric orthopedic surgeon and pediatric rehabilitation medicine physician
Gillette physicians, including the neurosurgery team, also hold a brachial plexus injury clinic and a craniofacial clinic in conjunction with pediatric plastic surgeons.
“Gillette is one of the few institutions in the country with multidisciplinary clinics dedicated to complex movement disorders and spasticity management,” Dr. Graupman says. “In the clinic, the other specialists and I evaluate patients together. We share findings and insights in real time — dialogues that allow us to collaborate with one another and avoid putting patients through separate episodes of care.”
Dr. Kim explains how coordinating with colleagues, each bringing his or her expertise to the table, makes the entire team better diagnosticians and problem solvers.
“As a clinician, there is always a temptation to refer a patient to another specialist if I need more information about a particular aspect of his or her presentation or symptomology,” Dr. Kim says. “If that specialist is there with me, however, we can put our heads together and figure out as a team what’s going on.”
Treating Abnormal Muscle Tone
Spasticity — increased muscle tone that occurs due to focal brain or spinal cord damage — affects approximately 80 percent of individuals with cerebral palsy and multiple sclerosis, according to the American Association of Neurological Surgeons. It can have a profound effect on children’s musculoskeletal function, including the ability to walk. If nonsurgical options, such as oral medications and botulinum toxin injections, fail to manage spasticity effectively, the hospital’s three-pronged spasticity management team can offer two possible neurosurgical interventions: placement of an intrathecal baclofen pump and selective dorsal rhizotomy (SDR) surgery.
Gillette has one of the busiest pediatric intrathecal baclofen pump programs in the country. The pump, which a surgeon places beneath the skin of the abdomen, injects the antispastic drug baclofen into the spinal fluid via a catheter. The pump must be refilled during an outpatient visit every few weeks or months, depending on the dosage.
“Many patients need both focal injections and a pump, as, over time, they often develop a tolerance to injections alone,” Dr. Song says. “We look to the physiatrists and orthopedists who see cerebral palsy patients regularly to advise us as to when patients need better tone control. Our goal, if we put a pump in, is to provide that control for the rest of the patient’s life.”
Certain children, especially those with a combination of lower-extremity spasticity and dystonia, are candidates for SDR surgery. Gillette is among the nation’s top providers of SDR surgery. The ideal patients are ages 4 to 8, able to walk with the help of braces or assistive devices, and willing to comply with intensive postoperative rehabilitation. The rehabilitation process — which is crucial to the SDR surgery’s success — is supervised by the neurosurgery team’s physician colleagues in pediatric rehabilitation medicine.
“In SDR surgery, we make a two- to four-inch incision in the midline of the back, find the sensory nerve roots in the lumbar area that serve lower-extremity function and dissect them into small rootlets,” Dr. Kim says. “We stimulate each rootlet to look for abnormal responses and pinpoint the ones that are driving the spasticity. We cut the abnormal rootlets and leave the normal ones alone, which should cause the patient to lose essentially all abnormal tone without loss of sensation.”
Careful patient selection and intensive follow-up allow the neurosurgeons at Gillette to cut as much as 50 percent fewer nerve roots than other centers, according to Dr. Kim, so their patients are more likely to retain sensation while also experiencing symptom relief. As a result of these optimal outcomes, patients from around the U.S. and world seek out Gillette for SDR surgery.
Alex is doing well following his decompression surgery with Debbie Song, MD.
Unburdening the Brain
Shunt surgery for hydrocephalus — an accumulation of cerebrospinal fluid in the brain’s ventricles that affects approximately two out of every 1,000 children born in the U.S. — is one of the most common procedures performed by Gillette neurosurgeons.
“Often, the decision to place a shunt is obvious, such as when the soft spot on top of a baby’s head is bulging,” Dr. Song says. “Sometimes, however, the decision is more complicated — an infant’s ventricles may be getting bigger on imaging, for example, but she has no other clinical symptoms. We always investigate carefully and obtain input from neurology and imaging to make sure shunt surgery is the most appropriate treatment for each patient.”
A shunt allows cerebrospinal fluid to drain away from the brain via a catheter for resorption. A valve regulates fluid flow through the shunt. Patients who receive a shunt often require it for the rest of their lives. In some cases, shunt placement is the first step in treating multiple concurrent disorders.
“We care for a population of patients who have both hydrocephalus and Chiari malformation — an abnormal invasion of the spinal canal by the cerebellum,” Dr. Song says. “Treating hydrocephalus with shunt surgery first can make decompression surgery for Chiari malformation safer.”
In certain children, an obstruction in a part of the brainstem called the cerebral aqueduct hinders the brain’s ability to absorb cerebrospinal fluid. These patients may be candidates for endoscopic third ventriculostomy (ETV), an alternative to shunt surgery. During ETV, the neurosurgeon uses a neuroendoscope to open a small hole in the third ventricle through which cerebrospinal fluid can drain and bypass the blockage.
The neuroendoscope can also help neurosurgeons make shunts more effective.
“Sometimes, there are sequestered portions of the cerebrospinal fluid system that the shunt isn’t reaching,” Dr. Kim says. “We use a neuroendoscope to connect compartments so the shunt can work on the whole ventricular system. We also have a range of options that allow us to assess when a shunt is and isn’t working and adjust our approach as necessary, including nuclear flow studies and a technology called ShuntCheckT that uses subtle changes in temperature to evaluate flow.”
Calming Dystonia with Deep Brain Stimulation
Deep brain stimulation (DBS), originally developed to treat adults with Parkinson’s disease, was approved by the Food and Drug Administration in 2012 as a treatment for children with dystonia — involuntary muscle contractions that occur without an identifiable cause. Gillette was one of the first two hospitals in the U.S. to offer the procedure for children. To administer DBS, Gillette neurosurgeons and neurologists coordinate to perform a two-stage procedure that takes place over the course of a week. First, a neurosurgeon places electrodes in the brain, which are then connected to a pacemaker-like neurostimulator that rests beneath the skin of the chest. The device sends electrical impulses to areas of the brain, such as the thalamus, subthalamic nucleus and globus pallidus, where the signals prompting dystonia originate. As Co-director of the Complex Movement Disorder Clinic and Rett Clinic at Gillette, neurologist Timothy Feyma plays an important role in planning and administering DBS to patients.
Recently, Gillette began offering DBS to patients with secondary dystonia, which develops as a result of disease or injury to the brain or spinal cord, or may be caused by exposure to certain toxins.
“Gillette and several other centers nationwide are still trying to pin down which subgroups of secondary dystonia patients respond best to DBS,” Dr. Feyma says. “In the past three years, we’ve performed 32 DBS implantations on children as young as 4 with full-term hypoxia, a variety of genetic conditions and post-traumatic brain injury.”
“We’ve seen good results with children who had post-traumatic brain injury at a young age and those who had hypoxia after full-term birth,” Dr. Graupman continues. “Patients have regained function, seen chronic neck pain caused by abnormal movements disappear and been able to do things with their hands they couldn’t do before. More data is necessary to identify which patients respond best to DBS, but the initial findings are extremely encouraging, and we are glad to be on the forefront of this emerging field.”
“The young patients we treat have complex health problems. That makes communication with other clinicians — whether they are our colleagues at Gillette or patients’ referring providers from other health systems — crucial. We embrace a collaborative model of care that makes physician-to-physician communication central to our work, and Gillette is supportive and accommodating of that.”
— Peter Kim, MD, PhD, pediatric neurosurgeon at Gillette Children’s Specialty Healthcare
Hope for Those with Seizures
Children who experience seizures as a result of medically intractable epilepsy may be candidates for vagus nerve stimulation (VNS), a treatment that is similar to DBS in its use of an implantable stimulator to interfere with the brain’s signaling. Here too, the Gillette neurology and neurosurgery teams work together seamlessly to support families considering a VNS procedure for their child — and following the procedure, to monitor the child for seizure activity.
“Offering VNS to children with epilepsy is another example of the multidisciplinary care that is essential in neurosurgery,” Dr. Song says. “Neurologists manage the care of these patients, and we work closely with them to select appropriate candidates for VNS.”
To prepare patients for VNS, the neurosurgeon places an electrode with small coils around the left vagus nerve, which travels from the brainstem through the neck to the chest and stomach. The nerve is responsible for the motor and sensory functions of a variety of organs and structures along its path. The surgeon connects the lead to a generator in the chest wall that electrically stimulates the nerve, which can reduce the frequency and intensity of seizures. The neurologist optimizes the generator’s settings to produce the most effective electrical pulse.
“Like DBS and the intrathecal baclofen pump, VNS has the advantage of being a reversible treatment — it can always be removed,” Dr. Graupman says. “That is one of the core premises of functional neurosurgery and our practice: We want to do reversible therapies first before turning to permanent ones. Doing this helps us realize our ultimate goal — helping our young patients lead lives that are as normal as possible.”
For more information about the neurosurgery team, available treatments and clinic locations, visit gillettechildrens.org/conditions-care/neurosurgery.