Cerebral Palsy Surgery Lagging Research?

Patients with cerebral palsy (CP) present special problems to their orthopedists. Often a single patient must be treated for numerous issues involving spasticity, problems with gait, large joints, the spine and extremities. Therein lies the question: since so many CP patients are children, should they be treated with multiple procedures spaced throughout their adolescence to take into account their growing bodies, or should multiple procedures be done all at once?

According to United Cerebral Palsy 1.5 to 2 million children and adults have CP in the United States. An additional 10, 000 infants are diagnosed each year along with an estimated 1, 500 pre-school children. Their diagnoses fall into five areas:

1. Hemiplegic Cerebral Palsy – affects one side of the body
2. Diplegic Cerebral Palsy – affects all four limbs, although weakness and spasticity are more severe in the lower limbs
3. Quadriplegic Cerebral Palsy – affects all four limbs to an equal extent
4. Monoplegic Cerebral Palsy – affects a single limb
5. Unspecified Cerebral Palsy – extent affected is not specified

Patients with CP are further classified by their level of severity under the Gross Motor Function Classification System (GMFCS). This ranges from Level 1, very few symptoms of cerebral palsy, to Level 5, which covers severe physical restrictions requiring full assistance.

The levels are:

1. Patient has few indications of CP
2. Patient uses an orthotic and may have trouble with stairs
3. Patient needs crutches or walkers
4. Patient needs an electric wheelchair
5. Patient requires full time care

The treatment goal for ambulatory children at levels 1 and 2 is to improve the patient’s ability to walk. For those classified in levels 4 or 5, the goal is to increase comfort and improve posture. Physicians have traditionally achieved these goals by performing standard tenotomy (cutting of a tendon) and osteotomy (division of bone to reposition or adjust alignment) procedures numerous times throughout adolescence as patients mature.

This treatment involves many trips to the hospital, an increased amount of physical therapy, interruption of schooling and a diminished quality of life. Mercer Rang, Ph.D., has termed these multiple interventions on children with CP a dreaded “Birthday Syndrome” because the lengthy procedures and recovery times often keep children in the hospital even during their birthdays when they end up celebrating with nurses instead of with family and friends. It seems increasingly evident that the alternative of performing multiple procedures at once would greatly increase a CP patient’s quality of life, but would it still effectively treat the patient’s condition?

Research conducted over the past decade indicates that instead of spacing out multiple surgeries over a period of years, performing a single-event, multilevel surgical (SEMS) procedure can actually yield better results. Most recently, researchers like Spiro et al (who studied 84 children with spastic CP who underwent SEMS procedures) and Otsuko et al (who studied 57 patients treated for sagittal imbalance and gait disturbance) published in the article “What’s New in Pediatric Orthopaedics”, in the Journal of Bone and Joint Surgery, May, 2007, both concluded that their results validated the use of SEMS.

With the help of advanced gait analysis and computer-based systems which are continually improving the ability to break down motion and study it in detail, physicians are now more able to diagnose various CP conditions. In many of the recent studies, both motor function and walking scores—along with quality of life—improved for children treated with a SEMS procedure.

Despite these promising studies, one look into the PearlDiver Patient Records Database reveals that physician practice has been slow to reflect the research. Chart 1 shows the percentage of 389 patients who underwent multiple tenotomies and osteotomies versus those who had SEMS procedures.

Chart 1: Single Event Multilevel Surgical Procedure Frequency

Source: PearlDiver Patient Records Database 2004 – 2007

In 2004, 51.9% of children with cerebral palsy underwent a SEMS procedure. Despite the fact that researchers continued to release studies noting the effectiveness of SEMS procedures over the alternative of multiple procedures over a period of time, by 2007 the number of SEMS procedures had increased only seven percentage points to 58.9% over the course of three years. With the potential to drastically improve a CP patient’s quality of life, one would instead hope to see an equally drastic shift toward SEMS procedures.

Graph 2: Single Event Multilevel Surgical Procedure Trend

Source: PearlDiver Patient Records Database 2004 – 2007

This graph shows the trend as research continues to bolster the practice of SEMS procedures, though 41.1% are still being treated with multiple procedures.

Fortunately, the push toward the single-event multilevel surgical procedure has led physicians to improve spasticity management and delay the surgical procedure until patients are older. Dr. Hank Chambers, President of the American Academy for Cerebral Palsy and Development Medicine says,

Our goal with spasticity management, physical therapy and bracing is to delay the surgery as long as we can...until about age seven or eight.

He believes that by using gait analysis and spasticity management, physicians can decrease the number of procedures, increase quality of life and improve patient results when patients have a SEMS procedure.

Dr. Chambers notes that children will usually have one more growth spurt and several more procedures may have to be done. This is in sharp contrast to the older school of thought that starts procedures as early as age two because of the belief that early corrections will allow children to grow and develop more like those without CP.

The key to successfully delaying surgery lies in the new forms of spasticity management. In the ambulant patients, typically GMFCS levels 1 through 3 with focal spasticity (spasticity which creates a focal problem treatable by local means), surgeons can now inject Botulinum toxin typa A (BTX-A) directly into problematic muscles, blocking the neurotransmitter acetylcholine which initiates involuntary muscle contractions. For patients with pure spasticity (having limited or no dystonia, muscle contractions causing a twisting of the affected body part), an additional option used in patients level 2 or 3 is the selective dorsal rhizotomy. This procedure involves severing problematic nerves that don’t receive gamma amino butyric acid, which helps to control nerve impulses. For non-ambulatory patients—GMFCS levels 4 or 5 who also suffer from involuntary muscle contractions, an additional option is the intrathecal baclofen pump—which delivers medication directly to the spinal fluid.

These additional options allow for better spasticity management and the delay of major procedures, which further opens the door for the single-event, multilevel surgical procedure.

Table 1 shows that the number of tenotomy procedures for children under the age of five has decreased since 2004. Table 2 shows that changes in the number of patients who underwent osteotomies were far more dramatic, dropping from 23% for children under five in 2004 to 8% in 2007. Looking forward, the number of osteotomy procedures performed on patients under five will have little room to decrease further, and the number of tenotomies performed on this age group also seems to have leveled off.

Table 1: Age Trend of Tenotomy Procedures

Source: PearlDiver Patient Records Database 2004 – 2007

Table 2: Age Trend of Osteotomy Procedures

Source: PearlDiver Patient Records Database 2004 – 2007

Surprisingly, while the percentage of children under the age of five being treated with tenotomy and osteotomy procedures has dropped substantially, the percentage increases in other age groups has not followed the expected pattern. From 2004 through 2007 children between the ages of five and nine receiving an osteotomy, tenotomy or both, increased only slightly. For both procedures, the largest increase was in the age group of 15 to 19 and not in the expected five to nine group. The advances in spasticity management are clearly helping physicians delay surgery for patients under age five.

So the dramatic change in CP surgery trends is happening not in the number of SEMS procedures over multiple, separate procedures but in the timing of the surgeries. The majority of patients still undergo tenotomy and osteotomy procedures in the five to nine age group, and with continued growth spurts, many of these patients may need more than one procedure to fully treat their condition. However, the fact that physicians can delay surgery should help increase the number of successful SEMS procedures. The numbers may not be increasing as sharply as one might hope, but the door is open for continued progress. Physicians will hopefully be able to continue on this trend so that the treatment of cerebral palsy will make fewer interruptions in patients’ lives, allowing them more time—and more birthdays—with friends and family.

For more articles by this author, please select the following link: http://www.pearldiverinc.com/pdi/large.jsp.