Intrathecal Baclofen
Selective Rhizotomy
Deep Brain Stimulation
Orthopedic Surgery

Intrathecal Baclofen

Baclofen is a drug that helps reduce spasticity and dystonia. Taken orally, little Baclofen enters the spinal fluid, spinal cord or brain. If Baclofen is given directly into the spinal fluid, it soaks into the spinal cord and is far more effective, with far fewer side.

See for Treatment of Spasticity

See for Treatment of Dystonia

Selective Rhizotomy

A rhizotomy is an operation in which a nerve or part of a nerve is intentionally cut. Lumbar rhizotomies are operations on the lower back to partially divide nerves from the legs. Selective lumbar rhizotomies are operations in which the neurosurgeon divides the various nerves coming into the spine from the legs into several branches, tests each branch with an electrical stimulus, then cuts the branches which give abnormal responses. There is debate as to whether selective lumbar rhizotomies give better results than non¬selective rhizotomies. The problem in deciding is that both operations are effective and no one knows if one is better.

Screening/Selection Criteria

Candidates for a rhizotomy are usually young (four to eight years old), have relatively good leg strength, and do not have severe leg contractures. The primary goal of surgery is often to improve walking. Rhizotomy can be done at any age to facilitate care. Rhizotomies will relieve the spasticity but will not improve contractures (shortening of muscles and tendons) that are already present, nor will they improve dystonia.

Surgery

Rhizotomy surgery generally lasts about two to three hours. The procedure involves a midline incision about 3-4 inches long in the lumbar region. Muscles are separated away from the spine and the nerve roots coming and going to the legs are exposed. Each nerve root divided into 3-5 branches and is tested with special monitoring equipment to identify nerves that give abnormal responses when they are electrically stimulated. The nerve roots that give abnormal responses are cut; usually 50- 60% of the top half of each nerve is divided.

Expectations

Children are at bed rest for 2-3 days postoperatively. They are hospitalized for 3-5 days and then go home. We ask them to wait for one month after operation to start intensive physical therapy. The frequency of PT varies with the goals, from 1-2 times a week if the goal is to improve range of motion to 4-5 times a week if the goals are to improve strength and walking.

Myths/Facts

1. MYTH: Selective rhizotomy is usually permanent but the effects sometimes wear off.
   
   FACT: Whenever children get significantly tighter a few months or years after rhizotomy, it is almost always because they have dystonia (which is not improved by rhizotomy) rather than because their spasticity has returned.
2. MYTH: Rhizotomies have a high complication rate.
   
   FACT: The complication rate is surprisingly low: 5-10%, lover than the rate of complications for insertion of baclofen pumps.

Deep Brain Stimulation

Deep brain stimulation (DBS) is a method of treating dystonia and tremor that involves an operation in which thin blunt wires (electrodes) are surgically implanted precisely into a small area deep in the brain. If the abnormal movement affects one side of the body, one electrode is inserted (on the opposite side of the brain than the body is affected). If both sides of the body are affected, bilateral (both sides) electrodes are inserted. The electrodes are tunneled under the skin down the neck and are connected to an electrical stimulator unit than can be programmed with a computer to stimulate the area of the brain at the tip of the electrode. The idea behind DBS is that fast electrical stimulation (130 times a second) interrupts the abnormal electrical circuit within the brain that is causing the abnormal movements.

At the present time, we have an FDA approved protocol to study the use of DBS in children 12-21 with severe dystonia. We have also used DBS to treat tremor in a few teenagers. DBS is not presently approved for the general (non-research) treatment of movement disorders in children.

Screening/Selection Criteria

Candidates for DBS usually have severe dystonia that has not responded adequately to oral medications or to ITB. They are 12-21 years old (at present) and do not have any other medical condition that might increase the risks of DBS.

Implantation

The operation in children is done under a general anesthetic. Patients are anesthetized then a stereotactic frame (a metal frame marked like a ruler) is attached to their head and an MR scan is done. The target for the electrodes is determined by selecting its location on the MR scan, then calculations are made based on markings on the frame to determine the trajectory and depth the electrodes need to be inserted. A small hole is drilled in the skull and the electrode is guided to the desired spot, which is usually within one millimeter of the desired location. The wires are then tunneled under the skin and connected to the stimulator. The stimulator is turned on the next day and is adjusted frequently during the first few weeks after operations to improve the movements as much as possible.

Follow-up

Patients are seen approximately every two weeks for adjustments postoperatively, then at one, two and three years.

Expectations

DBS is too new to know how well it will work. Based on studies done in Europe, it appears to be very beneficial for children with primary (genetic) dystonia and somewhat less effective for dystonia due to CP or head injuries, but the numbers of patients treated so far are too small to come to any firm conclusions.

Orthopedic Surgery

Surgery is performed to increase the ease of care, improve function and on occasion, particularly in the upper extremity, to improve the appearance. Both bony and soft tissue surgery are involved. All four extremities can have surgery at the same setting. Although quite time consuming and initially a bit more uncomfortable, it allows the patient to recover more appropriately because most of the deformities have been corrected at once.

Procedures

The major soft tissue procedure involves lengthening the muscle-tendon unit. This usually involves cutting the tendon where it overlaps the muscle belly. This stretches the entire unit which improves the length of the extremity. On occasion, particularly at the Achilles tendon, the tendon is partially cut in 3 areas through tiny stab wounds during the skin. This allows the tendon to lengthen appropriately without a large incision. Post-operatively, casting is needed for both methods for 4-6 weeks.

Bony surgery (osteotomy) is performed at the upper femur (thigh bone) to reposition the femoral head (ball) into the acetabulum (cup) and to realign inappropriate rotational deformities. Osteotomies are also performed at the lower tibia (shin bone) to correct malrotation deformities. Internal fixation of some sort is needed to hold the bones together until they heal. Sometimes, and particularly in the tibia, additional external support is needed with a cast.

Spine surgery is sometimes needed to correct deformities as well. If needed, the patients are referred to our spine surgery colleagues .

Expectations

Because the cerebral palsy still exists, the patient cannot ever be returned to a normal status. However, at a minimum, care is usually significantly improved. In ambulatory patients, their gait is usually improved. In the upper extremities, the appearance and care are improved. By testing, assuming independent activity is present, the function is usually improved. However, unless this is the dominant extremity, the overall function may not be improved. Post-operatively, physical and occupational therapy are necessary to regain the strength and agility needed to gain the appropriate level of function. This may take 3-12 months depending on how much surgery is done. At the hip, when repositioned and especially when done early, the risk
of degenerative arthritis is significantly diminished. If the hip remains out of position, arthritis will occur and lead to pain and leg deformity.

Follow-up

Post-operative follow up is needed for cast removal and x-ray evaluation to determine the healing of the osteotomies. Additional follow-up is needed to access the progression of the therapy. For patients who live far away from Pittsburgh, we do try to arrange follow-up visits with a local orthopedic surgeon if they are willing to do so.

Myths/Facts

1. MYTH: Surgery corrects everything and makes the patient normal .
   
   FACT: Surgery will improve the alignment of the extremities. If decent function is present, function should improve but it depends on how well the muscles can be controlled and how well the patient works with therapy.
2. MYTH: Surgery only needs to be done one time.
   
   FACT: With soft tissue surgery, the younger the patient, the more likely that it may have to be repeated in several years, especially at the Achilles tendon. However with spasticity reduction (baclofen pump/rhizotomy), the incidence of recurrent surgery has significantly diminished.
3. MYTH: It is okay to leave a partially dislocated hip alone for a long time.
   
   FACT: The longer the hip is partially dislocated, the more serious the deformity becomes and the more degeneration will occur. The earlier the deformity is corrected, the more remodelling may occur and the more likely the hip will become nearly, if not completely normal. Sometimes, when done early enough, only soft tissue surgery is needed. If done late, sometimes bony surgery will need to be done on both the femur and pelvic sides of the hip.