70 Cerebral Palsy and Selective Dorsal Rhizotomies

Case 70 Cerebral Palsy and Selective Dorsal Rhizotomies


Jean-Pierre Farmer and Abdulrahman J. Sabbagh


Image Clinical Presentation




Image

Fig. 70.1 Computed tomography scan of the head showing presence of periventricular leukomalacia without hydrocephalus.


Image Questions




  1. What are your surgical treatment options?
  2. What key features would you like to elicit in the history to determine that this young boy is a candidate for selective dorsal rhizotomy?
  3. What physical features would make you think that he is a candidate for this procedure?
  4. What features, if present in the history or physical examination, would make you rule against offering rhizotomies?
  5. What factors would favor offering rhizotomies in your interpretation of the imaging?
  6. What other investigations would you like to obtain on this child prior to planning rhizotomies?
  7. What can you tell the family with respect to the potential outcome for the child with selective dorsal rhizotomies as opposed to continuing with physiotherapy and occupational therapy alone?
  8. If there is involvement in the upper extremities, how would this influence your decision in terms of offering rhizotomies versus a baclofen pump?
  9. What is the relative cost of the two treatment procedures over a lifetime?
  10. What are the risks and long-term sequelae following selective dorsal rhizotomies?
  11. What would you recommend your anesthetist to use intraoperatively during stimulation?
  12. What criteria would you use to determine your lesioning pattern?
  13. What do you do to reduce the risk of sphincteric difficulties in these patients?
  14. How do you control pain and spasticity perioperatively?
  15. What adjuvant treatment will the child benefit from in the 2 months following the procedure?
  16. What is the expected long-term outcome with respect to lower extremity function, activities of daily living, and upper extremity fine motor control following rhizotomies?
  17. Is the preoperative status of the patient a strong determinant with respect to the long-term outcome of the child?

Image Answers




  1. What are your surgical treatment options?

  2. What key features would you like to elicit in the history to determine that this young boy is a candidate for selective dorsal rhizotomy?

    • A history of prematurity, low birth weight, late acquisition of motor milestones, but progress being made.1
    • Other key points: The absence of previous surgical interventions, the absence of movement disorders other than spasticity, the absence of a family history involving neurologic diseases, and a good collaboration with therapists are all positive features.

  3. What physical features would make you think that he is a candidate for this procedure?

    • Candidates should show evolving locomotor skills with adequate balance in the sitting position and good protective responses in the short leg sitting position.
    • Patient should show no associated movement disorder such as chorea or choreoathetosis and should show velocity-dependent increased tone and hyperreflexia with clonus, as well as limited range of motion.1
    • There should be adequate underlying strength with the squat-to-stand testing.
    • Patient should show at least quadruped falling or bunny hopping.
    • If he or she shows upright locomotor function, this should be even better.

  4. What features, if present in the history or physical examination, would make you rule against offering rhizotomies?

    • Absolute contraindications include the presence of multiple orthopedic releasing procedures for short tendons, choreoathetosis, double hemiplegia, the inability to collaborate with therapists, associated significant cognitive difficulties, and dislocated hip.4
    • Relative contraindications include atypical perinatal history with birth at term, but with associated confounding factors such as meningitis, trauma in the neonatal period, or other prenatal factors.4
    • Hydrocephalus, if well dealt with, is not an absolute contraindication.
    • Other absolute contraindications would be severe motor restrictions or the presence of severe scoliosis preoperatively.

  5. What factors would favor offering rhizotomies in your interpretation of the imaging?

    • Imaging of the brain with a CT scan or preferably magnetic resonance imaging (MRI) should show presence of periventricular leukomalacia (Fig. 70.1) without hydrocephalus.
    • Presence of basal ganglia damage would be a relative contraindication.1
    • The spine x-rays should not show significant scoliosis.
    • The hip x-ray should show at least 50% or better femoral head coverage.

  6. What other investigations would you like to obtain on this child prior to planning rhizotomies?

    • Other investigations would include the presence of gross motor measure scores, alignment scores, occupational therapy grading, and urodynamic testing.5

  7. What can you tell the family with respect to the potential outcome for the child with selective dorsal rhizotomies as opposed to continuing with physiotherapy and occupational therapy alone?

    • The family has to be aware that randomized control trials have revealed an advantage of doing rhizotomy over simply continuing with physiotherapy and occupational therapy alone.
    • Abundant literature attests that there are persistent significant gains in gross motor function measure (GMFM), in urodynamic profile, and the upper extremity, as well as activities of daily living gains, which are durable and substantial.1

  8. If there is involvement in the upper extremities, how would this influence your decision in terms of offering rhizotomies versus a baclofen pump?

  9. What is the relative cost of the two treatment procedures over a lifetime?

    • The relative cost of the two procedures is about 4:1 (baclofen pump compared with rhizotomies).
    • Baclofen pumps remain a significantly more expensive way to treat spasticity.1

  10. What are the risks and long-term sequelae following selective dorsal rhizotomies?

    • Long-term sequelae of selective dorsal rhizotomies are few if the procedure is done with stimulation and in a moderate lesioning fashion.
    • Reported problems include a higher incidence of scoliosis, which has to be compared with the natural history of the disease.6
    • In our experience, the rate of scoliosis is relatively high; however, the scoliosis curves are almost exclusively between 10 and 15 degrees, which present a debatably low clinical significance.6
    • Hyperlordosis and spinal stenosis have also been reported, although these are very rare. The clinical importance is again similar to the scoliosis cases, where cases of significance are the few ones who have undergone laminotomy or laminoplasty procedure.6
    • Bladder dysfunction is also a rare event.5

  11. What would you recommend your anesthetist to use intraoperatively during stimulation?

    • During surgery, if done with stimulation, the anesthetist should use a combination of sufentanil or remifentanil at a low dose and propofol with nitrous oxide.
    • The neuromuscular junction transmission and the spinal cord transmission should not be altered by the anesthetic regimen during stimulation.4

  12. What criteria would you use to determine your lesioning pattern?

    • The criterion most frequently used is that of spread to contralateral or upper extremity segments from stimulation of lumbosacral dorsal roots.
    • Afterdischarges and amplitude of stimulus response also play a role if spread is demonstrated to contralateral or upper extremity segments.7

  13. What do you do to reduce the risk of sphincteric difficulties in these patients?

    • Care has to be taken to preserve S3 and S4 roots and to limit the lesioning at S2.
    • We tend to limit lesioning to 50% of both dorsal S2 roots combined, preserving at least one third of S2 dorsal rootlets on each side.
    • With this pattern we have not identified long-term problems with bladder dysfunction and have even documented improvements in urodynamic profile.1

  14. How do you control pain and spasticity perioperatively?

    • Postoperative pain is usually related to the length and depth of the incision, but also to some element of deafferentation as a result of the sectioning of the nerves.
    • An epidural catheter placed for the delivery of epidural morphine at T9–T10 that is above the surgical site is very helpful.1
    • Additionally, oral diazepam can be beneficial but should be given at half of the recommended dose to avoid depressant effects on respiratory drive.

  15. What adjuvant treatment will the child benefit from in the 2 months following the procedure?

    • Most centers will recommend enhanced physiotherapy and occupational therapy with the use of a pool therapy, arts and crafts and the use of an exercise program to stretch resistant contractures, strengthen musculature, and date training.1

  16. What is the expected long-term outcome with respect to lower extremity function, activities of daily living, and upper extremity fine motor control following rhizotomies?

    • Lower extremity function using objective measurement such as the GMFM score and alignment scores shows durable improvements, as do activities of daily living and upper extremity fine motor control following rhizotomies.4,8,9
    • These improvements have been found to last up to 5 years postoperatively and still improving at that point.8

  17. Is the preoperative status of the patient a strong determinant with respect to the long-term outcome of the child?

    • Yes, the better the patient is preoperatively, the more likely the outcome will be favorable.
    • In more favorable cases, gains will bring the patient closer to the normal age-matched controls with respect to motor and upper extremity function.1
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Jul 16, 2016 | Posted by in NEUROSURGERY | Comments Off on 70 Cerebral Palsy and Selective Dorsal Rhizotomies

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