Cerebral Palsy


174 Cerebral Palsy


Vedant Kulkarni MD


Shriners Hospital for Children – North California, Sacramento, CA, USA


Clinical scenario



  • Twin siblings with bilateral spastic cerebral palsy (CP) secondary to prematurity, present at age 7. Twin A walks with ankle foot orthoses (AFOs) and the support of a walker – a Gross Motor Function Classification System (GMFCS, level III evidence). His parents report one year of increasing fatigue with walking, problems with balance and tripping, and decreasing tolerance of his AFOs. He walks with a jump gait pattern, up on his tip toes, with his feet and knees rotated internally, and flexed at his knees. His physical examination reveals bilateral equinus contractures involving the gastrocnemius (but not soleus), medial hamstring contractures, and increased femoral anteversion.
  • His brother, Twin B, uses a power wheelchair and is able to stand with assistance for transfers (GMFCS, level IV). His parents note decreased hip abduction over the past few years but this does not interfere with care, and he has no hip pain with sitting, standing, or transfers. An anteroposterior (AP) radiograph of the pelvis demonstrates a hip migration percentage (MP) of 35% bilaterally.

Top three questions



  1. Does multilevel orthopedic surgery (MLS) improve gait outcomes for children with ambulatory CP?
  2. Is three‐dimensional gait analysis (3DGA) essential for surgical decision‐making for children with ambulatory CP?
  3. Does surveillance for hip displacement result in improved outcomes for nonambulatory children with CP?

Question 1: Does multilevel orthopedic surgery (MLS) improve gait outcomes for children with ambulatory CP?


Rationale


Ambulatory children with CP have gait abnormalities due to tendon contractures, bony torsional malalignments, joint instability, muscle weakness, and abnormal muscle activation patterns, which can limit their independence and participation in activities. The musculoskeletal consequences of CP get worse over time. Left untreated, children with bilateral CP experience deterioration of gait and function as they approach puberty and adolescence.13 This can manifest as a decrease in walking endurance (decreased walking distance and fatigue), poor stability, increasing reliance on walking aids, pain, and reduced physical or recreational activity. The previously common practice of multiple episodes of orthopedic surgery addressing abnormalities at a single level at a time over successive years, has been replaced by simultaneously performed MLS that addresses abnormalities at multiple levels of both lower limbs under a single anesthetic and episode of hospitalization, followed by an intensive course of rehabilitation.


Clinical comment


The objective of MLS is to optimize lower limb biomechanics – restore muscle length and balance, and bony alignment, in order to preserve or improve gait‐related function and appearance and to promote independence and increased participation. Additional surgery may be required in the future as a child grows.4 The effectiveness of such resource‐intensive interventions deserves clarification.


Available literature and quality of the evidence



  • Level II: 1 randomized controlled trial (RCT)5 and1 prospective cohort.4
  • Level III: 1 retrospective cohort.6

Findings


In a small RCT by Thomason et al., patients were allocated to either MLS (n = 11) or progressive resistance strength training (n = 8).5 At 12 months, the MLS group had a 35% improvement in the Gait Profile Score (GPS), an improvement over 4 times the minimally clinically important difference (MCID) of 1.6°. The patients in the MLS group were followed to 24 months, showing a clinically significant improvement of 4.9% in their Gross Motor Function Measure‐66 (GMFM‐66) score. The five‐year outcomes of the same MLS cohort showed improvements from baseline of 64% in GPS and 3.29% in GMFM‐66. The Functional Mobility Scale (FMS) improved in nearly half the children at 50 m and 500 m, and no child had an FMS rated worse than before surgery.4


In 2018, Dreher et al. reported the outcomes of a retrospective cohort of 231 patients with bilateral CP who underwent MLS at three high‐volume centers that utilized 3DGA for decision‐making and outcome assessment.6 At one year, GPS improved by an average of 5° which was maintained at a mean of nine years after surgery. Seventy‐seven percent of children maintained their improvement in the long‐term. Up to 40% of children required additional but smaller interventions at a later date.


The outcome measures used in these studies are primarily measurements of gait impairment derived from 3DGA (GPS), and physiotherapist observed functional measures (GMFM‐66). The FMS measures the type of walking aid used for different walking distances (5 m, 50 m, and 500 m). Although studies do not adequately evaluate patient‐reported outcomes (PROMs) that are aligned with the goals of patients and parents, a recent study has shown a strong correlation between the GPS and the GOAL questionnaire, which is a goal‐based PROM derived from the priorities of children with ambulatory CP and their parents.


Resolution of clinical scenario


Evidence from one small RCT and one large multicenter retrospective cohort (without controls) study of prospectively collected data over the long term suggests that Twin A would benefit from appropriately selected MLS, particularly if performed at a high‐volume center utilizing 3D gait analysis with access to experienced rehabilitation personnel. The functional outcomes he and his parents seek are likely to be achieved in the short term and maintained in the long term. However, he might require additional surgery before the end of growth.


Question 2: Is three‐dimensional gait analysis (3DGA) essential for surgical decision‐making for children with ambulatory CP?


Rationale


Although MLS might be beneficial, the question remains whether 3DGA is necessary to inform which operations to include for MLS, and whether this leads to improved outcomes over MLS performed without 3DGA to guide decisions. 3DGA has the potential for such a benefit, since gait deviations in CP are often multiplanar and complex interactions of pathologic and compensatory patterns that are difficult to interpret and quantify with observational gait analysis (OGA) alone. 3DGA in a motion lab is the best technology available to objectively quantify gait deviations and has greatly improved our understanding of the biomechanics of pathological gait patterns in children with CP.


Clinical comment


Surgical decision‐making in ambulatory CP involves integrating information from the history of a patient’s gait problems, physical examination, including observation of gait, patient goals, and expectations, lower extremity imaging, in addition to 3DGA.7 In order for 3DGA to be deemed essential for surgical decision‐making, the data from 3DGA should be reliable, should alter surgical decision‐making at least some of the time, and should lead to improved patient outcomes. This is crucial to establish because there are many centers that perform MLS without access to, or use of, 3DGA.


Available literature and quality of the evidence


3DGA reliability



  • Level II: 2 prospective diagnostic studies.8,9Level III: 6 retrospective diagnostic studies.1015

3DGA alters decision making



  • Level II: 1 RCT.16
  • Level IV: 1 retrospective cohort.17

3DGA improves outcome



  • Level I: 1 RCT.18

Findings


3DGA reliability


Concerns were raised in 2003 by Noonan et al. about the variability of data generated by different motion labs testing the same 11 patients.8

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May 14, 2023 | Posted by in Uncategorized | Comments Off on Cerebral Palsy

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