Gait correction surgery in children with Hereditary Spastic

Gait correction surgery in children with
Hereditary Spastic Paraparesis (HSP)
J Mahy B.Physio (Hons)1, P Thomason M Physio1,2, HK Graham MD1,2,3
1. Hugh Williamson Gait Analysis Laboratory, The Royal Children’s Hospital, Melbourne, Australia
2. Murdoch Childrens Research Institute, Melbourne, Australia
3. The University of Melbourne, Melbourne, Australia
Children with Hereditary Spastic Paraparesis (HSP) present with a similar range of gait
disorders and clinical phenotypes to children with Cerebral Palsy (CP)1,2,3. However, there
is more uncertainty about the natural history of gait and function in HSP which translates
to uncertainty about appropriate management, especially invasive surgical procedures.
We report the gait and function of ten children with HSP, who had gait corrective surgery,
with short and medium term follow-up based on Instrumented Gait Analysis (IGA).
Conclusion
25
Figure 1: Median GPS at short term and
medium term IGA
For the majority of this cohort, gait was maintained or
improved following surgery and standard rehabilitation.
Individually, some children with patterns such as crouch
gait showed very significant improvements in gait and
functioning which were maintained at T3. Others with
milder gait impairments showed less evidence of change.
It would seem appropriate therefore to refer such patients
for IGA and consider targeted surgical intervention followed
by intensive rehabilitation using protocols based on the
management of children with CP 5.
20
gps
Introduction
15
10
5
Pre op
Retrospective cohort study.
Method
Data were extracted for children with a
diagnosis of HSP who had IGA for surgical
decision making from a clinical database
pre and post surgery:
• Kinematic and kinetic traces
• Gait Profile Score (GPS)
• Gross Motor Function Classification
System (GMFCS)
• Functional Mobility Scale (FMS)
Results
• Ten children had a baseline IGA
within 12 months of surgery (T1) and
at 12-24 months post operatively (T2)
Table 1: Number of children functioning at each
GMFCS level
GMFCS level
n
I
2
II
5
III
3
7/10 children showed a clinically significant
improvement in GPS (Figure 1) at T2 and/or
T3 in comparison to baseline, based on a
minimal clinically important difference (MCID)
of 1.6° 4. Figure 2 shows the change in gait
parameters (gait variable scores) at each
joint over time.
Encouragingly, only 1/10 children showed a
deterioration in GPS in excess of the MCID.
Given the heterogeneous nature of the cohort
and the wide standard deviation at baseline,
these values did not reach significance.
Medium term
(>48m) post op
time
T1
Figure 2: Movement analysis profile
at T1, T2, T3
T2
T3
No Pathology
30
References
20
1.Cimolin, V., Piccinini, L., D’Angelo, M. G., Turconi, A. C., Berti, M., Crivellini,
M., et al. (2007). Are patients with hereditary spastic paraplegia different
from patients with spastic diplegia during walking? Gait evaluation using 3D
gait analysis. [Comparative Study]. Functional Neurology, 22(1), 23-28.
10
0
Pel
tilt
Knee
flex
Ank
dors
Pel
obl
Hip
abd
11
Figure 3: Changes in FMS over 5 years
FMS data was collected for 8 children
Hip
flex
Declined
9
7
Hip
rot
Foot
prog
Maintained
GPS
Improved
3.Wolf, S. I., Braatz, F., Metaxiotis, D., Armbrust, P., Dreher, T., Doderlein, L.,
et al. (2011). Gait analysis may help to distinguish hereditary spastic
paraplegia from cerebral palsy. Gait & Posture, 33(4), 556-561.
4.Baker, R., McGinley, J. L., Schwartz, M., Thomason, P., Rodda, J., & Graham,
H. K. (2012). The minimal clinically important difference for the Gait Profile
Score. [Comparative Study]. Gait & posture, 35(4), 612-615.
5
3
1
–1
Pel
rot
2.Piccinini, L., Cimolin, V., D’Angelo, M. G., Turconi, A. C., Crivellini, M., & Galli,
M. (2011). 3D gait analysis in patients with hereditary spastic paraparesis
and spastic diplegia: a kinematic, kinetic and EMG comparison. [Comparative
Study]. European Journal of Paediatric Neurology, 15(2), 138-145.
5m
50m
5 years
500m
5.Thomason, P., Selber, P., & Graham, H. K. (2013). Single Event Multilevel
Surgery in children with bilateral spastic cerebral palsy: A 5 year prospective
cohort study. Gait & Posture, 37(1), 23-28.
ERC 140049 January 2014
• Eight children had an additional IGA
at 4–6 years post operatively (T3)
At T1, mean (SD) age was 10 years 6 months
(2 years 7 months). Children were functioning
at GMFCS levels I–III (Table 1).
frequency
Design
Short term
(12-24m) post op