SP 32-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

25 Introduction Figure 1: Median GPS at short term and Conclusion medium term IGA Children with Hereditary Spastic Paraparesis (HSP) present with a similar range of gait 20 For the majority of this cohort, gait was maintained or disorders and clinical phenotypes to children with Cerebral Palsy (CP)1,2,3. However, there improved following surgery and standard rehabilitation. is more uncertainty about the natural history of gait and function in HSP which translates Individually, some children with patterns such as crouch 15 to uncertainty about appropriate management, especially invasive surgical procedures. gps gait showed very significant improvements in gait and We report the gait and function of ten children with HSP, who had gait corrective surgery, functioning which were maintained at T3. Others with 10 with short and medium term follow-up based on Instrumented Gait Analysis (IGA). milder gait impairments showed less evidence of change. It would seem appropriate therefore to refer such patients 5 Pre op Short term Medium term for IGA and consider targeted surgical intervention followed (12-24m) post op (>48m) post op At T1, mean (SD) age was 10 years 6 months Design time by intensive rehabilitation using protocols based on the (2 years 7 months). Children were functioning 5 Retrospective cohort study. management of children with CP . at GMFCS levels I–III (Table 1). T1 T2 T3 No Pathology Figure 2: Movement analysis profile 30 Method Table 1: Number of children functioning at each at T1, T2, T3 GMFCS level Data were extracted for children with a

diagnosis of HSP who had IGA for surgical GMFCS level n 20 References decision making from a clinical database I 2 1. Cimolin, V., Piccinini, L., D’Angelo, M. G., Turconi, A. C., Berti, M., Crivellini, pre and post surgery: M., et al. (2007). Are patients with hereditary spastic paraplegia different II 5 from patients with spastic diplegia during walking? Gait evaluation using 3D • Kinematic and kinetic traces 10 III 3 gait analysis. [Comparative Study]. Functional Neurology, 22(1), 23-28. • Gait Profile Score (GPS) 2. Piccinini, L., Cimolin, V., D’Angelo, M. G., Turconi, A. C., Crivellini, M., & Galli, • Gross Motor Function Classification 7/10 children showed a clinically significant M. (2011). 3D gait analysis in patients with hereditary spastic paraparesis System (GMFCS) improvement in GPS (Figure 1) at T2 and/or 0 and spastic diplegia: a kinematic, kinetic and EMG comparison. [Comparative Pel Hip Knee Ank Pel Hip Pel Hip Foot GPS Study]. European Journal of Paediatric Neurology, 15(2), 138-145. • Functional Mobility Scale (FMS) T3 in comparison to baseline, based on a tilt flex flex dors obl abd rot rot prog minimal clinically important difference (MCID) 3. Wolf, S. I., Braatz, F., Metaxiotis, D., Armbrust, P., Dreher, T., Doderlein, L., 11 of 1.6° 4. Figure 2 shows the change in gait Declined Maintained Improved et al. (2011). Gait analysis may help to distinguish hereditary spastic Results Figure 3: Changes in FMS over 5 years parameters (gait variable scores) at each 9 paraplegia from cerebral palsy. Gait & Posture, 33(4), 556-561. • Ten children had a baseline IGA joint over time. FMS data was collected for 8 children 7 4. Baker, R., McGinley, J. L., Schwartz, M., Thomason, P., Rodda, J., & Graham, within 12 months of surgery (T1) and 5 H. K. (2012). The minimal clinically important difference for the Gait Profile at 12-24 months post operatively (T2) Encouragingly, only 1/10 children showed a 3 Score. [Comparative Study]. Gait & posture, 35(4), 612-615. deterioration in GPS in excess of the MCID. frequency • Eight children had an additional IGA 1 Given the heterogeneous nature of the cohort 5. Thomason, P., Selber, P., & Graham, H. K. (2013). Single Event Multilevel at 4–6 years post operatively (T3) –1 5m 50m 500m Surgery in children with bilateral spastic cerebral palsy: A 5 year prospective 2014 January 140049 ERC and the wide standard deviation at baseline, 5 years these values did not reach significance. cohort study. Gait & Posture, 37(1), 23-28.