Long‐term walking ability and patient satisfaction after lower limb functional surgery in patients affected by Charcot‐Marie‐Tooth disease: A retrospective study

Abstract

Structural foot deformities consequent to Charcot Marie Tooth (CMT) can be treated by functional surgery (FS). This study aims to evaluate both long‐term walking ability and patients' satisfaction in CMT subjects who underwent FS during their lifetime. We conducted a retrospective observational study. Age, sex, CMT type, comprehensive surgical history, current walking ability assessed by the Walking Handicap Scale (WHS) and patients' global impression of change (pGIC) were retrieved from a custom database managed at our institution. WHS and pGIC were assessed between mid‐2018 and mid‐2019. Data from 79 patients were screened and 63 were included, 35W‐28M, mean age 42 (15), with demyelinating (75%), axonal (20%), and other types (5%) of CMT, who underwent FS between 1967 and 2018. FS evolved significantly over the years from bone‐related procedures (e.g., arthrodesis) to both bone and soft tissues‐related procedures. The re‐intervention rate decreased from 70% before 2000 to 32% in the last decade. Complications arose in five cases. FS was mainly performed on adults (73%). WHS was ≥ 5 in three‐quarters of the sample (range 1‐6) and was significantly affected by age groups in patients with demyelinating CMT (n=47, p<0.01, non‐parametric ANOVA). Nearly 80% of patients were satisfied with FS (pGIC ≥ 4). In conclusion, CMT subjects who underwent FS surgery maintained a high gait efficiency in the long‐term period, with middle to high levels of satisfaction in the majority of the cases. This confirms the validity of FS in the management of acquired foot deformities in CMT patients.

A prospective study on surgical management of foot deformities in Charcot Marie tooth disease

“…The majority of patients were male (18/25, 72%) and had CMT1A due to chromosome 17 duplication (17/25, 68%). The mean CMTES v2 was 11 (SD 4.5) (range [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19].…”

“…Direct comparison with previous studies is difficult since all the studies so far performed have been retrospective and only few studies have looked at the long‐term outcome of foot and ankle surgery in CMT. Previous studies have used patients' satisfaction or self‐reported questionnaires such as the FFI, but not validated physical outcomes 6‐9 . Only one prospective study has been recently performed and this was on a cohort of 21 children undergoing a combination of surgical procedures 5 using validated physical, functional, parent‐self reported and biomechanical outcome measures.…”

“…Previous studies have used patients' satisfaction or selfreported questionnaires such as the FFI, but not validated physical outcomes. [6][7][8][9] Only one prospective study has been recently performed and this was on a cohort of 21 children undergoing a combination of surgical procedures 5 using validated physical, functional, parent-self reported and biomechanical outcome measures. The most significant findings in the paediatric cohort were related to improvement of foot alignment and frequency of trips and falls, similar to our results.…”

“…[6][7][8] More recently a large retrospective study assessing patients' walking ability and patients' satisfaction in CMT patients who had surgery during their life showed higher gait efficiency and high-patient satisfaction in patients who underwent surgery. 9 However evidence regarding optimal surgical management is lacking due to small sample size and the absence of case-control studies. In addition, no prospective studies have been performed before.…”

“…A number of retrospective studies have been performed looking at the outcome of surgical procedures in CMT patients 6‐8 . More recently a large retrospective study assessing patients' walking ability and patients' satisfaction in CMT patients who had surgery during their life showed higher gait efficiency and high‐patient satisfaction in patients who underwent surgery 9 . However evidence regarding optimal surgical management is lacking due to small sample size and the absence of case‐control studies.…”