Introduction: Foot deformities are frequent complications in Charcot–Marie–Tooth disease (CMT) patients, often requiring orthopedic surgery. However, there are no prospective, randomized studies on surgical management, and there is variation in the approaches among centers both within and between countries. Methods: In this study we assessed the frequency of foot deformities and surgery among patients recruited into the Inherited Neuropathies Consortium (INC). We also designed a survey addressed to orthopedic surgeons at INC centers to determine whether surgical approaches to orthopedic complications in CMT are variable. Results: Foot deformities were reported in 71% of CMT patients; 30% of the patients had surgery. Survey questions were answered by 16 surgeons working in different specialized centers. Most of the respondents were foot and ankle surgeons. There was marked variation in surgical management. Discussion: Our findings confirm that the approaches to orthopedic management of CMT are varied. We identify areas that require further research. Muscle Nerve
57: 255–259, 2018
Total hip arthroplasty revision is increasingly becoming a common procedure, but the acetabular bone loss and the advanced age of the patients make revision procedures extremely complex and technically demanding. The aim of the present work is to examine the clinical and radiological results of the Burch-Schneider anti-protrusio cage (APC) implanted in revision hip arthroplasty with severe acetabular bone deficiency in elderly people. Between February 1994 and November 2005, a total of 60 revision operations of acetabular components were performed in 60 select patients (42 females and 18 males), using the Burch-Schneider APC. The indication for the Burch-Schneider APC use was massive pelvic bone loss with migration of the prosthesis cup and high-grade acetabular defects (type III or IV AAOS). The mean age at the time of surgery was 82 years (range 78-85 years). Polymethylmetacrylate cement (PMMA) was used to fill bony deficiencies in all the procedures. No graft was used. The average Harris Hip Score had improved from 28.2 preoperatively to 82.5 points at the time of follow-up. According to the classification of Gill et al., we have seen no Burch-Schneider cages definitely loose or probably. Only one acetabular component has been revised (for recurrent dislocation). Acetabular reconstruction with the use of cement and an acetabular support ring appears to have a useful role in the treatment of severe acetabular bony deficiency in elderly patients and may provide a definitive reconstruction.
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.
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