Foot deformities are frequently observed in patients with Charcot Marie tooth disease (CMT) and orthopaedic surgery is often required. Currently there is no evidence-based guideline on surgical management and only a few studies which have evaluated long-term outcomes of surgical procedures. The aim of the study was to evaluate longitudinally the effect of foot surgery in a cohort of CMT patients.Twenty-five CMT adult patients were assessed using a comprehensive group of validated scales and questionnaires before and after surgery. A wide range of surgical procedures was performed by one team of dedicated foot ankle orthopaedic surgeons. Foot alignment as measured by the foot posture index, pain, quality of life and callosities significantly improved after one year and the improvement was maintained up to 4 years after surgery. There was a trend towards a reduction in the number of falls post-operatively. Surgery had no effect on fatigue, balance and CMT examination score. Our findings showed significant improvement of pain, foot alignment, callosities and quality of life after surgery and suggested that foot deformity correction in adults with CMT performed in a specialised foot and ankle unit is beneficial.
Aims To assess the characteristic clinical features, management, and outcome of patients who present to orthopaedic surgeons with functional dystonia affecting the foot and ankle. Methods We carried out a retrospective search of our records from 2000 to 2019 of patients seen in our adult tertiary referral foot and ankle unit with a diagnosis of functional dystonia. Results A total of 29 patients were seen. A majority were female (n = 25) and the mean age of onset of symptoms was 35.3 years (13 to 71). The mean delay between onset and diagnosis was 7.1 years (0.5 to 25.0). Onset was acute in 25 patients and insidious in four. Of the 29 patients, 26 had a fixed dystonia and three had a spasmodic dystonia. Pain was a major symptom in all patients, with a coexisting diagnosis of chronic regional pain syndrome (CRPS) made in nine patients. Of 20 patients treated with Botox, only one had a good response. None of the 12 patients who underwent a surgical intervention at our unit or elsewhere reported a subjective overall improvement. After a mean follow-up of 3.2 years (1 to 12), four patients had improved, 17 had remained the same, and eight reported a deterioration in their condition. Conclusion Patients with functional dystonia typically presented with a rapid onset of fixed deformity after a minor injury/event and pain out of proportion to the deformity. Referral to a neurologist to rule out neurological pathology is advocated, and further management should be carried out in a movement disorder clinic. Response to treatment (including Botulinum toxin (Botox) injections) is generally poor. Surgery in this group of patients is not recommended and may worsen the condition. The overall prognosis remains poor. Cite this article: Bone Joint J 2021;103-B(6):1127–1132.
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