Triple Arthrodesis in the Treatment of Fixed Cavovarus Deformity in Adolescent Patients with Charcot-Marie-Tooth Disease

Foot & Ankle ◽  
1992 ◽  
Vol 13 (1) ◽  
pp. 1-6 ◽  
Author(s):  
David C. Mann ◽  
John D. Hsu

Ten adolescent patients with Charcot-Marie-Tooth disease (12 feet) were evaluated an average of 7 years, 7 months following triple arthrodesis (TA) for the treatment of cavovarus deformity. The TA was the primary or index bone procedure. At follow-up, five feet were plantigrade, asymptomatic, and radiographically fused at all three joints. Three feet were plantigrade and asymptomatic, but two had talonavicular pseudarthrosis and one had calcaneocuboid pseudarthrosis. Three feet were radiographically fused at all three joints, but in nonplantigrade positions and symptomatic. One foot required a revision TA. In general, TA offers adolescent Charcot-Marie-Tooth patients a stable plantigrade foot in the face of a progressive disorder. The residual deformity, revision, and pseudarthrosis rates are similar to those for adults. Postoperative pain was related to a nonplantigrade foot and not to the presence of pseudarthrosis. It is not known whether a nonplantigrade foot was a result of incomplete surgical correction or recurrent deformity due to progressive muscle imbalance.

2018 ◽  
Vol 57 (5) ◽  
pp. E126-E128
Author(s):  
Brett A. McCray ◽  
William Hurst ◽  
Thomas O. Crawford ◽  
Thomas E. Lloyd

2013 ◽  
Vol 10 (1) ◽  
pp. 65 ◽  
Author(s):  
Maurizio Ferrarin ◽  
Tiziana Lencioni ◽  
Marco Rabuffetti ◽  
Isabella Moroni ◽  
Emanuela Pagliano ◽  
...  

Neurology ◽  
2020 ◽  
Vol 94 (9) ◽  
pp. e884-e896 ◽  
Author(s):  
Vera Fridman ◽  
Stefan Sillau ◽  
Gyula Acsadi ◽  
Chelsea Bacon ◽  
Kimberly Dooley ◽  
...  

ObjectiveTo evaluate the sensitivity of Rasch analysis-based, weighted Charcot-Marie-Tooth Neuropathy and Examination Scores (CMTNS-R and CMTES-R) to clinical progression in patients with Charcot-Marie-Tooth disease type 1A (CMT1A).MethodsPatients with CMT1A from 18 sites of the Inherited Neuropathies Consortium were evaluated between 2009 and 2018. Weighted CMTNS and CMTES modified category responses were developed with Rasch analysis of the standard scores. Change from baseline for CMTNS-R and CMTES-R was estimated with longitudinal regression models.ResultsBaseline CMTNS-R and CMTES-R scores were available for 517 and 1,177 participants, respectively. Mean ± SD age of participants with available CMTES-R scores was 41 ± 18 (range 4–87) years, and 56% were female. Follow-up CMTES-R assessments at 1, 2, and 3 years were available for 377, 321, and 244 patients. A mixed regression model showed significant change in CMTES-R score at years 2 through 6 compared to baseline (mean change from baseline 0.59 points at 2 years, p = 0.0004, n = 321). Compared to the original CMTES, the CMTES-R revealed a 55% improvement in the standardized response mean (mean change/SD change) at 2 years (0.17 vs 0.11). Change in CMTES-R at 2 years was greatest in mildly to moderately affected patients (1.48-point mean change, 95% confidence interval 0.99–1.97, p < 0.0001, for baseline CMTES-R score 0–9).ConclusionThe CMTES-R demonstrates change over time in patients with CMT1A and is more sensitive than the original CMTES. The CMTES-R was most sensitive to change in patients with mild to moderate baseline disease severity and failed to capture progression in patients with severe CMT1A.ClinicalTrials.gov identifierNCT01193075.


Foot & Ankle ◽  
1984 ◽  
Vol 4 (5) ◽  
pp. 267-273 ◽  
Author(s):  
Nathaniel Gould

Crippling equinocavovarus position of the foot in advanced Charcot-Marie-Tooth disease with its attendant metatarsalgia tender calluses and rigidity creates painful, limited, hobbling ambulation with lowered work and general activity tolerance. The object of the surgeries is to convert this foot into a plantigrade, more flexible, painless unit. Surgery consists of plantar fasciotomies off the tubercle of the os caleis and also transversely through the middle of the longitudinal arch. Dorsally angulated green-stick osteotomies are performed at the bases of all the metatarsals. A Jones operation with transfer of the long extensor great toe tendon through the neck and head of the first metatarsal and arthrodesis of the IP joint of the great toe complete the surgery. Often it is necessary to do Bunnell repair lengthening of the flexor hallucis longus tendon. Occasionally, the first metatarsophalangeal joint needs dorsal capsulotomy and lengthening of the short extensor tendon. Variations in and simplicity of techniques are demonstrated with a case report. Ten patients (eight men and two women: 18 feet), ranging in age from 18 to 46 years (average age, 29 years), with a 3- to 6-year follow-up are herein reported. Correction has been maintained in the oldest case.


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