Surgical outcomes of cavovarus foot deformity in children with Charcot-Marie-Tooth disease

Neuromuscul Disord. 2019 Jun;29(6):427-436. doi: 10.1016/j.nmd.2019.04.004. Epub 2019 Apr 26.

Abstract

Charcot-Marie-Tooth disease (CMT) causes disabling cavovarus foot deformity. Orthopaedic surgery is performed in severe cases; however few studies have investigated whether surgery improves health outcomes during childhood. This study investigated the impact of cavovarus surgery on validated physical, functional, parent/self-reported and biomechanical measures in 21 consecutive patients (mean age at surgery 12.5 years, SD 2.7) evaluated before and after surgery (mean duration 15.7 months, SD 5.9), and compared to natural history data from 206 children with CMT. Measures from the CMT Pediatric Scale evaluated foot alignment (Foot Posture Index), ankle flexibility (lunge test), strength (foot dorsiflexion/plantarflexion by hand-held dynamometry), function (balance, long jump, 6-minute walk test) and self-reported symptoms. Quality of life (Child Health Questionnaire) and gait (pressure loading) were also assessed. Foot Posture Index and lunge improved with surgery by 6.0 points (SD 3.2) and 6.1° (SD 7.3) respectively (p< 0.01), and differed to the natural course of the disease (p< 0.005). Self-reported daily trips/falls reduced from 60% to 13% (p = 0.016). Pressure improved beneath the rearfoot and midfoot (p = 0.043). Surgery had no effect on strength, function or quality of life, which generally mirrored the natural course. Cavovarus surgery improved foot alignment, ankle flexibility and self-reported trips/falls in children with CMT.

Keywords: Charcot-Marie-Tooth disease; Child health; Clinical outcome assessment; Foot deformities; Orthopaedic procedures.

MeSH terms

  • Adolescent
  • Charcot-Marie-Tooth Disease / complications*
  • Child
  • Female
  • Humans
  • Male
  • Quality of Life
  • Talipes Cavus / complications
  • Talipes Cavus / surgery*
  • Treatment Outcome