Changes in the range of motion of the lower limb joints during extensive tibial lengthening in achondroplasia

Abstract

Increase in the magnitude of lengthening during the limb lengthening procedure involves a high risk of decreased range of motion (ROM) in adjacent joints. Even though patients with achondroplasia can tolerate a relatively larger amount of lengthening owing to its inherent soft-tissue laxity, they often exhibit significant joint contractures during extensive lengthening. In the present study, we evaluated temporal changes in the ROM of the hip, knee, and ankle joints throughout the treatment period in 12 limbs of six patients with achondroplasia who had undergone extensive tibial lengthening. The ROM of hip extension, knee extension, and ankle dorsiflexion were measured before distraction, at every 1-cm length gained during distraction, and at monthly intervals after the termination of distraction until the frame removal. The average amount of lengthening was 9.2±1.2 cm, corresponding to 52.8±6.8% of the original bone length. Equinus deformity of the ankle was observed in the early phase of distraction, whereas flexion contracture of the knee and hip appeared in the middle and the late phase of distraction, respectively. With dedicated physiotherapy and deliberate orthosis wearing, all of the contracture gradually resolved up to the preoperative state after the termination of distraction. This is the first report showing the development of contracture in the hip, a nonadjacent joint for the tibial lengthening.

Two-stage Bone Lengthening With Reuse of a Single Intramedullary Telescopic Nail in Patients With Achondroplasia

“…Although patients with achondroplasia can tolerate a relatively larger degree of lengthening owing to their inherent soft tissue laxity, existing research suggests that they may develop significant joint contractures during extensive lengthening with EF,20 whereas our research shows a lower rate of joint stiffness, likely due to the absence of muscle transfixion from pins used with EF and the ease with which patients perform stretching and strengthening exercises to maintain joint movement after TIMN. Fracture of the regenerate has been frequently described with the use of EF 29.…”

“…Limb lengthening is a common procedure in achondroplastic patients, aiming to provide them with a more functional stature and improve their self-esteem and HRQoL 10,11. Table E-1 (Electronic Appendix Table E-1, Supplemental Digital Content 3, http://links.lww.com/BPO/A468)15–23 summarizes the data from the published series on surgical limb lengthening in achondroplastic patients in the last 10 years, while the metanalysis published by Schiedel and Rödl24 includes another set of similar studies before 2010. Together, this body of research shows that all the lengthening procedures to date have been accomplished with EF, apart from one case report that uses a telescopic nail 12.…”

Limb lengthening history, evolution, complications and current concepts

“…The incidence of complications is affected by the aetiology of the shortening and magnitude of the regenerated area. Extensive bone lengthening can adversely affect growth in children and increase the possibility of joint contractures [46,47].…”

Efficacy and safety of botulinum toxin type A in distraction osteogenesis of the lower extremities: a meta-analysis of randomized controlled trials

“…Although current evidence do not support the use of BoNT in DO in the aspect of ROM, we believe that BoNT might still have the potential to play a role in joint contracture after DO of the lower limbs. Previous researches had reported that patients often experienced joint stiffness during the distraction phase, which gradually resolved after the individuals entered the consolidation phase with the help of intensive physiotherapy and splinting throughout the entire process [ 6 , 7 , 10 ]. However, articles have found that patients with excessive lengthening of the tibia and femur were more prone to sustained joint stiffness despite splinting and physical therapy, which requires further surgical correction [ 8 , 9 ].…”

“…One frequent complication is contracture of muscles and adjacent soft tissues, which may result from tension generated during lengthening and may also lead to joint luxation, axial deviation, and joint stiffness [ 3 ]. Previous studies have commonly observed loss of motion in the joints after the procedure of DO [ 6 ] although most patients of contracture gradually resolve through vigorous physiotherapy and orthosis use [ 7 ]. However, some cases require surgical intervention to improve contracture and other comorbidities, especially those with a larger amount of lengthening [ 8 , 9 ].…”