Quality of Life in Children With Achondroplasia Undergoing Paired Limb Lengthening With an External Fixator and Modified Distraction Control: Observational Nonrandomized Study

Abstract

BackgroundTransosseous distraction osteosynthesis is prioritized in orthopedic care for children with achondroplasia. However, difficulties encountered during treatment and rehabilitation directly impact patients’ quality of life. Using rod external fixators within a semicircular frame for osteosynthesis is less traumatic compared to spoke circular devices. Their straightforward assembly and mounting on the limb segment can help significantly reduce treatment duration, thereby improving children’s quality of life during treatment and rehabilitation. ObjectiveThis study aimed to conduct a comparative analysis of the quality of life (measured by postoperative pain syndrome, physical activity, and emotional state) among children with achondroplasia undergoing paired limb lengthening using either an external fixator with modified distraction control or a circular multiaxial system developed by the authors. MethodsThis was an observational, prospective, nonrandomized, and longitudinal study with historical control. The study group consisted of 14 patients ranging from 5 to 15 (mean 7.6, SD 2.3) years old with a genetically confirmed diagnosis of achondroplasia. All patients underwent paired limb lengthening with a rod external fixator and a modified distraction control developed by the authors. A total of 28 limb segments, among them 4 (14%) humeri, 8 (29%) femurs, and 16 (57%) tibias, were lengthened in 1 round. Unpublished data from the previous study served as the control group, comprising 9 patients (18 limb segments) of the same age group (mean age at surgery 8.6, SD 2.3 years), who underwent limb lengthening surgery using a circular multiaxial system—2 (11%) humeri, 6 (33%) femurs, and 10 (56%) tibias. The Wong-Baker Faces Rating Scale was used to measure pain symptoms, while the Russified Pediatric Quality of Life (PedsQL) v4.0 questionnaire assessed quality of life. ResultsDuring the latent phase (7 to 10 days after surgery), a more pronounced decrease in the indicators of physical activity and emotional state on the PedsQL v4.0 questionnaire was noted in the control group (mean 52.4, SD 4.8 versus mean 52.8, SD 5.5 points according to children’s responses and their parents’ responses, respectively) compared to the experimental group (mean 59.5, SD 6.8 points and mean 61.33, SD 6.5 points according to the children’s responses and their parents’ responses, respectively). The differences between the groups were statistically significant (P<.05 for children's responses and P<.01 for parents’ responses). Importantly, 6 months after surgery, these quality-of-life indicators, as reported by children in the experimental group, averaged 70.25 (SS 4.8) points. Similarly, their parents reported a mean of 70.54 (SD 4.2) points. In the control group, the corresponding values were 69.64 (SD 5.6) and 69.35 (SD 6.2), respectively. There was no statistically significant difference between the groups. ConclusionsThe external fixator with modified distraction control developed by the authors provides a higher standard of living compared with the circular multiaxial system during the latency phase.