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

BACKGROUND: Transosseous 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. OBJECTIVE: This 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. METHODS: This 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 Unpublished data from the previous study served as the control group, comprising 9 patients (18 limb segments) of the same age group (mean age at surgery 8.6, SD 2.3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. RESULTS: During 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. CONCLUSIONS: The 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.

The role of social, demographic and territorial factors in the late detection of hip dysplasia in children in the Republic of Kazakhstan.

BACKGROUND: The aim of this research is to identify and study the role of social, demographic and territorial factors in the late detection of children with hip dysplasia. METHODS: We conducted a retrospective cohort study of epidemiological data of patients treated in a hospital in the department of orthopedics of the unitary enterprise based on the right of "Multidisciplinary children's municipal hospital No.2" Nur-Sultan (Kazakhstan) in the period from September 2019 to February 2021. The analysis of archival case histories of 309 patients was carried out. There were 214 early and 95 late detections of this disease. Late detection of hip dysplasia was significantly more likely at birth in cranial presentation (81%, p <0.004). RESULTS: Two-dimensional analysis also showed that late detection was more likely in patients from rural areas (228 children, 73.8%, p < 0.001), and that (26 children, 11.4%, p = 0.005) these were children from regions with lower income (42500 tenge per month, p<0.001). There were also significant differences (p = 0.015) in the early (214 children, 69.26%) and late (95 children, 30.7%) diagnosis of hip dysplasia among children whose parents used a national cradle with tight swaddling (95% CI: 1.16 - 4.49). CONCLUSIONS: In our study, we found that children from rural regions of the Republic of Kazakhstan, indigenous Kazakh nationality, using the national cradle in their everyday life, as well as from regions with low average incomes, were significantly more likely to be exposed to late detection of hip dysplasia.

Evaluating the results of long tubular bone distraction with an advanced rod monolateral external fixator for achondroplasia.

The work aimed to evaluate the effectiveness of the developed distraction system based on the rod external monolateral fixation mechanisms by comparing it with the classical technique of long tubular bones distraction based on the circular multi-axial system. The study included patients with a genetically confirmed diagnosis of achondroplasia. The experimental group consisted of 14 patients who underwent surgical limb lengthening by the rod monolateral external fixator with a distraction system developed by the authors. The lengthening was performed on 28 segments of tubular bones. The majority of the experimental group patients achieved the lengthening value close to the planned one and the deformation correction. The fixation period was averagely 83.8 ± 3.7 days, the regenerate length was 8.5 ± 0.6 cm, and the mechanical strength of the distraction regenerate was 10.3° ± 2.18°. The rod external fixator with a control distraction system developed by the authors has small dimensions and low weight of the external supporting elements of high durability. It is reported to provide a good psychological tolerance of the treatment process and significantly outperforms the circular multi-axis system. Considering the aforementioned, the proposed apparatus can grant good orthopedic care to patients with achondroplasia.