Limb Lengthening for Achondroplasia: Systematic Review and Meta-analysis

Abstract

Background: Achondroplasia is the most common genetic skeletal dysplasia. It is characterized by a rhizomelic form of dwarfism, exaggerated lumbar lordosis, a prominent forehead and a low nasal bridge. The trunk is generally of near normal length. The incidence of achondroplasia is approximately 1 in 25000 people. Objectives: In the present systematic review, we have aimed to synthesize evidence from the published literature about the safety and efficacy of limb lengthening in patients with achondroplasia. Also, we have aimed to assess which age is preferred to start the lengthening and effect of early management on natural growth and psychiatric health. Methods: An electronic search was conducted from the inception till September 2018 in Medline via PubMed. Data entry and processing were carried out using a standardized Excel sheet and reviewers extracted the data from the included studies. We performed all statistical analyses using Review Manager (RevMan) 5.3 for windows. Results: In the present study, 17 studies were included in the present systematic review and meta-analysis. In terms of gained length of femur, the overall effect estimate showed that limb lengthening procedure achieved a mean increase in femur length by 11.9 (95% CI 9.39 – 14.43) cm. In addition, the overall effect estimate showed that limb lengthening procedure achieved a healing index of femur 30.46 (95% CI 24.326 – 36.59) days/cm. Similarly, the overall effect estimate showed that limb lengthening procedure achieved a mean increase in tibia length by 9.5 (95% CI 8.169 – 10.927) cm. The overall effect estimate showed that limb lengthening procedure achieved a healing index of tibia of 32.09 (95% CI 18.65 – 25.52) days/cm Conclusion: It could be concluded that limb lengthening is a reliable procedure with a relatively low complication rate in increasing the length of femur and tibia in patients with achondroplasia.