INTRODUCTION: Surgical lengthening and angular correction of the limbs are an option for treating the orthopedic clinical manifestations in patients with achondroplasia. This study assesses a staged limb lengthening protocol, performing simultaneous bilateral lengthening of the femur and tibia (stage I [S1]), and humeral lengthening (stage II [S2]). MATERIALS AND METHODS: Twenty-one achondroplastic patients were included in this study, and 106 segments (34 femurs, 34 tibias and 38 humeri) were lengthened. Achondroplasia patients with a growth curve below the mean of the standard growth curves for achondroplasia were included in S1. The remaining patients were included directly in S2. Variables analyzed included anthropometric measurements, lengthening outcomes, difficulties, and functionality. RESULTS: Of the all patients included in the protocol, 15 patients completed S1 and S2, 4 only completed S2, and 2 only completed S1. Height and limb-trunk ratio before S1 were 107.65 ± 7.14 cm and 1.89 ± 0.10 and after S1 were 126.50 ± 9.19 cm and 1.64 ± 0.09, respectively. Limbs were lengthened 14.43 ± 1.41 cm (femurs and tibias) for S1 and 9.95 ± 0.60 cm for S2 (humeri), with a stage healing index of 18.23 ± 3.54 in S1 and 28.92 ± 4.42 in S2. Correction of lower angular deviations, functional improvement, and a controlled complications rate were achieved in all patients. CONCLUSIONS: The limb lengthening protocol proposed in this study is a suitable treatment for achondroplasia patients to achieve the agreed-upon objectives (limb-trunk ratio, improved functionality, and lower limb alignment). The reproducibility of the procedure and patient safety were upheld.
INTRODUCTION: Surgical lengthening and angular correction of the limbs are an option for treating the orthopedic clinical manifestations in patients with achondroplasia. This study assesses a staged limb lengthening protocol, performing simultaneous bilateral lengthening of the femur and tibia (stage I [S1]), and humeral lengthening (stage II [S2]). MATERIALS AND METHODS: Twenty-one achondroplasticpatients were included in this study, and 106 segments (34 femurs, 34 tibias and 38 humeri) were lengthened. Achondroplasiapatients with a growth curve below the mean of the standard growth curves for achondroplasia were included in S1. The remaining patients were included directly in S2. Variables analyzed included anthropometric measurements, lengthening outcomes, difficulties, and functionality. RESULTS: Of the all patients included in the protocol, 15 patients completed S1 and S2, 4 only completed S2, and 2 only completed S1. Height and limb-trunk ratio before S1 were 107.65 ± 7.14 cm and 1.89 ± 0.10 and after S1 were 126.50 ± 9.19 cm and 1.64 ± 0.09, respectively. Limbs were lengthened 14.43 ± 1.41 cm (femurs and tibias) for S1 and 9.95 ± 0.60 cm for S2 (humeri), with a stage healing index of 18.23 ± 3.54 in S1 and 28.92 ± 4.42 in S2. Correction of lower angular deviations, functional improvement, and a controlled complications rate were achieved in all patients. CONCLUSIONS: The limb lengthening protocol proposed in this study is a suitable treatment for achondroplasiapatients to achieve the agreed-upon objectives (limb-trunk ratio, improved functionality, and lower limb alignment). The reproducibility of the procedure and patient safety were upheld.
Authors: Ravi Savarirayan; Penny Ireland; Melita Irving; Dominic Thompson; Inês Alves; Wagner A R Baratela; James Betts; Michael B Bober; Silvio Boero; Jenna Briddell; Jeffrey Campbell; Philippe M Campeau; Patricia Carl-Innig; Moira S Cheung; Martyn Cobourne; Valérie Cormier-Daire; Muriel Deladure-Molla; Mariana Del Pino; Heather Elphick; Virginia Fano; Brigitte Fauroux; Jonathan Gibbins; Mari L Groves; Lars Hagenäs; Therese Hannon; Julie Hoover-Fong; Morrys Kaisermann; Antonio Leiva-Gea; Juan Llerena; William Mackenzie; Kenneth Martin; Fabio Mazzoleni; Sharon McDonnell; Maria Costanza Meazzini; Josef Milerad; Klaus Mohnike; Geert R Mortier; Amaka Offiah; Keiichi Ozono; John A Phillips; Steven Powell; Yosha Prasad; Cathleen Raggio; Pablo Rosselli; Judith Rossiter; Angelo Selicorni; Marco Sessa; Mary Theroux; Matthew Thomas; Laura Trespedi; David Tunkel; Colin Wallis; Michael Wright; Natsuo Yasui; Svein Otto Fredwall Journal: Nat Rev Endocrinol Date: 2021-11-26 Impact factor: 47.564
Authors: Renée Shediac; Olga Moshkovich; Heather Gerould; Rachel Ballinger; Agnes Williams; M Alex Bellenger; Jennifer Quinn; Julie Hoover-Fong; Klaus Mohnike; Ravi Savarirayan; Dominique Kelly Journal: Mol Genet Genomic Med Date: 2022-02-09 Impact factor: 2.183
Authors: Pamela K Foreman; Femke van Kessel; Rosa van Hoorn; Judith van den Bosch; Renée Shediac; Sarah Landis Journal: Am J Med Genet A Date: 2020-08-17 Impact factor: 2.802