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Staged upper and lower limb lengthening performing bilateral simultaneous surgery of the femur and tibia in achondroplastic patients

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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.


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.


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.

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  1. 1.

    Pauli RM (2019) Achondroplasia: a comprehensive clinical review. Orphanet J Rare Dis. https://doi.org/10.1186/s13023-018-0972-6

  2. 2.

    Merker A, Neumeyer L, Hertel NT, Grigelioniene G, Mohnike K, Hagenäs L (2018) Development of body proportions in achondroplasia: sitting height, leg length, arm span, and foot length. Am J Med Genet A 176:1819–1929

  3. 3.

    Horton WA, Rotter JI, Rimoin DL, Scott CI, Hall JG (1978) Standard growth curves for achondroplasia. J Pediatr 93:435–438

  4. 4.

    Hoover-Fong J, McGready J, Schulze K, Alade AY, Scott C (2017) A height-for-age growth reference for children with achondroplasia: expanded applications and comparison with original reference data. Am J Med Genet 173:1226–1230

  5. 5.

    Merker A, Neumeyer L, Hertel NT, Grigelioniene G, Mäkitie O, Mohnike K, Hagenäs L (2018) Growth in achondroplasia: development of height, weight, head circumference, and body mass index in a European cohort. Am J Med Genet A 176:1723–1734

  6. 6.

    Shelmerdine SC, Brittain H, Arthurs OJ, Calder AD (2016) Achondroplasia: really rhizomelic? Am J Med Genet A 170:2039–2043

  7. 7.

    Unger S, Bonafé L, Gouze E (2017) Current care and investigational therapies in achondroplasia. Curr Osteoporos Rep 15:53–60

  8. 8.

    Kim SJ, Pierce W, Sabharwal S (2014) The etiology of short stature affects the clinical outcome of lower limb lengthening using external fixation. A systematic review of 18 trials involving 547 patients. Acta Orthop 85:181–186

  9. 9.

    Vaidya SV, Song HR, Lee SH, Suh SW, Keny SM, Telang SS (2006) Bifocal tibial corrective osteotomy with lengthening in achondroplasia: an analysis of results and complications. J Pediatr Orthop 26:788–793

  10. 10.

    Ko KR, Shim JS, Chung CH, Kim JH (2019) Surgical results of limb lengthening at the femur, tibia, and humerus in patients with achondroplasia. CiOS Clin Orthop Surg 11(2):226–232. https://doi.org/10.4055/cios.2019.11.2.226

  11. 11.

    Donaldson J, Aftab S, Bradish C (2015) Achondroplasia and limb lengthening: results in a UK cohort and review of the literature. J Orthop 12(1):31–34. https://doi.org/10.1016/j.jor.2015.01.001

  12. 12.

    Burghardt RD, Yoshino K, Kashiwagi N, Yoshino S, Bhave A, Paley D, Herzenberg JE (2015) Bilateral double level tibial lengthening in dwarfism. J Orthop 12:242–247

  13. 13.

    Schiedel F, Rödl R (2012) Lower limb lengthening in patients with disproportionate short stature with achondroplasia: a systematic review of the last 20 years. Disabil Rehabil 34:982–987

  14. 14.

    Kocaoglu M, Bilen FE, Dikmen G, Balchi HI, Eralp L (2014) Simultaneous bilateral lengthening of femora and tibiae in achondroplastic patients. Act Orthop Traumatol Turc 48:157–163

  15. 15.

    Ilizarov GA (1990) Clinical application of the tension-stress effect for limb lengthening. Clin Orthop Relat Res 250:8–26

  16. 16.

    Prader A, Largo RH, Molinari L, Issler C (1989) Physical growth of Swiss children from birth to 20 years of age. First Zurich longitudinal study of growth and development. Helv Paediatr Acta Suppl 52:1–125

  17. 17.

    Paley D, Herzenberg JE, Tetsworth K, McKie J, Bhave A (1994) Deformity planning for frontal and sagital plane corrective osteotomies. Orthop Clin North Am 25:425–465

  18. 18.

    Paley D (1990) Problems, obstacles, and complications of limb lengthening by the Ilizarov technique. Clin Orthop Relat Res 250:81–104

  19. 19.

    Shadi M, Musielak B, Koczewski P, Janusz P (2018) Humeral lengthening in patients with achondroplasia and in patients with post-septic shortening: comparison of procedure efficiency and safety. Int Orthop 42:419–426

  20. 20.

    Ginebreda I, Campillo-Recio D, Cárdenas C, Tapiolas J, Rovira P, Isart A (2018) Surgical technique and outcomes for bilateral humeral lengthening for achondroplasia: 26-year experience. Musculoskelet Surg. https://doi.org/10.1007/s12306-018-0583-3

  21. 21.

    Matsushita M, Kitoh H, Mishima K, Yamashita S, Haga N, Fujiwara S, Ozono K, Kubota T, Kitaoka T, Ishiguro N (2019) Physical, mental, and social problems of adolescent and adult patients with achondroplasia. Calcif Tissue Int 104:364–372

  22. 22.

    Ireland PJ, Ware RS, Donaghey S, McGill J, Zankl Pacey V, Ault J, Savarirayan R, Sillence D, Thompson E, Townshend S, Johnston LM (2013) The effect of height, weight and head circumference on gross motor development in achondroplasia. J Paediatr Child Health 49:e122–e127

  23. 23.

    Song SH, Kim SE, Agashe MV, Lee H, Refai MA, Park YE et al (2012) Growth disturbance after lengthening of the lower limb and quantitative assessment of physeal closure in skeletally immature patients with achondroplasia. J Bone Joint Surg Ser B 94(B4):556–563. https://doi.org/10.1302/0301-620X.94B4.28375

  24. 24.

    Venkatesh KP, Modi HN, Devmurari K, Yoon JY, Anupama BR, Song HR (2009) Femoral lengthening in achondroplasia: magnitude of lengthening in relation to patterns of callus, stiffness of adjacent joints and fracture. Journal of Bone and Joint Surgery Series B 91(12):1612–1617. https://doi.org/10.1302/0301-620X.91B12.22418

  25. 25.

    Horn J, Hvid I, Huhnstock S, Breen AB, Steen H (2019) Limb lengthening and deformity correction with externally controlled motorized intramedullary nails: evaluation of 50 consecutive lengthenings. Acta Orthop 90(1):81–87. https://doi.org/10.1080/17453674.2018.1534321

  26. 26.

    Bloemeke J, Sommer R, Witt S, Bullinger M, Nordon C, Badia FJ et al (2019) Cross-cultural selection and validation of instruments to assess patient-reported outcomes in children and adolescents with achondroplasia. Qual Life Res. https://doi.org/10.1007/s11136-019-02210-z

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We thank the ALPE Achondroplasia Foundation (Gijón, Spain), especially to Carmen Alonso and Susana Noval, for its valuable collaboration on patient coordination and support activities throughout the standardized protocol. We thank Belén Pimentel from the AME Unit at IBIMA_FIMABIS for critical reading of the manuscript. We thank Biostatech Advice Training & Innovation in Biostatistics, S.L for statistical review.

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Correspondence to Antonio Leiva-Gea.

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No author has commercial associations that might pose a conflict of interest in connection with the submitted article. This was an independent study. The contracts of the clinicians and the public resources used in the study were funded by the Andalusian Public Health Service, Regional Ministry of Health in Andalucía, Spain, and by the corresponding Regional Ministry of Health for each patient from outside of Andalusia, supported by the National Public Health Service of Spain.

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Leiva-Gea, A., Delgado-Rufino, F.B., Queipo-de-Llano, A. et al. Staged upper and lower limb lengthening performing bilateral simultaneous surgery of the femur and tibia in achondroplastic patients. Arch Orthop Trauma Surg (2020). https://doi.org/10.1007/s00402-020-03360-3

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  • Achondroplasia
  • Lower limb lengthening
  • Humeral lengthening
  • External fixator