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Limb lengthening in achondroplasia

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Indian Journal of Orthopaedics Aims and scope Submit manuscript

Abstract

Background

Stature lengthening in skeletal dysplasia is a contentious issue. Specifc guidelines regarding the age and sequence of surgery, methods and extent of lengthening at each stage are not uniform around the world. Despite the need for multiple surgeries, with their attendant complications, parents demanding stature lengthening are not rare, due to the social bias and psychological effects experienced by these patients. This study describes the outcome and complications of extensive stature lengthening performed at our center.

Materials and Methods

Eight achondroplasic and one hypochondroplasic patient underwent bilateral transverse lengthening for tibiae, humeri and femora. Tibia lengthening was carried out using a ring fxator and bifocal corticotomy, while a monolateral pediatric limb reconstruction system with unifocal corticotomy was used for the femur and humerus. Lengthening of each bone segment, height gain, healing index and complications were assessed. Subgroup analysis was carried out to assess the effect of age and bone segment on the healing index.

Results

Nine patients aged five to 25 years (mean age 10.2 years) underwent limb lengthening procedures for 18 tibiae, 10 femora and 8 humeri. Four patients underwent bilateral lengthening of all three segments. The mean length gain for the tibia, femur and humerus was 15.4 cm (100.7%), 9.9 cm (52.8%) and 9.6 cm (77.9%), respectively. Healing index was 25.7, 25.6 and 20.6 days/cm, respectively, for the tibia, femur and humerus. An average of 33.3% height gain was attained. Lengthening of both tibia and femur added to projected height achieved as the 3rd percentile of standard height in three out of four patients. In all, 33 complications were encountered (0.9 complications per segment). Healing index was not affected by age or bone segment.

Conclusion

Extensive limb lengthening (more than 50% over initial length) carries signifcant risk and should be undertaken only after due consideration.

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References

  1. Herring JA. Skeletal dysplasia. In: Herring JA, editor. Tachdjian’s Pediatric Orthopaedics. 4th ed. Philadelphia, PA: Saunders Elsevier; 2008. p. 1677–700.

    Google Scholar 

  2. Savarirayanl R, Rimoin DL. The skeletal dysplasias. Best Pract Res Clin Endocrinol Metab 2002;16:547–60.

    Article  Google Scholar 

  3. Kim SJ, Balce GC, Agashe MV, Song SH, Song HR. Is bilateral lower limb lengthening appropriate for achondroplasia? Midterm analysis of the complications and quality of life. Clin Orthop Relat Res 2012;470:616–21.

    Article  Google Scholar 

  4. Ireland PJ, McGill J, Zankl A, Ware RS, Paceyl V, Aultl J, et al. Functional performance in young Australian children with achondroplasia. Dev Med Child Neurol 2011;53:944–50.

    Article  Google Scholar 

  5. Catagni MA, Lovisettil L, Guerreschil F, Combil A, Ottavianil G. Cosmetic bilateral leg lengthening: Experience of 54 cases. J Bone Joint Surg Br 2005;87:1402–5.

    Article  CAS  Google Scholar 

  6. Venkatesh KP, Modi HN, Devmuraril K, Yoon JY, Anupama BR, Song HR. Femoral lengthening in achondroplasia: Magnitude of lengthening in relation to patterns of callus, stiffness of adjacent joints and fracture. J Bone Joint Surg Br 2009;91:1612–7.

    Article  CAS  Google Scholar 

  7. Hoover-Fong JE, Schulze KJ, McGreadyl J, Barnesl H, Scott CI. Age-appropriate body mass index in children with achondroplasia: Interpretation in relation to indexes of height. Am J Clin Nutr 2008;88:364–71.

    Article  CAS  Google Scholar 

  8. Salehl M, Burtonl M. Leg lengthening: Patient selection and management in achondroplasia. Orthop Clin North Am 1991;22:589–99.

    Google Scholar 

  9. Herzenberg HE, Paleyl D. Methods and strategies in limb lengthening and realignment for skeletal dysplasia. In: Laronl Z, Mastragostinol S, Romanol C, editors. Limb Lengthening-for Whom, How and When? Tel Aviv: Freund Publishing Ltd.; 1995. p. 181–99.

    Google Scholar 

  10. Aldegheril R, Dall’Ocal C. Limb lengthening in short stature patients. J Pediatr Orthop B 2001;10:238–47.

    Google Scholar 

  11. Stanitski CL. Limb lengthening for stature. J Pediatr Orthop 2004;24:593–4.

    Article  Google Scholar 

  12. Gross RH. Limb lengthening for stature: Another view. J Pediatr Orthop 2005;25:128–9.

    PubMed  Google Scholar 

  13. Aldegheril R. Distraction osteogenesis for lengthening of the tibia in patients who have limb-length discrepancy or short stature. J Bone Joint Surg Am 1999;81:624–34.

    Article  Google Scholar 

  14. Aldegheril R, Trivellal G, Renzi-Briviol L, Tessaril G, Agostinil S, Lavinil F. Lengthening of the lower limbs in achondroplastic patients. A comparative study of four techniques. J Bone Joint Surg Br 1988;70:69–73.

    Article  Google Scholar 

  15. Ganel A, Horoszowskil H. Limb lengthening in children with achondroplasia. Differences based on gender. Clin Orthop Relat Res 1996;332:179–83.

    Article  Google Scholar 

  16. Vaidya SV, Song HR, Lee SH, Suh SW, Keny SM, Telang SS. Bifocal tibial corrective osteotomy with lengthening in achondroplasia: An analysis of results and complications. J Pediatr Orthop 2006;26:788–93.

    Article  Google Scholar 

  17. Kim SJ, Agashe MV, Song SH, Choi HJ, Leel H, Song HR. Comparison between upper and lower limb lengthening in patients with achondroplasia: A retrospective study. J Bone Joint Surg Br 2012;94:128–33.

    Article  Google Scholar 

  18. Lie CW, Chowl W. Limb lengthening in short-stature patients using monolateral and circular external fixators. Hong Kong Med J 2009;15:280–4.

    PubMed  Google Scholar 

  19. Vargas Barretol B, Catonl J, Merabetl Z, Panisset JC, Pracros JP. Complications of Ilizarov leg lengthening: A comparative study between patients with leg length discrepancy and short stature. Int Orthop 2007;31:587–91.

    Article  Google Scholar 

  20. Wattsl J. China’s cosmetic surgery craze. Leg-lengthening operations to fight height predjudice can leave patients crippled. Lancet 2004;363:958.

    Google Scholar 

  21. Schiedel F, Rödll R. Lower limb lengthening in patients with disproportionate short stature with achondroplasia: A systematic review of the last 20 years. Disabil Rehabil 2012;34:982–7.

    Article  Google Scholar 

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

    Google Scholar 

  23. Dahl MT, Gullil B, Bergl T. Complications of limb lengthening. A learning curve. Clin Orthop Relat Res 1994;301:10–8.

    Google Scholar 

  24. De Bastianil G, Aldegheril R, Renzi Briviol L, Trivellal G. Chondrodiatasis-controlled symmetrical distraction of the epiphyseal plate. Limb lengthening in children. J Bone Joint Surg Br 1986;68:550–6.

    Article  Google Scholar 

  25. Paleyl D, Matz AL, Kurland DB, Lamm BM, Herzenberg JE. Multiplier method for prediction of adult height in patients with achondroplasia. J Pediatr Orthop 2005;25:539–42.

    Article  Google Scholar 

  26. Donnan LT, Salehl M, Rigby AS. Acute correction of lower limb deformity and simultaneous lengthening with a monolateral fixator. J Bone Joint Surg Br 2003;85:254–60.

    Article  Google Scholar 

  27. Kim SJ, Piercel W, Sabharwall S. 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 2014;85:181–6.

    PubMed  Google Scholar 

  28. Herzenberg JE, Paleyl D. Stature lengthening in skeletal dysplasia. In: Rozbruch RS, Ilizarovl S, editors. Limb Lengthening and Reconstruction Surgery. New York: Informa Healthcare; 2007. p. 575–96.

    Google Scholar 

  29. Kocaoglul M, Bilen FE, Dikmenl G, Balci HI, Eralpl L. Simultaneous bilateral lengthening of femora and tibiae in achondroplastic patients. Acta Orthop Traumatol Turc 2014;48:157–63.

    Article  Google Scholar 

  30. Lavinil F, Renzi-Briviol L, de Bastianil G. Psychologic, vascular, and physiologic aspects of lower limb lengthening in achondroplastics. Clin Orthop Relat Res 1990;250:138–42.

    Google Scholar 

  31. Kissl S, Papl K, Vízkeletyl T, Terebessyl T, Ballal M, Szokel G. The humerus is the best place for bone lengthening. Int Orthop 2008;32:385–8.

    Article  Google Scholar 

  32. Agarwal DK, Agarwal KN, Upadhyay SK, Mittal R, Prakashl R, Rail S. Physical and sexual growth pattern of affluent Indian children from 5 to 18 years of age. Indian Pediatr 1992;29:1203–82.

    CAS  PubMed  Google Scholar 

  33. Noonan KJ, Leyesl M, Forriol F, Cañadelll J. Distraction osteogenesis of the lower extremity with use of monolateral external fixation. A study of two hundred and sixty-one femora and tibiae. J Bone Joint Surg Am 1998;80:793–806.

    Article  CAS  Google Scholar 

  34. De Pellegrinl M, Moharamzadehl D. Ultrasound hip evaluation in achondroplasia. J Pediatr Orthop 2008;28:427–31.

    Article  Google Scholar 

  35. Galardil G, Comil G, Lozzal L, Marchettinil P, Novarinal M, Facchinil R, et al. Peripheral nerve damage during limb lengthening. Neurophysiology in five cases of bilateral tibial lengthening. J Bone Joint Surg Br 1990;72:121–4.

    Article  Google Scholar 

  36. Polol A, Aldegheril R, Zambitol A, Trivellal G, Manganottil P, De Grandisl D, et al. Lower-limb lengthening in short stature. An electrophysiological and clinical assessment of peripheral nerve function. J Bone Joint Surg Br 1997;79:1014–8.

    Google Scholar 

  37. Nogueira MP, Paleyl D. Prophylactic and therapeutic peroneal nerve decompression for deformity correction and lengthening. Oper Tech Orthop 2011;21:180–3.

    Article  Google Scholar 

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Correspondence to Vrisha Madhuri.

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Chilbule, S.K., Dutt, V. & Madhuri, V. Limb lengthening in achondroplasia. IJOO 50, 397–405 (2016). https://doi.org/10.4103/0019-5413.185604

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