Tibial lengthening in achondroplasia patients aged 6–9 years as the first stage of growth correction

Background. Height increase and improvement of body proportions for achondroplasia patients normally require two or more stages of reconstructive treatment to be followed by rehabilitation between lengthening periods, and growth correction can take a significant part of life in the cohort of patients. What is the best age to start growth correction is an important question.

The purpose of this paper was to present an argument for arranging the first stage of growth correction in achondroplasia patients aged 6–9 years based on the structural and functional muscle evaluation of tibiae to be lengthened.

Material and methods. Achondroplasia patients aged 6–9 years (n = 30) were examined preoperatively, during distraction, fixation and at 1.5 to 2 years of frame removal. Tibial lengthening was produced monofocally and bifocally. Contractile force of the dorsal and plantar flexion muscles of the foot was measured with dynamometer. Ultrasonography of tibial muscles was performed with HITACHI ultrasound imaging device (Japan).

Results. Achondroplasia patients aged 6–9 year who underwent tibial lengthening of at least 50% of the initial length developed neuropathy in 2.6 % of cases and soft tissue inflammation in 5.6 % of cases. Characteristic muscle striation of m. tibialis anterior and m. extensor digitorum longus appeared to restore at 1.5 to 2 years of tibial lengthening with clear contouring of the intermuscular septa and retained contractile force of the muscles. The contractile force restored to 96.15 % of preoperative level in the anterior tibial muscles, and to 101.92 % in the posterior muscles.

Conclusion. The comprehensive clinical, ultrasonographic and dynamometric evaluation of tibial muscles presented a good argument for tibial lengthening in achondroplasia patients aged 6–9 years. Regained muscle striation and spare capacity of m. tibialis anterior and extensor digitorum longus, the restored force of the anterior tibial muscles to 96.15 % of the preoperative level suggested the possibility for the next stage of growth correction.

1. Popkov A.V., Shevtsov V.I. eds. Akhondroplaziia: rukovodstvo dlia vrachei [Achondroplasia: guide for physicians]. M., Meditsina, 2001, 352 p. (in Russian)

2. Daugherty A. Achondroplasia: Etiology, Clinical Presentation, and Management. Neonatal Netw., 2017, vol. 36, no. 6, pp. 337-342. DOI: 10.1891/0730-0832.36.6.337

3. Trotter T.L., Hall J.G. American Academy of Pediatrics Committee on Genetics. Health supervision for children with achondroplasia. Pediatrics, 2005, vol. 116, no. 3, pp. 771-783. DOI: 10.1542/peds.2005-1440

4. Shirley E.D., Ain M.C. Achondroplasia: manifestations and treatment. J. Am. Acad. Orthop. Surg., 2009, vol. 17, no. 4, pp. 231-241. DOI: 10.5435/00124635-200904000-00004

5. Satiroglu-Tufan N.L., Tufan A.C., Semerci C.N., Bagci H. Accurate diagnosis of a homozygous G1138A mutation in the fibroblast growth factor receptor 3 gene responsible for achondroplasia. Tohoku J. Exp. Med., 2006, vol. 208, no. 2, pp. 103-107. DOI: 10.1620/tjem.208.103

6. Vasserman N.N., Shchagina O.A., Poliakov A.V. Rezultaty ispolzovaniia novoi meditsinskoi tekhnologii «Sistema detektsii naibolee chastykh mutatsii gena FGFR3, otvetstvennogo za akhondroplaziiu i gipokhondroplaziiu v DNK-diagnostike [Results of using new medical technology “A system for detecting the most frequent mutations of FGFR3 gene, responsible for achondroplasia and hypochondroplasia” in DNA-diagnosis]. Meditsinskaia Genetika, 2016, vol. 15, no. 2, pp. 37-41. (in Russian)

7. Kanaka-Gantenbein C. Present status of the use of growth hormone in short children with bone diseases (diseases of the skeleton). J. Pediatr. Endocrinol. Metab., 2001, vol. 14, no. 1, pp. 17-26. DOI: 10.1515/jpem.2001.14.1.17

8. Klimov O.V., Aranovich A.M. Povtornoe udlinenie goleni kak etap rekonstruktsii ODS patsientov s akhondroplaziei [Repeated tibial lengthening as a stage of locomotor reconstruction in achondroplasia patients]. Genij Ortopedii, 2017, vol. 23, no. 4, pp. 401-404.

9. Diachkova G.V., Aranovich A.M., Diachkov K.A., Klimov O.V., Novikov K.I., Sutiagin I.V. Ortopedicheskie, psikhologicheskie, sotsialnye i filosofskie aspekty v reshenii problemy lecheniia bolnykh akhondroplaziei po metodu Ilizarova [Orthopaedic, psychological, social and philosophical aspects of achondroplasia patients treated with the Ilizarov method]. Genij Ortopedii, 2018, vol. 24, no. 4, pp. 465-473.

10. Song S.H., Agashe M.V., Huh Y.J., Hwang S.Y., Song H.R. Physeal growth arrest after tibial lengthening in achondroplasia: 23 children followed to skeletal maturity. Acta Orthop., 2012, vol. 83, no. 3, pp. 282-287. DOI: 10.3109/17453674.2012.678802

11. Dolganov D.V., Erokhin A.N., Ershov E.V., Neretin A.S., Popkov A.V., Popkov D.A., Saifutdinov M.S., Filimonova G.N., Shchurov V.A. Vrozhdennoe ukorochenie nizhnikh konechnostei u detei [Congenital shortening of the lower limbs in children]. Ed. by Popkov A.V. Chelyabinsk, 2011, 510 p. (in Russian)

12. Aldegheri R. Distraction osteogenesis for lengthening of the tibia in patients who have limb-length discrepancy or short stature. J. Bone Joint Surg. Am., 1999, vol. 81, no. 5, pp. 624-634. DOI: 10.2106/00004623-199905000-00004.

13. Kadono I., Kitoh H., Mishima K., Matsushita M., Sato K., Kako M., Ishiguro N. Changes in the range of motion of the lower limb joints during extensive tibial lengthening in achondroplasia. J. Pediatr. Orthop. B, 2018, vol. 27, no. 6, pp. 535-540. DOI: 10.1097/BPB.0000000000000526

14. Griffith S.I., McCarthy J.J., Davidson R.S. Comparison of the complication rates between first and second (repeated) lengthening in the same limb segment. J. Pediatr. Orthop., 2006, vol. 26, no. 4, pp. 534-536. DOI: 10.1097/01.bpo.0000226275.70706.d5

15. Chilbule S.K., Dutt V., Madhuri V. Limb lengthening in achondroplasia. Indian J. Orthop., 2016, vol. 50, no. 4, pp. 397-405. DOI: 10.4103/0019-5413.185604

16. Ilizarov G.A., Gracheva V.I., Shchurov V.A., Maltsev V.D., Saifutdinov M.S. Udlinenie nizhnikh konechnostei pri akhondroplazii [Lengthening of the lower limbs in achondroplasia]. Problemy chreskostnogo osteosinteza v ortopedii i travmatologii: Zakonomernosti regeneratsii i rosta tkanei pod vliianiem napriazheniia rastiazheniia. Sb. nauch. tr. [Transosseous Osteosynthesis Problems in Orthopaedics and Traumatology: Regularities of tissue regeneration and growth under tension stress influence. Proceedings of the Scientific Conference]. KNIIEKOT. Kurgan, 1982, Issue 8, pp. 143-152. (in Russian)

17. Paley D. Lengthening reconstruction surgery of the foot and ankle for fibular hemimelia. 3rd Meeting of the A.S.A.M.I. International: Abstr. Book. Istanbul, 2004, pp. 167-171.

18. Schiedel F., Rödl R. Lower limb lengthening in patients with disproportionate short stature with achondroplasia: a systematic review of the last 20 years. Disabil. Rehabil., 2012, vol. 34, no. 12, pp. 982-987. DOI: 10.3109/09638288.2011.631677

19. Ganel A ., Horoszowski H. Limb lengthening in children with achondroplasia. Differences based on gender. Clin. Orthop. Relat. Res., 1996, no. 332, pp. 179-183. DOI: 10.1097/00003086-199611000-00024

20. Burghardt R.D., Yoshino K., Kashiwagi N., Yoshino S., Bhave A., Paley D., Herzenberg J.E. Bilateral double level tibial lengthening in dwarfism. J. Orthop., 2015, vol. 12, no. 4, pp. 242-247. DOI: 10.1016/j.jor.2015.05.006

21. Paley D. Problems, obstacles, and complications of limb lengthening by the Ilizarov technique. Clin. Orthop. Relat. Res., 1990, no. 250, pp. 81-104.

22. Bhave A., Shabtai L., Woelber E., Apelyan A., Paley D., Herzenberg J.E. Muscle strength and knee range of motion after femoral lengthening. Acta Orthop., 2017, vol. 88, no. 2, pp. 179-184. DOI: 10.1080/17453674.2016.1262678

23. Park K.W., Garcia R.A., Rejuso C.A., Choi J.W., Song H.R. Limb Lengthening in Patients with Achondroplasia. Yonsei Med. J., 2015, vol. 56, no. 6, pp. 1656-1662. DOI: 10.3349/ymj.2015.56.6.1656

24. Ko K.R., Shim J.S., Chung C.H., Kim J.H. Surgical Results of Limb Lengthening at the Femur, Tibia, and Humerus in Patients with Achondroplasia. Clin. Orthop. Surg., 2019, vol. 11, no. 2, pp. 226-232. DOI: 10.4055/cios.2019.11.2.226

25. Donaldson J., Aftab S., Bradish C. Achondroplasia and limb lengthening: Results in a UK cohort and review of the literature. J. Orthop., 2015, vol. 12, no. 1, pp. 31-34. DOI: 10.1016/j.jor.2015.01.001

26. Aranovich A.M., Stogov M.V., Kireeva E.A., Menshchikova T.I. Prognozirovanie i kontrol techeniia distraktsionnogo osteogeneza. Analiticheskii obzor [Prediction and control of distraction osteogenesis course. Analytical review]. Genij Oropedii, 2019, vol. 25, no. 3, pp. 400-406.

27. Shchurov V.A., Dolganova T.I., Dolganov D.V. Ustanovka dlia izmereniia sily myshts goleni [An assembly for measuring the leg muscle strength]. Meditsinskaia Tekhnika, 2016, no. 2, pp. 37-39. (in Russian)

28. MakNelli Iu. Ultrazvukovye issledovaniia kostno-myshechnoi sistemy: prakticheskoe rukovodstvo [Ultrasound investigations of the osteomuscular system: practical guide]. M., Vidar, 2007, pp. 179-302. (in Russian)

29. Menshchikova T.I., Aranovich A.M. Otsenka rezervnykh vozmozhnostei perednei gruppy myshts goleni u bolnykh akhondroplaziei v protsesse udlineniia (ultrazvukovoe issledovanie) [Assessment of the reserve scopes of the anterior muscle group of the leg in patients with achondroplasia in the process of lengthening (An ultrasound study)]. Vestnik Travmatologii i Ortopedii im. N.N. Priorova, 2015, no. 3, pp. 13-19. (in Russian)