Abstract
Background
The operative procedures to correct multiplanar bone deformities may be indicated for prevention of secondary orthopaedic complications in children with X-linked hereditary hypophosphatemic rickets (XHPR). Different problems related to surgical correction were reported: increased rate of non-union, delayed union, recurrent deformity, deep intramedullary infection, refracture, nerve palsy, and pin tract infection. The aim of this retrospective study was comparison of results of correction in children with XHPR who underwent the treatment with either the Ilizarov device alone or a combined technique: the Ilizarov fixator with flexible intramedullary nailing (FIN) with hydroxyapatite bioactive coating and FIN.
Material and methods
We retrospectively analysed 47 cases (children of age under 14 years) affected by XHPR. Simultaneous deformity correction in femur and tibia was performed with the Ilizarov device (group I) or the combined method (group II). This article is based on the results of a historical comparative retrospective study from the same institution.
Results
The duration of external fixation is noted to be shorter applying the combined technique: 124.7 days (group I) vs 87.4 days (group II). In both groups deformity correction was achieved with a proper alignment. Nevertheless, while a child continues to grow during long-term follow-up, deviations of the mechanic axis from the centre of the knee joint have been developing again and values of mLDFA, mMPTA have become pathologic in the most of the cases. In group I location of a newly developed deformity resembled a pre-operative one, whereby both diaphyseal and metaphyseal parts were deformed. In group II in all the cases an apex of deformity was located in distal metadiaphyseal zone of the femur and proximal metadiaphyseal zone of the tibia. It is important to note that all of those in group II were out of the zone of the intramedullary nail.
Conclusion
Simultaneous correction of femoral and tibial deformities by means of circular external fixators is preferable. Application of a combined osteosynthesis allows to considerably reduce the duration of external fixation and decrease the number of complications. There were no recurrent deformities in parts of bone reinforced by intramedullary nails.
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References
Albright F, Butler AM, Bloomberg E (1937) Rickets resistant to vitamin D therapy. Am J Dis Child 54:529–547
Cho HY, Lee BH, Knag JH, Ha IS, Cheong HI, Choi Y (2005) A clinical and molecular genetic study of hypophosphatemic rickets in children. Pediatr Res 58:329–333
Imel EA, DiMeglio LA, Hui SL, Carpenter TO, Econs MJ (2010) Treatement of X-linked hypophosphatemia with calcitriol and phosphate increases circulating fibroblast growth factor 23 concentrations. J Clin Endocrinol Metab 95(4):1846–1859
Beck-Nielsen SS, Brock-Jacobsen B, Gram J, Brixen K, Jensen TK (2009) Incidence and prevalence of nutritional and hereditary rickets in southern Denmark. Eur J Endocrinol 160:491–497
Davies M, Stanbury SW (1981) The rheumatic manifestations of metabolic bone disease. Clin Rheum Dis 7:595–646
Ruppe MD X-Linked Hypophosphatemia. GeneReviews® [Internet]. http://www.ncbi.nlm.nih.gov/books/NBK83985/. Initial posting: February 9, 2012; Last update: October 16, 2014
Rubinovitch M, Said SE, Glorieux FH, Cruess RL, Rogala E (1988) Principles and results of corrective lower limb osteotomies for patients with vitamin D-resistant hypophosphatemic rickets. Clin Orthop Relat Res 237:264–270
Kocaoglu M, Bilen FE, Sen C, Eralp L, Balci HI (2011) Combined technique for the correction of lower-limb deformities resulting from metabolic bone disease. J Bone Joint Surg (Br) 93(1):52–56
Zivičnjak M, Schnabel D, Billing H, Staude H, Filler G, Querfeld U, Schumacher M, Pyper A, Schröder C, Brämswig J, Haffner D, Hypophosphatemic Rickets Study Group of the “Arbeitsgemeinschaft für Pädiatrische Endokrinologie” and “Gesellschaft für Pädiatrische Nephrologie” (2011) Age-related stature and linear body segments in children with X-linked hypophosphatemic rickets. Pediatr Nephrol 26(2):223–231
Chesney RW, Mazess RB, Rose P, Hamstra AJ, DeLuca HF, Breed AL (1983) Long-term influence of calcitriol (1,25-dihydroxyvitamin D) and supplemental phosphorous in X-linked hypophosphatemic rickets. Pediatrics 71:559–567
Petersen DJ, Boniface AM, Schranck FW, Rupich RC, Whyte MP (1992) X-linked hypophosphatemic rickets: a study (with literature review) of linear growth response to calcitriol and phosphate therapy. J Bone Miner Res 7(6):583–597
Carpenter TO, Imel EA, Holm IA, Jan de Beur SM, Insogna KL (2012) A clinician’s guide to X-linked hypophosphatemia. J Bone Miner Res 26(7):1381–1388
Fucentese SF, Neuhaus TJ, Ramseier LE, Ulrich Exner G (2008) Metabolic and orthopedic management of X-linked vitamin D-resistant hypophosphatemic rickets. J Child Orthop 2:285–291
Petje G, Meizer R, Radler C, Aigner N, Grill F (2008) Deformity correction in children with hereditary hypophosphatemic rickets. Clin Orthop Relat Res 466:3078–3085
Evans GA, Arulanantham K, Gage JR (1980) Primary hypophosphatemic rickets: effect of oral phosphate and vitamin D on growth and surgical treatment. J Bone Joint Surg Am 62:1130–1138
Eyres KS, Brown J, Douglas DL (1993) Osteotomy and intramedullary nailing for the correction of progressive deformity in vitamin D-resistant hypophosphataemic rickets. J R Coll Surg Edinb 38:50–54
Matsubara H, Tsuchiya H, Kabata T, Sakurakichi K, Watanabe K, Tomita K (2008) Deformity correction for vitamin D-resistant hypophosphatemic rickets of adults. Arch Orthop Trauma Surg 128(10):1137–1143
Choi IH, Kim JK, Chung CY, Cho TJ, Lee SH, Suh SW, Whang KS, Park HW, Song KS (2002) Deformity correction of knee and leg lengthening by Ilizarov method in hypophosphatemic rickets: outcomes and significance of serum phosphate level. J Pediatr Orthop 22(5):626–631
Song HR, Soma Raju VV, Kumar S, Lee SH, Suh SW, Kim JR, Hong JS (2006) Deformity correction by external fixation and/or intramedullary nailing in hypophosphatemic rickets. Acta Orthop 77(2):307–314
Ferris B, Walker C, Jackson A, Kirwan E (1991) The orthopaedic management of hypophosphataemic rickets. J Pediatr Orthop 11(3):367–373
Popkov D, Journeau P, Popkov A, Haumont T, Lascombes P (2010) Ollier’s disease limb lengthening: Should intramedullary nailing be combined with circular external fixation? Orthop Traumatol Surg Res 96:348–353
Popkov D, Popkov A, Haumont T, Journeau P, Lascombes P (2010) Flexible intramedullary nail use in limb lengthening. J Pediatr Orthop 30:910–918
Popkov D (2006) ECMES dans les corrections des deformations chez l’enfant atteint de rachitisme vitaminorésistant hypophosphatémique familial. In: Lascombes P (ed) Embrochage centromédullaire élastique stable. Elsevier, pp 301–309
Lascombes P (2006) Embrochage centromédullaire élastique stable. Elsevier
Paley D, Herzenberg JE, Tetsworth K, McKie J, Bhave A (1994) Deformity planning for frontal and sagittal plan corrective osteotomies. Orthop Clin N Am 25:425–465
Popkov D, Lascombes P, Berte N, Hetzel L, Baptista BR, Popkov A, Journeau P (2015) The normal radiological anteroposterior alignment of the lower limb in children. Skelet Radiol 44(2):197–206
Lascombes P, Popkov D, Huber H, Haumont T, Journeau P (2012) Classification of complications after progressive long bone lengthening: proposal for a new classification. Orthop Traumatol Surg Res 98(6):629–637
Boutaud B, Laville JM (2004) L’embrochage centromédullaire dans l’ostéogenèse imparfaite. Rev Chir Orthop Reparatrice Appar Mot 90(4):304–311
Kanel JS, Price CT (1995) Unilateral external fixation for corrective osteotomies in patients with hypophosphatemic rickets. J Pediatr Orthop 15(2):232–235
Ozono K (2010) Bone and joint diseases in children. Phosphaturic hormone, FGF23 and bone metabolism. Clin Calcium 20(6):896–903
Shimada T, Yamazaki Y, Takahashi M, Hasegawa H et al (2005) Vitamin D receptor-independent FGF23 actions in regulating phosphate and vitamin D metabolism. Am J Physiol Ren Physol 289(5):1088–1095
Harrell RM, Lyles KW, Harrelson JM, Friedman NE, Drezner MK (1985) Healing of bone disease in X-linked hypophosphatemic rickets/osteomalacia: induction and maintenance with phosphorus and calcitriol. J Clin Invest 75:1858–1868
Larson AN, Trousdale RT, Pagnano MW, Hanssen AD, Lewallen DG, Sanchez-Sotelo J (2010) Hip and knee arthroplasty in hypophosphatemic rickets. J Arthroplasty 25(7):1099–1103
Gubin AV, Borzunov DY, Malkova TA (2013) The Ilizarov paradigm: thirty years with the Ilizarov method, current concerns and future research. Int Orthop 37(8):1533–1539
Eren I, Eralp L, Kocaoglu M (2013) Comparative clinical study on deformity correction accuracy of different external fixators. Int Orthop 37(11):2247–2252
Lan X, Zhang L, Tang P, Xia H, Li G, Peng A, Han Y, Yuan B, Xu W (2013) S-osteotomy with lengthening and then nailing compared with traditional Ilizarov method. Int Orthop 37(10):1995–2000
Chatelet JC, Setiey L (2004) Comportement osseux à long terme après implantation d’un pivot fémoral totalement revêtu d’hydroxyapatite. Rev Chir Orthop Reparatrice Appar Mot 90(7):628–635
Popkov DA, Kononovich NA, Shutov RB (2014) The effect of tibial transphyseal reinforcement on the growth and response of leg tissues. Ross Fiziol Zh Im I M Sechenova 100(7):881–890, Russian
Acknowledgments
The authors thank Natalia Popkova for her assistance in preparing the publication.
Conflicts of Interest
The authors have no competing interest to declare. Informed consent was obtained from all individual participants (parents or guardians) included in the study.
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Popkov, A., Aranovich, A. & Popkov, D. Results of deformity correction in children with X-linked hereditary hypophosphatemic rickets by external fixation or combined technique. International Orthopaedics (SICOT) 39, 2423–2431 (2015). https://doi.org/10.1007/s00264-015-2814-7
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DOI: https://doi.org/10.1007/s00264-015-2814-7