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A tale of two tibias: a review of treatment options for congenital pseudarthrosis of the tibia

  • Original Clinical Article
  • Published:
Journal of Children's Orthopaedics

Abstract

Purpose

Two common treatment options for congenital pseudarthrosis of the tibia (CPT) are intramedullary fixation following resection/shortening of the pseudarthrosis site and reconstruction with an Ilizarov external fixator following resection. We present in detail a narrative of two cases with similar degrees of tibial dysplasia associated with NF-1 treated using these different methods and followed to completion.

Methods

Technical issues and details of the treatment methods from case reports are discussed in depth. The eventual profoundly different outcomes are correlated to the technical variations used.

Results

Treatment with the Charnley–Williams rodding method and aggressive bone grafting supplemented by rh-BMP2 resulted in a normal functioning limb at maturity, while treatment with first, an ineffective version of IM rodding, followed by two sessions of bone transport using an Ilizarov fixator failed to gain useful union and eventually resulted in amputation.

Conclusions

Technical details, heretofore inadequately reviewed in the literature, are crucial to the success of either of these commonly utilized treatment methods for CPT.

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References

  1. Johnston CE II (2002) Congenital pseudarthrosis of the tibia. Results of technical variations in the Charnley–Williams procedure. J Bone Joint Surg Am 84:1799–1810

    PubMed  Google Scholar 

  2. Williams PF (1965) Fragmentation and rodding in osteogenesis imperfecta. J Bone Joint Surg Br 47:23–31

    PubMed  CAS  Google Scholar 

  3. Langenskiöld A (1967) Pseudarthrosis of the fibula and progressive valgus deformity of the ankle in children: treatment by fusion of the distal tibial and fibular metaphyses. Review of three cases J Bone Joint Surg Am 49:463–470

    PubMed  Google Scholar 

  4. Karol LA, Haideri NF, Halliday SE, Smitherman B, Johnston CE II (1998) Gait analysis and muscle strength in children with congenital pseudarthrosis of the tibia: the effect of treatment. J Pediatr Orthop 18:381–386

    PubMed  CAS  Google Scholar 

  5. Joseph B, Somaraju VV, Shetty SK (2003) Management of congenital pseudarthrosis of the tibia under age 3 years of age: effect of early surgery on union of the pseudarthrosis and growth of the limb. J Pediatr Orthop 23:740–746

    Article  PubMed  Google Scholar 

  6. Aegerter EE (1950) The possible relationship of neurofibromatosis, congenital pseudarthrosis and fibrous dysplasia. J Bone Joint Surg Am 32:618–626

    PubMed  Google Scholar 

  7. Briner J, Yunis E (1973) Ultrastructure of congenital pseudarthrosis of the tibia. Arch Pathol 95:97–99

    PubMed  CAS  Google Scholar 

  8. Cui G, Lei W, Li J et al (2002) Histopathology of congenital pseudarthrosis of the tibia. Zhonghua Yi Xue Za Zhi 82:487–491

    PubMed  CAS  Google Scholar 

  9. Charnley J (1956) Congenital pseudarthrosis of tibia treated by intramedullary nail. J Bone Joint Surg Am 38:283–290

    PubMed  Google Scholar 

  10. Richards BS, Welch RD, Shrader MW, Johnston CE (2005) Use of rhBMP-2 in congenital pseudarthrosis of the tibia. Presented at the Annual Meeting of the Pediatric Orthopedic Society of North America, Ottawa, May 12–15 2005

  11. Grill F, Bollini G, Dungl P, Fixsen J, Hefti F, Ippolito E, Romanus B, Tudisco C, Weintroub S (2000) Treatment approaches for congenital pseudarthrosis of the tibia: results of the EPOS multicenter study. J Pediatr Orthop B 9:75–89

    Article  PubMed  CAS  Google Scholar 

  12. Paley D, Catagni M, Argnani F, Prevot J, Bell D, Armstrong P (1992) Treatment of congenital pseudoarthrosis of the tibia using the Ilizarov technique. Clin Orthop 280:81–93

    PubMed  Google Scholar 

  13. Green SA, Jackson JM, Wall DM, Marinow H, Ishkanian J (1992) Management of segmental defects by the Ilizarov intercalary bone transport method. Clin Orthop 280:136–142

    PubMed  Google Scholar 

  14. Cattaneo R, Catagni M, Johnson EE (1992) The treatment of non-unions and segmental defects of the tibia by the methods of Ilizarov. Clin Orthop 280:143–152

    PubMed  Google Scholar 

  15. Boero S, Catagni M, Donzelli O, Facchini R, Frediana PV (1997) Congenital pseudarthrosis of the tibia associated with neurofibromatosis-1: treatment with Ilizarov’s device. J Pediatr Orthop 17:675–684

    Article  PubMed  CAS  Google Scholar 

  16. Ghanem I, Damsin JP, Carlioz H (1997) Ilizarov technique in the treatment of congenital pseudarthrosis of the tibia. J Pediatr Orthop 17:685–690

    Article  PubMed  CAS  Google Scholar 

  17. Keret D, Bollini G, Dungl P, Fixsen J, Grill F, Hefti F, Ippolito E, Romanus B, Tudisco C, Weintroub S (2000) The fibula in congenital pseudarthrosis of the tibia: the EPOS multicenter study. J Pediatr Orthop B 9:69–74

    Article  PubMed  CAS  Google Scholar 

  18. El-Rosasy MA, Paley D, Herzenburg JE (2007) Congenital Pseudarthrosis of the Tibia. In: Rozbruch RS, Ilizarov S (eds) Limb lengthening and reconstruction surgery. Informa Healthcare USA, New York, pp 485–493

    Google Scholar 

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Acknowledgments

The authors wish to acknowledge the value of Charles Dickens’ perspective in the completion of this manuscript.

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Correspondence to Charles E. Johnston.

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Johnston, C.E., Birch, J.G. A tale of two tibias: a review of treatment options for congenital pseudarthrosis of the tibia. J Child Orthop 2, 133–149 (2008). https://doi.org/10.1007/s11832-008-0084-2

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  • DOI: https://doi.org/10.1007/s11832-008-0084-2

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