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CONGENITAL PSEUDARTHROSIS of the TIBIA

CONGENITAL PSEUDARTHROSIS of the TIBIA. ICLL: CPT. Pathology. Fibrous Hamartoma surrounding bone Associated with NF in over 50% No NF at CPT site. Where is the disease? Bone vs Periosteum. Observation: Slow bone remodelling of pin holes Remodelling is a periosteal function

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CONGENITAL PSEUDARTHROSIS of the TIBIA

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  1. CONGENITAL PSEUDARTHROSIS of the TIBIA ICLL: CPT

  2. Pathology • Fibrous Hamartoma surrounding bone • Associated with NF in over 50% • No NF at CPT site

  3. Where is the disease?Bone vs Periosteum Observation: Slow bone remodelling of pin holes Remodelling is a periosteal function New theory (Paley 1995: CPT is a periosteal disease and not a bone disease (the bone is affected secondarily)

  4. Hermanns-Sachweh B et al: Vascular Changes in the Periosteum of Congenital Pseudarthrosis of the Tibia. Pathology Research and Practice 201, 305-312, 2005. • Neural cells forming a tight sheath around the periosteal arteries reminiscent of the way Schwann cells surround peripheral nerve fibers. • Result: accumulation of nerve cells in the periosteum cause narrowing or obliteration of the periosteal vessels. • Result: periosteum undergoes hypoxemic degeneration resulting in the formation of a thick fibrous cuff. This leads to impaired oxygen and nutrient supply to the subperiosteal bone with secondary atrophic bone changes.

  5. Is this theory really new? • Codivilla (Italy) 1903 autogenous osteoperiosteal graft • Cambras (Cuba) 1977 periosteal graft from mother

  6. CPT: Classifications • • Camurati 1930 • • Badgley 1952 • • Boyd 1958 • • Apoil 1970 • • Andersen 1973 • • Crawford 1986 • • Crawford 1999 • El-Rosassy-Paley 2000

  7. I II III El-Rosassy-Paley Classification

  8. Type I • Atrophic, narrow bone ends • Mobile • No previous surgery El-Rosassy-Paley Classification

  9. Type II • Atrophic, narrow bone ends • Mobile • Previous surgery El-Rosassy-Paley Classification

  10. Type III • Hypertrophic, wide bone ends • Stiff •  Previous surgery El-Rosassy-Paley Classification

  11. Type I Treatment Lateral AP

  12. preop postop

  13. 3 year follow-up Flexible IM nail

  14. Fassier-Duval Telescopic Nail

  15. LLD

  16. Type II Treatment Bone transport Acute shortening

  17. CPT resection

  18. acute shortening re-lengthening

  19. bone transport healed

  20. ReFx IM rod follow-up

  21. Treatment • Type III • Closed distraction + compression • Gradual deformity correction • ± Lengthening • ± IM Rod (late)

  22. distraction of CPT follow-up

  23. pre-op compression healed

  24. Treatment • Type III • Closed distraction • Gradual deformity correction • ± Lengthening • ± IM Rod (late)

  25. distraction of CPT follow-up

  26. RESULTS Periosteal & Bone Grafting with IM rod and Ilizarov 1997-2008 20 patients treated Union: 20/20

  27. Don’t Forget Bracing until Skeletal Maturity

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