1. ICLL: CPT CONGENITAL PSEUDARTHROSIS of the TIBIA

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-Paley2000

7. IIIIII 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

18. preoppostop

20. Flexible IM nail 3 year follow-up

21. Fassier-Duval Telescopic Nail

28. LLD

29. Type II Treatment Bone transport Acute shortening

30. CPT resection

31. acute shortening re-lengthening

33. bone transporthealed

34. ReFxfollow-upIM rod

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

36. distraction of CPTfollow-up

37. pre-opcompression healed

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

39. distraction of CPTfollow-up

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

41. Don’t Forget Bracing until Skeletal Maturity