Presentation on theme: "ICLL: CPT CONGENITAL PSEUDARTHROSIS of the TIBIA."— Presentation transcript:
ICLL: CPT CONGENITAL PSEUDARTHROSIS of the TIBIA
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 New theory (Paley 1995: CPT is a periosteal disease and not a bone disease (the bone is affected secondarily)
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.
Is this theory really new? Codivilla (Italy) 1903 autogenous osteoperiosteal graft Cambras (Cuba) 1977 periosteal graft from mother