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
CPT: Classifications • Camurati • Badgley • Boyd • Apoil • Andersen • Crawford • El-Rosassy-Paley 1930 1952 1958 1970 1973 1986 1999 2000
I II III El-Rosassy-Paley Classification
Type I • Atrophic, narrow bone ends • Mobile • No previous surgery El-Rosassy-Paley Classification
Type II • Atrophic, narrow bone ends • Mobile • Previous surgery El-Rosassy-Paley Classification
Type III • Hypertrophic, wide bone ends • Stiff • Previous surgery El-Rosassy-Paley Classification
Type I Treatment Lateral AP
preop postop
Flexible IM nail 3 year follow-up
Fassier-Duval Telescopic Nail
LLD
Type II Treatment Bone transport Acute shortening
CPT resection
acute shortening re-lengthening
bone transport healed
Re. Fx IM rod follow-up
Treatment Type III • Closed distraction + compression • Gradual deformity correction • ± Lengthening • ± IM Rod (late)
distraction of CPT follow-up
pre-op compression healed
Treatment Type III • Closed distraction • Gradual deformity correction • ± Lengthening • ± IM Rod (late)
distraction of CPT follow-up
RESULTS Periosteal & Bone Grafting with IM rod and Ilizarov 1997 -2008 20 patients treated Union: 20/20
Don’t Forget Bracing until Skeletal Maturity
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