ICLL: CPT CONGENITAL PSEUDARTHROSIS of the TIBIA.
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Transcript of ICLL: CPT CONGENITAL PSEUDARTHROSIS of the TIBIA.
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CONGENITAL PSEUDARTHROSIS of the TIBIA
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Pathology
• Fibrous Hamartoma surrounding bone
• Associated with NF in over 50%
• No NF at CPT site
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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)
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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.
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Is this theory really new?
• Codivilla (Italy) 1903
autogenous osteoperiosteal graft
• Cambras (Cuba) 1977
periosteal graft from mother
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CPT: Classifications
• Camurati 1930• Badgley 1952• Boyd 1958• Apoil 1970• Andersen 1973• Crawford 1986• Crawford 1999• El-Rosassy-Paley 2000
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I II III
El-Rosassy-Paley Classification
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Type I
• Atrophic, narrow bone ends• Mobile• No previous surgery
El-Rosassy-Paley Classification
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Type II
• Atrophic, narrow bone ends• Mobile• Previous surgery
El-Rosassy-Paley Classification
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Type III
• Hypertrophic, wide bone ends• Stiff Previous surgery
El-Rosassy-Paley Classification
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Type I Treatment
Lateral
AP
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preop postop
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Flexible IM nail3 year follow-up
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Fassier-Duval Telescopic Nail
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LLD
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Type II Treatment
Bone transport
Acute shortening
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CPT resection
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acute shortening
re-lengthening
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bone transport healed
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ReFx follow-upIM rod
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Treatment
Type III • Closed distraction + compression
• Gradual deformity correction
• ± Lengthening
• ± IM Rod (late)
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distraction of CPT follow-up
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pre-op compression healed
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Treatment
Type III • Closed distraction
• Gradual deformity correction
• ± Lengthening
• ± IM Rod (late)
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distraction of CPT follow-up
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Periosteal & Bone Grafting with IM rod and Ilizarov
Union: 20/20
1997-200820 patients treated
RESULTS
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Don’t ForgetBracing until Skeletal Maturity