ICLL: CPT CONGENITAL PSEUDARTHROSIS of the TIBIA.

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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, , 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 1930 Badgley 1952 Boyd 1958 Apoil 1970 Andersen 1973 Crawford 1986 Crawford 1999 El-Rosassy-Paley2000

IIIIII 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

preoppostop

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 transporthealed

ReFxfollow-upIM rod

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

distraction of CPTfollow-up

pre-opcompression healed

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

distraction of CPTfollow-up

Periosteal & Bone Grafting with IM rod and Ilizarov Union: 20/ patients treated RESULTS

Don’t Forget Bracing until Skeletal Maturity