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Congenital Pseudoarthrosis Tibia

By Prof Kit Song, Seattle Children’s Hospital, USA

POSICON 2012
Courtesy:
Dr Taral Nagda, India
Dr Sandeep Patwardhan, SIOR, Pune

CONGENITAL PSEUDOARTHROSIS OF TIBIA
ETIOLOGY
• Unknown
• Speculation: Many theories
• Sporadic familial associations
• Approximately 50% associated with NF-1
• Only 2-6% of NF-1 patients have CPT
• Presence of NF-1 does not impact healing rates

HISTOPATHOLOGY
-Focal isolated lesions
-Fibrous hamartoma: fibromatosis like tissue at CPT site
• Highly cellular
• Undifferentiated
• capable of several mesenchymal line differentiation
• Poor vascularity
• Thickened periosteum

  • Nf1 gene implicated in rat model pseudarthrosis
  • Encodes neurofibromin
  • Negative regulator for Ras-GTP to inactive Ras GDP
  • Impaired osteoblast differentiation
  • May be increased osteoclast commitment/differentiation

CLASSIFICATION
Several descriptive classifications exist

Boyd-6 types
Anderson -5 types
Crawford -4 types
El-Rosasy Paley -3 types
Masserman -3 types

No classification provides guidance as to treatment Or predictor of outcome

Early fracture = worse prognosis

Prognosis depends on

  • Presence or absence of fracture
  • Age at which fracture first occurs (Early onset /= 4 years)
  • Severe LLD, poor ankle function, fibular Pseudarthrosis

Treatment Goals

Once Fracture occurs, surgery is required

  • Obtain bony union and Maintain union Rate
  • If treated nonoperatively, ability to unite is only 25-40% by5 Years
  • Maintain functional Alignment
  • Minimize limb Length inequality
  • Preserve joints

Non operative management less Successful

Surgical Management

Principles :
• Resect hamartomatous Tissue
• Resect periosteum
• Resect fibular Pseudarthrosis if Present

Methods :
• Bone to bone contact using IM rodding
• Bone grafting
• Vascularized fibula graft
• Synostosis – bypass graft
• RhBMP-2

IM Stabilization (80-90%)

• Williams rods
• Rush rods
• Telescoping rods
• Flexible rods
• K-wire/Steinman pins
Stabilize fibula and ankle (avoid crossing ankle whenever possible as long term function could be affected)

External fixation

• Stability + lengthening via
• Proximal corticotomy
• Risk of proximal nonunion
• High union rates (60- 100%)
• Late refracture (15-40%)
Combine with IM fixation
Combine with vascularized Fibular graft

Vascularized fibular graft

  • Union rates 95% (recent studies: 60%
  • Technically demanding
  • Contralateral / Ipsilateral Graft
  • Average rate of surgeries to obtain Union: 2. 5 Surgeries per patient, as they need secondary bone graft
  • Can lead to LLD, Angular deformities, Valgus deformity

Amputation

  • Traditionally viewed as “when all else fails”
  • Limited access to child prosthetics
  • Sensate limb superior to Prosthesis
  • Prognosis at first visit
    • Higher initial union rates
    • Better stabilization
    • ?Better biology RhBMP-2

Reality in Limb Preservation:

  • High refracture rates
  • Limited function
    • LLD
    • Residual deformity
    • Joint function
  • Loss of life to save the Limb
  • Excellent prostheses for Children are available nowadays

Indications/risk factors for Amputation

• Anticipated LLD > 5 cm
• Presence of NF
• Failure to achieve union by age 6 years
• >32 months to achieve Union

 

AMPUTATION

  • Syme’s amputation-  retain the tibial stump (even there is nonunion at pseudoarthrosis site)
  • Goal is wearable Prosthesis
  • Nonunions rarely painful

SUMMARY
• CPT is rare/difficult problem
• Little understanding of pathology
• No consensus of management
• Initial reconstructive efforts better
• Unclear if longer term function improved
• Advise families of possibility and consider Amputation early

Post Views: 1,435

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