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Congenital Pseudoarthrosis of the Tibia

 
  • the most common cause of anterolateral bowing.
  • It is often accompanied by neurofibromatosis (50%-but only 10% of patients with neurofibromatosis have this disorder). NF1 patients commonly have axillary and groin freckling, multiple café-au-lait spots, optic gliomas, and Lisch nodules.
  • Classification (Boyd) is based on bowing and the presence of cystic changes, sclerosis, or dysplasia and cystic changes are most common.
  • Neurofibromatosis and congenital pseudoarthrosis are associated with anterolateral bowing of the tibia, not posteromedial bowing of the tibia.  Posteromedial bowing of the tibia is associated with calcaneal valgus deformity of the foot and is thought to be related to intrauterine positioning. 

Tibial Pseudoarthrosis Treatment

  • total contact brace to protect from fractures
  • intramedullary fixation with excision of hamartomatous tissue, and autogenous bone grafting (osteosynthesis) for nonhealing fractures.
  • Vascularized fibular graft or Ilizarov methods should be considered if bracing fails.
  • Osteotomies and electrical stimulation alone are contraindicated.
  • Amputation (Syme's) and prosthetic fitting are indicated after two or three failed surgical attempts.

Review References

  • Murray HH, CORR 1982;166:14
  • Crossett LS, CORR 1989;245:16

 

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