Case 30: Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 2)

Abstract

Congenital pseudarthrosis of the tibia (CPT) is defined as a nonunion of a tibial fracture that develops spontaneously or after trivial trauma in a dysplastic bone segment of the tibial diaphysis. The condition is associated with NF-1 in over 50 % of cases; however, no neurofibromatosis tissue has been found at the CPT site. Pathologically, a fibrous hamartoma surrounds the bone at the CPT site. Histobiochemical studies have revealed increased osteoclastic activity in the periosteum surrounding the CPT. CPT is both a biological and mechanical problem; the surgical treatment should address both aspects of the condition. The recommended protocol of treatment includes complete excision of the pathological periosteum, insertion of autogenous iliac crest bone graft (ICBG), and combined fixation using intramedullary rod (IMR) and Ilizarov external fixator . Leg length discrepancy, due to tibial shortening, in a walking child has been noticed to induce femoral overgrowth which compensates for the discrepancy and may obviate the need for tibial lengthening in young children during treatment of tibial shortening.