A tale of two tibias: A review of treatment options for congenital pseudarthrosis of the tibia

Abstract

Purpose Two common treatment options for congenital pseudarthrosis of the tibia (CPT) are intramedullary fixation following resection/shortening of the pseudarthrosis site and reconstruction with an Ilizarov external fixator following resection. We present in detail a narrative of two cases with similar degrees of tibial dysplasia associated with NF-1 treated using these different methods and followed to completion. Methods Technical issues and details of the treatment methods from case reports are discussed in depth. The eventual profoundly different outcomes are correlated to the technical variations used. Results Treatment with the Charnley–Williams rodding method and aggressive bone grafting supplemented by rh-BMP2 resulted in a normal functioning limb at maturity, while treatment with first, an ineffective version of IM rodding, followed by two sessions of bone transport using an Ilizarov fixator failed to gain useful union and eventually resulted in amputation. Conclusions Technical details, heretofore inadequately reviewed in the literature, are crucial to the success of either of these commonly utilized treatment methods for CPT.

Periosteal Grafting for Congenital Pseudarthrosis of the Tibia: A Preliminary Report

“…In a review of CPT, Johnston and Birch [22] advocated using BMP as an adjuvant treatment in all primary and recalcitrant cases. Despite optimism with the use of BMP, one must also consider theoretical risk of tumorigenesis because BMP stimulates the RAS pathway, which is also a tumor pathway.…”

New Insights into Osteogenic and Chondrogenic Differentiation of Human Bone Marrow Mesenchymal Stem Cells and Their Potential Clinical Applications for Bone Regeneration in Pediatric Orthopaedics

“…Conservative treatment includes bracing of the affected limb continued until the end of skeletal growth. Surgical treatment includes several procedures, which are resection of the nonunion and reconstruction with either autologous nonvascularized bone grafting and intramedullary fixation or one-stage shortening followed by an Ilizarov external fixator to perform lengthening [99, 100]. All these procedures have frequently unsatisfactory results because of the need for several operations for recalcitrant nonunion, residual deformity, and limb-length discrepancies [101].…”

Ilizarov Treatment of Congenital Pseudarthrosis of the Tibia: A Multi-Targeted Approach Using the Ilizarov Technique

“…Although the primary aim of treatment is union of the pseudarthrosis site, the therapeutic considerations should include other associated problems, such as leg-length discrepancy, multilevel and multidirectional tibial deformity, foot deformity, associated fibular pseudarthrosis and subsequent ankle valgus 22,24,25). According to the literatures produced by retrospective case series, the overall union rate for the Ilizarov technique ranges from 60 to 100% 20).…”