Approaches to treating NF1 tibial pseudarthrosis: consensus from the Children's Tumor Foundation NF1 Bone Abnormalities Consortium.

Abstract

BACKGROUND Neurofibromatosis 1 (NF1) is an autosomal dominant disorder with various skeletal abnormalities occurring as part of a complex phenotype. Tibial dysplasia, which typically presents as anterolateral bowing of the leg with subsequent fracture and nonunion (pseudarthrosis), is a serious but infrequent osseous manifestation of NF1. Over the past several years, results from clinical and experimental studies have advanced our knowledge of the role of NF1 in bone. On the basis of current knowledge, we propose a number of concepts to consider as a theoretical approach to the optimal management of tibial pseudarthrosis. METHODS A literature review for both clinical treatment and preclinical models for tibial dysplasia in NF1 was performed. Concepts were discussed and developed by experts who participated in the Children's Tumor Foundation sponsored International Bone Abnormalities Consortium meeting in 2011. RESULTS Concepts for a theoretical approach to treating tibial pseudarthrosis include: bone fixation appropriate to achieve stability in any given case; debridement of the "fibrous pseudarthrosis tissue" between the bone segments associated with the pseudarthrosis; creating a healthy vascular bed for bone repair; promoting osteogenesis; controlling overactive bone resorption (catabolism); prevention of recurrence of the "fibrous pseudarthrosis tissue"; and achievement of long-term bone health to prevent recurrence. CONCLUSIONS Clinical trials are needed to assess effectiveness of the wide variation of surgical and pharmacologic approaches currently in practice for the treatment of tibial pseudarthrosis in NF1. LEVEL OF EVIDENCE Level V, expert opinion.

DOI: 10.1097/BPO.0b013e31828121b8
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@article{Stevenson2013ApproachesTT, title={Approaches to treating NF1 tibial pseudarthrosis: consensus from the Children's Tumor Foundation NF1 Bone Abnormalities Consortium.}, author={David A. Stevenson and David Graham Little and Linlea Armstrong and Alvin H. Crawford and Deborah M. Eastwood and J. M. Friedman and Tiziana Greggi and Gloria E. Gutierrez and Kim Hunter-Schaedle and David L. Kendler and Mateusz Kolanczyk and Fergal P. Monsell and Matthew Oetgen and Benjamin Stephens Richards and Aaron Schindeler and Elizabeth K. Schorry and David Wilkes and David Viskochil and Fengchun Yang and Florent Elefteriou}, journal={Journal of pediatric orthopedics}, year={2013}, volume={33 3}, pages={269-75} }