CONGENITAL TIBIAL DYSPLASIA (CONGENITAL PSEUDOARTHROSIS OF THE TIBIA): AN ATYPICAL VARIATION.

Abstract

Purpose: The purpose of the study was to identify those characteristics of congenital tibial dysplasia (CTD) that portend the worst prognosis, including the probable failure of all surgical attempts to achieve union. Clear identification/ classification of this select population of cases could add earlier consideration of relevant treatment options.

Method: While several classification systems for CTD exist, the Crawford classification was used to review the literature and our hospital cases (6) to examine the relationship between case characteristics, treatment, and outcome.

Results: An atypical variation of Crawford’s type IIC was identified, herein referred to as type IID, that was characterized by early onset, frank pseudoarthrosis, and deformity of the distal-one third of the tibia and fibula. As well, these patients underwent failed multiple surgeries and numerous type treatments over time without good functional outcome and with prolonged physical difficulties.

Conclusion: The cases that fall into this newly defined category (IID) of the Crawford classification for CTD are best treated with early amputation rather than repeated attempts to gain union.

Significance: Amputation for patients with congenital tibial dysplasia (congenital pseudoarthrosis of the tibia) that are identified as type IID cases and carry the characteristics for a bad prognosis, provides better functional results than repeated surgeries, even when union is achieved. The family should be warned from the beginning that a stable union is unlikely in these cases. Amputation should be offered as a primary or an early option to the family with full disclosure of the long-term advantages and disadvantages of all therapeutic paths.