Tibial hemimelia and its complex presentation-a case series
|IJOCP | Volume – 2015| Issue – 2015| Article ID – 2015:83
Conference: Indian Orthopaedic Association Annual Conference IOACON-2013, India [Click for Full details]
Authors: Natarajan N, Pasupathy A
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Introduction: Complex congenital anomalies of the lower extremities include tibial hemimelia with ipsilateral femoral bifurcation & diastasis of distal tibiofibular joint with central ray deficiency of the hand called as gallop wolfgang syndrome. Congenital absence of tibia alone is a rare anomaly & may be partial or total, unilateral or bilateral. total absence is more frequent than partial. Family history is usually negative for congenital abnormalities & other diseases.
Materials: We did this study in our institute from Jan 2011 to May 2013,a total of 8 cases of which 3 cases are gallop wolfgang, 4 cases of unilateral tibial hemimelia & a case of bilateral presentation. Methods: In this classification and score the main pathological findings of the complete leg are included (coxa-femur-patella-tibia-fibula-pes), with specific importance of the tibia. tibial malformations are divided into seven main groups and five of them into two subgroups. The cartilaginous anlage of the tibia which has not yet received much attention in the literature plays an important role in this classification. Only seven main types of tibial defect have to be recognized and, if necessary, additional defects of the whole leg, including the function of the affected parts, can be summarized in a-five-class-system.
Discussion: If the entire tibia is absent, there is often a fixed proximal and lateral position of the fibula, with severe flexion deformity. Knee disarticulation is generally preferred for this condition, although centralization of the fibula (Brown procedure) combined with Symes amputation has been described for this situation. In the absence of good quadriceps function, which is uncommon with total tibial hemimelia, longterm results of the Brown procedure have been unpromising. When the proximal tibia is present, it can be fused to the fibula with a Symes amputation, and a very reasonable functional limb is achieved.
Conclusion: Treatment mainly includes surgical correction of the deformity where possible. Common surgical procedures include disarticulation at Knee, Syme’s amputation or Chopart amputation. The sooner the amputation is performed the easier and faster the rehabilitation and adaptation to the prosthesis. The absence of the cartilaginous anlage increases the difficulty of the operative procedure. The surgical option for the index case with favorable result would include disarticulation of the knee joint and prosthesis. The surgical option for the index case with favorable result would include disarticulation of the knee joint and prosthesis.
|How to Cite this Abstract|
|Natarajan N, Pasupathy A . Tibial hemimelia and its complex presentation-a case series. International Journal of Conference Proceedings 2015;(2015):83|