RECONSTRUCTION OF BILATERAL TIBIAL APLASIA AND SPLIT HAND-FOOT SYNDROME IN A FATHER AND DAUGHTER

Reconstruction of bilateral tibial aplasia and split hand-foot syndrome in a father and daughter

Reconstruction of bilateral tibial aplasia and split hand-foot syndrome in a father and daughter

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Background: Tibial aplasia is of heterogeneous aetiology, the majority of reports are sporadic.We describe the reconstruction procedures in two subjects - a daughter and father manifested autosomal dominant (AD) inheritance of the bilateral tibial aplasia and split hand-foot syndrome.Materials and Methods: Reconstruction of these patients required multiple surgical procedures and orthoprosthesis was mandatory.The main goal of treatment was to achieve walking.

Stabilization of the ankle joint by fibular-talar-chondrodesis on both sides, followed tenga flip orb by bilateral Brown-procedure at the knee joint level has been applied accordingly.Results: The click here outcome was with improved function of the deformed limbs and walking was achieved with simultaneous designation of orthotic fitting.Conclusion: This is the first study encompassing the diagnosis and management of a father and daughter with bilateral tibial aplasia associated with variable split hand/foot deformity without foot ablation.Our patients showed the typical AD pattern of inheritance of split-hand/foot and tibial aplasia.

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