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Paraxial Tibial Hemimelia



- Discussion:
    - distal end of femur is hypoplastic, tibia is absent, & proximal dislocation of fibular head;
    - incidence is 1 in 1 million.
    - has a familial inheritance;
    - tibial hemimelia may be terminal or intercalary, complete or incomplete.
    - 30% percent of cases are bilateral;

- Clinical Presentation:
    - extremity is shortened, w/ foot in rigid supinated position;
    - knee joint may be unstable and contracted in flexion (it is important to determine whetherthe knee joint and the quadriceps are 
           functional enough to use a BKA or Syme prosthesis);
    - limb length inequality;

- Associated Anomalies:
    - approx 75% of all pts have associated skeletal anomalies;
    - DDH occurs in 20%;
    - lobster hand is also common;
    - knee has severe flexion contracture & instability;
    - foot has fixed equinovarus deformity w/ absence of one or more medial rays;

- Treatment:
    - centralization of fibula (unpredictable)
    - patients w/ a functional knee joint and quadriceps mechanism may function best with a Syme or BKA amputation (see pediatric 
           amputations);
           - consider synostosis of the tibia to the fibula to prevent overgrowth;
    - patients w/ a significant knee joint contracture may require knee disarticulation

- Radiographs: Case Examples:

         

         

         

         



Congenital longitudinal deficiency of the tibia.

Failure of centralization of the fibula for congenital longitudinal deficiency of the tibia.

Fibular transfer for congenital absence of the tibia: A reassessment.

Congenital aplasia and dysplasia of the tibia with intact fibula. Classification and management.

Tibial agenesis.