Paraxial Tibial Hemimelia
- 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;
- centralization of fibula (unpredictable)
- patients w/ a functional knee joint and quadriceps mechanism may function best with a Syme or BKA amputation (see pediatric
- 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.
Original Text by Clifford R. Wheeless, III, MD.
Last updated by Data Trace Staff on Thursday, May 31, 2012 3:18 pm