- Define tibial hemimelia
- Describe the spectrum of deformity seen within patients with tibial hemimelia
- Describe current treatment approaches for tibial hemimelia
Some forms of tibial hemimelia are genetically transmitted; others are
sporadic. Tibial hemimelia is a preaxial longitudinal deficiency with
variable degrees of absence of the tibia. Jones has described a
classification, type I being total absence of the tibia, type II
demonstrating a proximal tibia, type III (rare) denotes the presence of
a distal tibia only, and in type IV, a divergence of the distal tibia
and fibula, with proximal displacement of the talus. It may be difficult
at birth to tell whether there is any proximal tibia present, ultrasound
can be helpful in this case. 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. For the distal divergence, Symes amputation has most often
been performed as considerable limb length discrepancy often accompanies
this condition. There have been reported cases of successful open
reductions of the ankle and lengthening for type IV deficiencies;
treatment is essentially on an individual basis.
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- Herring JA, Birch JG. The child with a limb deficiency. Rosemont, Il: American Academy of Orthopaedic Surgeons; 1998.