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Tibial Hemimelia

Tibial hemimelia
Objectives
  1. Define tibial hemimelia
  2. Describe the spectrum of deformity seen within patients with tibial hemimelia
  3. Describe current treatment approaches for tibial hemimelia

Discussion

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.

References

  1. Turker R, Mendelson S, Ackman J, Lubicky JP. Anatomic considerations of the foot and leg in tibial hemimelia. Journal of Pediatric Orthopedics 1996;16(4):445-9.
  2. Simmons ED, Jr., Ginsburg GM, Hall JE. Brown's procedure for congenital absence of the tibia revisited. Journal of Pediatric Orthopedics 1996;16(1):85-9.
  3. Laor T, Jaramillo D, Hoffer FA, Kasser JR. MR imaging in congenital lower limb deformities. Pediatric Radiology 1996;26(6):381-7.
  4. Grissom LE, Harcke HT, Kumar SJ. Sonography in the management of tibial hemimelia. Clinical Orthopaedics & Related Research 1990(251):266-70.
  5. Loder RT, Herring JA. Fibular transfer for congenital absence of the tibia: a reassessment. Journal of Pediatric Orthopedics 1987;7(1):8-13.
  6. Jones D, Barnes J, Lloyd-Roberts GC. Congenital aplasia and dysplasia of the tibia with intact fibula. J Bone Joint Surg (Br) 1978;60:31-9.
  7. Schoenecker PL, Capelli AM, Millar EA, al. e. Congenital longitudinal deficiency of the tibia. J Bone Joint Surg (Am) 1989;71:278-87.
  8. Sedgwick WG, Schoenecker PL. Congenital diastasis of the ankle joint: Case report of a patient treated and followed to skeletal maturity. J Bone Joint Surg (Am) 1982;71:450-53.
  9. Herring JA, Birch JG. The child with a limb deficiency. Rosemont, Il: American Academy of Orthopaedic Surgeons; 1998.
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