1a: No proximal tibia visible on radiograph. Extensor mech absent. Hypoplastic distal femoral epiphysis
1b- Proximal tibia eventually ossifies and extensor mechanism will function. Distal femoral epiphysis appears normal
2- Proximal tibia present at birth but short tibia
3- diaphyseal and distal tibia present but proximal tibia absent
4- short tibia, fibula migrated proximal, diastasis of distal tib-fib joint
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A 13-month-old boy has shortening and deformity of the right leg. A clinical photograph and radiograph are provided in Figures A and B. Which of the following findings are indicative of a good outcome with a tibiotalar Syme amputation?
35 degree flexion contracture
Absence of tibiofibular synostosis
Full functional quadriceps strength
Family history of tibial hemimelia
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The clinical scenario and images demonstrate tibial hemimelia. An amputation below the knee has greater likelihood of success if there is a functional quadriceps and no flexion contracture of the knee. Otherwise, a knee disarticulation may be necessary.
Kalamchi et al review 24 cases of tibial hemimelia to establish a classification scheme. Type I is total absence of the tibia. Type II is distal absence of the tibia. Type III is distal deficiency with tibiofibular diastasis. Assessment of the quadriceps function and degree of flexion contracture is crucial to following the appropriate treatment option.
Kalamchi A, Dawe RV
J Bone Joint Surg Br. 1985 Aug;67(4):581-4. PMID: 4030854 (Link to Abstract)
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HPI - Deformity