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The anterior cruciate ligament is most likely deficient in patients with which of the following abnormalities?
Congenital radial head dislocation
Proximal focal femoral deficiency
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Proximal focal femoral deficiency (PFFD) is a developmental disorder that is present at birth and caused by a defect in the cartilage anlage of the proximal femur. It is associated with deficiency in development of the proximal femur, cervical pseudoarthrosis, fibular hemimelia, coxa vara, and ACL deficiency.
Westberry and Davids review the diagnosis, management, and various controversies regarding PFFD. While treatment options in PFFD are numerous, strategies should be used which maximize patient function by consideration of hip stability and projected limb length difference.
Manner et al performed a radiographic analysis on 34 knees in patients with longitudinal congenital deficiency of the lower limb to assess the relation to the cruciate ligaments. They found three distinct types of cruciate ligament deficiency, observed on tunnel view radiographs and suggest the diagnosis/differentiation between absence and aplasia can be made on radiographs alone.
1) Cleidocranial dysplasia - caused by mutation in the CBFA1 gene (aka Runx2) with associated cranial abnormalities and absent development of the clavicle.
2) Congenital radial head dislocation - not associated with ACL deficiency.
3) Apert syndrome - caused by mutation in the FGFR2 gene with associated facial and skeletal dysplasias, but no association with ACL deficiency.
4) Achondroplasia - caused by mutation in the FGFR3 gene and not associated with ACL deficiency.
Westberry DE, Davids JR
Hip Int. 2009 Jan-Mar;19 Suppl 6:S18-25. PMID: 19306244 (Link to Abstract)
Manner HM, Radler C, Ganger R, Grill F
J Bone Joint Surg Am. 2006 Jan;88(1):130-7. PMID: 16391258 (Link to Abstract)
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HPI - 2 yo child with PFFD right leg and no femoral head and dysplastic acetabulum.
How would you classify this problem?