Ankle and foot deformities and malformations in proximal femoral focal deficiency
Status PubMed-not-MEDLINE Language English Country Great Britain, England Media electronic-ecollection
Document type Journal Article
PubMed
39713180
PubMed Central
PMC11656460
DOI
10.1177/18632521241301942
PII: 10.1177_18632521241301942
Knihovny.cz E-resources
- Keywords
- Foot, fibula deficiency, lateral ray deficiency, proximal femoral focal deficiency, subtalar synostosis,
- Publication type
- Journal Article MeSH
PURPOSE: To describe foot abnormalities in proximal femoral focal deficiency and their correlation to the severity. METHODS: Eighty-nine extremities in 87 patients were evaluated between 1996 and 2020 clinically and radiologically. Fibula length, ankle shape, tarsal coalitions, and the number of foot rays were recorded. Extremities with proximal femoral focal deficiency were classified according to Pappas and divided into severe (classes II and V), medium severe (classes III and IV), and mild groups (classes VII, VIII, and IX). RESULTS: The fibula was short in 89% and absent in 11% of cases. An absent fibula occurred mostly in severe class III and only in 4% of mild grades (statistically significant, p = 0.004). The valgus ankle joint prevailed in 82% of cases. Spherical ankle joints (18% of cases) were associated in all cases with a tarsal coalition. Tarsal coalitions occurred in 14.6% and were present in all classes except class IV. Five ray feet were found in 83% of cases, four ray feet were found in 16%, and three ray feet in one extremity. Reduction in the number of foot rays occurred more commonly in association with fibular aplasia (30%). CONCLUSIONS: Abnormalities of the fibula and ankle joint represent a constant part of proximal femoral focal deficiency, whereas tarsal coalition and a reduction of foot rays do not. The severity of foot abnormalities does not correlate to the severity of proximal femoral focal deficiency but does with fibular aplasia.
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Hillmann JS, Mesgarzadeh M, Revesz G, et al.. Proximal femoral focal deficiency: radiologic analysis of 49 cases. Radiology 1987; 165: 769–773. PubMed
Manner HM, Radler C, Ganger R, et al.. Dysplasia of the cruciate ligaments: radiographic assessment and classification. J Bone Joint Surg Am 2006; 88: 130–137. PubMed
Chomiak J, Podskubka A, Dungl P, et al.. Cruciate ligaments in proximal femoral focal deficiency: arthroscopic assessment. J Pediatr Orthop 2012; 32(1): 21–28. PubMed
Grogan DP, Holt GR, Ogden JA. Talocalcaneal coalition in patients who have fibular hemimelia or proximal femoral focal deficiency. a comparison of the radiographic and pathological findings. J Bone Joint Surg Am 1994; 76: 1363–1370. PubMed
Stanitski DF, Stanitski CL. Fibular hemimelia: a new classification system. J Pediatr Orthop 2003; 23(1): 30–34. PubMed
Paley D. Surgical reconstruction for fibular hemimelia. J Child Orthop 2016; 10(6): 557–83. PubMed PMC
Zhang Z, Yi D, Xie R, et al.. Postaxial limb hypoplasia (PALH): the classification, clinical features, and related developmental biology. Ann N Y Acad Sci 2017; 1409: 67–78. PubMed PMC
Bronfen C, Rigault P, Padovani JP, et al.. Foot deformities in longitudinal ectromelia of the lower limbs. Int Orthop 1994; 18: 139–149. PubMed
Sorge G, Ardito S, Genuardi M, et al.. Proximal femoral focal deficiency (PFFD) and fibular A/Hypoplasia (FA/H): a Model of a developmental Field defect. Am J Med Genetics 1995; 55: 427–432. PubMed
Pappas AM. Congenital abnormalities of the femur and related lower extremity malformations. J Pediatr Orthop 1983; 3: 45–60. PubMed
Paley D. Principles of deformity corrections. Springer-Verlag Berlin Heidelberg New York, 2005
Koman LA, Meyer LC, Warren FH. Proximal femoral focal deficiency: natural history and treatment. Clin Orthop Relat Res 1982; 162: 135–143. PubMed
Stevens PM, Arms D. Postaxial hypoplasia of the lower extremity. J Pediatr Orthop 2000; 20: 166–172. PubMed
Hootnick DR, Vargesson N. The syndrome of proximal femur, fibula, and midline metatarsal long bone deficiencies. Birth Defects Res 2018; 110(15): 1188–1193. PubMed
Reyes BA, Birch JG, Hootnick DR. et al.. The nature of foot ray deficiency in congenital fibular deficiency. J Pediatr Orthop. 2017; 37:332–337. PubMed