Postaxial hypoplasia of the lower extremity.
Congenital deficiency of the fibula may present a variety of patterns. In a series of 20 patients with this diagnosis, we have observed limb-length inequality and a spectrum of musculoskeletal anomalies involving the ipsilateral hip, femur, knee, tibia/fibula, ankle, and foot. Considering the frequently associated abnormalities of the lower extremity, the term postaxial hypoplasia may be more descriptive than the traditional terms fibular hemimelia or fibular a/hypoplasia. By raising the awareness of associated deformities, the clinician is better prepared to advise patients and to intercede accordingly. Based on our experience, we advocate a modular treatment approach combining limb lengthening with hemiepiphysiodesis of the distal femur and/or ankle to correct valgus alignment and establish a neutral mechanical axis. Contralateral epiphysiodesis as an adjunct may be preferable to double or repeated lengthening. The goal is to achieve symmetry and stable joints at skeletal maturity with a minimal number of well-timed surgical interventions. By using this strategy and with minimal morbidity, 10 of our patients who have reached skeletal maturity have achieved a satisfactory outcome.
Stevens
P M
PM
University of Utah, Salt Lake City, USA.
Arms
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D
D000293:Adolescent; D000328:Adult; D001852:Bone Lengthening / Q000379:methods; D002648:Child; D002675:Child, Preschool; D004480:Ectromelia / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery*; D005260:Female; D005360:Fibula / Q000002:abnormalities* / Q000601:surgery; D005532:Foot Deformities, Congenital / Q000000981:diagnostic imaging* / Q000601:surgery*; D006621:Hip Joint; D006801:Humans; D007223:Infant; D007593:Joint Instability / Q000000981:diagnostic imaging / Q000209:etiology / Q000601:surgery; D007719:Knee Joint; D007870:Leg Length Inequality / Q000209:etiology / Q000601:surgery*; D008297:Male; D019637:Orthopedic Procedures / Q000379:methods; D011379:Prognosis; D011859:Radiography; D009626:Terminology as Topic