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 in
equality and a spectrum of musculoskeletal anomalies involving the ipsilate
ral hip, femur, knee, tibia/fibula. ankle, and foot. Considering the freque
ntly associated abnormalities of the lower extremity, the term post axial h
ypoplasia may be more descriptive than the traditional terms fibular hemime
lia or fibular a/hypoplasia. By raising the awareness of associated deformi
ties, the clinician is better prepared to advise patients and to intercede
accordingly. Based on our experience, we advocate a modular treatment appro
ach combining limb lengthening with hemiepiphysiodesis of the distal femur
and/or ankle to corner valgus alignment and establish a neutral mechanical
axis. Contralateral epiphysiodesis as an adjunct may be preferable to doubl
e or repeated lengthening. The goal is to achieve symmetry and stable joint
s at skeletal maturity with a minimal number of well-timed surgical interve
ntions. By using this strategy and with minimal morbidity, 10 of our patien
ts who have reached skeletal maturity have achieved a satisfactory outcome.