Aims

Osteofibrous dysplasia (OFD) is a rare benign lesion predominantly affecting the tibia in children. Its potential link to adamantinoma has influenced management. This international case series reviews the presentation of OFD and management approaches to improve our understanding of OFD.

In 1937 Blount described a series of 28 patients with ‘Tibia vara’. Since then, a number of deformities in the tibia and the femur have been described in association with this condition.

We analysed 14 children with Blount’s disease who were entered into a cross-sectional study. Their mean age was 10 (2 to 18). They underwent a clinical assessment of the rotational profile of their legs and a CT assessment of the angle of anteversion of their hips (femoral version). We compared our results to previously published controls. A statistically significant increase in femoral anteversion was noted in the affected legs, with on average the femurs in patients with Blount’s disease being 26° more anteverted than those in previously published controls.

We believe this to be a previously unrecognised component of Blount’s disease, and that the marked intoeing seen in the disease may be partly caused by internal femoral version, in addition to the well-recognised internal tibial version.

The Ilizarov method for leg lengthening was used for cosmetic reasons in 54 patients with constitutional short stature. A mean lengthening of 7 cm with a low rate of complications produced an excellent or good outcome in all the patients, including improvement in psychological disturbances related to short stature. Those who undergo the procedure must be highly motivated, fully informed and understand the technique and possible complications. We suggest that the Ilizarov method for cosmetic limb lengthening is a technique without major complications. However, it requires careful follow-up, and the involvement of orthopaedic surgeons who are familiar with use of the circular frame and are experienced in limb lengthening and correction of deformity for pathological conditions.

We reviewed 34 knees in 24 children after a double-elevating osteotomy for late-presenting infantile Blount’s disease. The mean age of patients was 9.1 years (7 to 13.5).

All knees were in Langenskiöld stages IV to VI. The operative technique corrected the depression of the medial joint line by an elevating osteotomy, and the remaining tibial varus and internal torsion by an osteotomy just below the apophysis. In the more recent patients (19 knees), a proximal lateral tibial epiphysiodesis was performed at the same time.

The mean pre-operative angle of depression of the medial tibial plateau of 49° (40° to 60°) was corrected to a mean of 26° (20° to 30°), which was maintained at follow-up. The femoral deformity was too small to warrant femoral osteotomy in any of our patients. The mean pre-operative mechanical varus of 30.6° (14° to 66°) was corrected to 0° to 5° of mechanical valgus in 29 knees. In five knees, there was an undercorrection of 2° to 5° of mechanical varus. At follow-up a further eight knees, in which lateral epiphysiodesis was delayed beyond five months, developed recurrent tibial varus associated with fusion of the medial proximal tibial physis.

Congenital unilateral anterolateral tibial bowing in combination with a bifid ipsilateral great toe is a very rare deformity which resembles the anterolateral tibial bowing that occurs in association with congenital pseudarthrosis of the tibia. However, spontaneous resolution of the deformity without operative treatment and with a continuously straight fibula has been described in all previously reported cases. We report three additional cases and discuss the options for treatment. We suggest that this is a specific entity within the field of anterolateral bowing of the tibia and conclude that it has a much better prognosis than congenital pseudarthrosis of the tibia, although conservative treatment alone may not be sufficient.