It is a retrospective case series study at one institution by two of the presenting authors as senior surgeons. Between years 1988 and 2001, ten patients were referred to our Department and received treatment for fibular hemimelia. Three patients excluded from the study as they did not met the criteria of a complete follow-up. Finally, seven patients presenting fibular hemimelia in eight fibulae included in the present study. Three patients were male and four were female. Mean age on first examination was 4 years and 2.4 months old (ranged from three days to nine years old).
Details of the patients and types of treatment are presented in Additional File 1, Table S1. No known syndromes were detected. Six of seven patients were treated surgically presenting average leg length discrepancy (LLD) of 5.3 cm (ranged 2.9 to 9.0 cm) preoperatively. The average projected LLD at skeletal maturity was evaluated to be 9.1 cm, combining all the available methods: the Green-Anderson charts and the Menelaus method in all patients, where as the Moseley graph was used selectively in three patients (patients 1, 2 and 3) [Additional file 1, table s1] who were followed for more than four years before surgical treatment [7–9]. All patients underwent CT topograms on first presence followed by repeated measurements in annual basis for accurate evaluation of current and projected LLD. In all patients with LLD there was even minimal contribution of femur hypoplasia to leg length inequality, as it was measured using CT topograms.
Fifteen totally procedures included in the initial treatment plan: five lengthening procedures of the tibia, two of the femur, four of the Achilles, two of the peroneal tendons, one corrective osteotomy of the tibia, one corrective osteotomy and arthrodesis of the tarsus [Additional file 1, table s1]. All patients received primary lengthening procedures in our department by two of the presenting authors expertised in this field (A.E.B and P.N.S.). LLD of more than 2.5 cm was initially planned to be treated by lengthening procedures. Average preoperative follow up was 3 years and 8 months (1 to 7 years).
LLD was treated by lengthening of the tibia alone in three patients (cases 1, 4, 6). Lengthening of both the tibia and femur in two different sequential time points was required in two patients (cases 2, 7). Average age on first lengthening procedure was 7.6 years (ranged 3 to 15). Average pre-lengthening follow-up to our center was 6.6 years (ranged 2 to 10). Monolateral fixator devices were used in all primary lengthening procedures. Lengthening of the tibia or of the femur was canceled during growth spur periods to avoid unpredictable results. Lengthening rate was 1 mm per day. Radiographic examination was routinely followed until the end of consolidation phase.
Leg length was evaluated postoperatively using CT topograms on 12 months intervals. Last measurement of leg length was made in the mean age of 15 years old (13 to 20) in four of the five patients who underwent lengthening procedures, depending on clinical and radiographic findings of the rate of LLD reoccurrence and the age of skeletal maturity of each patient. Last topogram was made in exception in patient 4 [Additional file 1, table s1], [Additional file 2, table s2] in the age of 8 years old, as there was no need for further evaluation of leg length beyond this age.
Additional lengthening of the tibia as a revision procedure was needed in patients 4, 6 and 7, additional lengthening of the femur in patient 7, two revision corrective osteotomies of the tibia in patient 6 and a revision achilles lengthening procedure in patient 6. Totally seven revision procedures were needed, with two totally lengthening procedures of the tibia in patient 4, two lengthening procedures of the Achilles, two lengthening procedures and three corrective osteotomies of the tibia in patient 6 and two lengthening procedures of the femur and of the tibia in patient 7. Circular fixator devices were used in all instances of additional lengthenings and osteotomies of the involved bones. All revision procedures were done in our Department by the two senior surgeons already mentioned. Average post-treatment follow up was 9.7 years (1 to 18 years). Functional outcome is shown in details [Additional file 3, table s3], and was evaluated according to Lower Extremity Functional Scale (LEFS) .