This study evaluated the effect of limb lengthening
on longitudinal growth in patients with achondroplasia. Growth of
the lower extremity was assessed retrospectively by serial radiographs
in 35 skeletally immature patients with achondroplasia who underwent
bilateral limb lengthening (Group 1), and in 12 skeletally immature
patients with achondroplasia who did not (Group 2). In Group 1,
23 patients underwent only tibial lengthening (Group 1a) and 12 patients
underwent tibial and femoral lengthening sequentially (Group 1b). The mean lengthening in the tibia was 9.2 cm (59.5%) in Group
1a, and 9.0 cm (58.2%) in the tibia and 10.2 cm (54.3%) in the femur
in Group 1b. The mean follow-up was 9.3 years (8.6 to 10.3). The
final mean total length of lower extremity in Group 1a was 526.6
mm (501.3 to 552.9) at the time of skeletal maturity and 610.1 mm
(577.6 to 638.6) in Group 1b, compared with 457.0 mm (411.7 to 502.3)
in Group 2. However, the mean actual length, representing the length
solely grown from the physis without the length of distraction,
showed that there was a significant disturbance of growth after
limb lengthening. In Group 1a, a mean decrease of 22.4 mm (21.3
to 23.1) (4.9%) was observed in the actual limb length when compared
with Group 2, and a greater mean decrease of 38.9 mm (37.2 to 40.8)
(8.5%) was observed in Group 1b when compared with Group 2 at skeletal
maturity. In Group 1, the mean actual limb length was 16.5 mm (15.8 to
17.2) (3.6%) shorter in Group 1b when compared with Group 1a at
the time of skeletal maturity. Premature physeal closure was seen
mostly in the proximal tibia and the distal femur with relative
preservation of proximal femur and distal tibia. We suggest that significant disturbance of growth can occur after
extensive limb lengthening in patients with achondroplasia, and
therefore, this should be included in pre-operative counselling
of these patients and their parents.
Lengthening of the humerus is now an established
technique. We compared the complications of humeral lengthening
with those of femoral lengthening and investigated whether or not
the callus formation in the humerus proceeds at a higher rate than
that in the femur. A total of 24 humeral and 24 femoral lengthenings
were performed on 12 patients with achondroplasia. We measured the
pixel value ratio (PVR) of the lengthened area on radiographs and
each radiograph was analysed for the shape, type and density of
the callus. The quality of life (QOL) of the patients after humeral
lengthening was compared with that prior to surgery. The complication
rate per segment of humerus and femur was 0.87% and 1.37%, respectively.
In the humerus the PVR was significantly higher than that of the
femur. Lower limbs were associated with an increased incidence of
concave, lateral and central callus shapes. Humeral lengthening
had a lower complication rate than lower-limb lengthening, and QOL
increased significantly after humeral lengthening. Callus formation
in the humerus during the distraction period proceeded at a significantly
higher rate than that in the femur. These findings indicate that humeral lengthening has an important
role in the management of patients with achondroplasia.