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1.  Is Bilateral Lower Limb Lengthening Appropriate for Achondroplasia?: Midterm Analysis of the Complications and Quality of Life 
Background
Use of the Ilizarov technique for limb lengthening in patients with achondroplasia is controversial, with a high risk of complications balancing cosmetic gains. Although several articles have described the complications of this procedure and satisfaction of patients after surgery, it remains unclear whether lengthening improves the quality of life (QOL) of these patients.
Questions/purposes
We asked whether bilateral lower limb lengthenings with deformity correction in patients with achondroplasia would improve QOL and investigated the correlation between complication rate and QOL.
Patients and Methods
We retrospectively reviewed 22 patients (average age, 12.7 years) diagnosed with achondroplasia who underwent bilateral lower limb lengthenings between 2002 and 2005. These patients were compared with 22 patients with achondroplasia for whom limb lengthening was not performed. The two groups were assessed using the American Academy of Orthopaedic Surgeons (AAOS) lower limb, SF-36, and Rosenberg self-esteem scores. Minimum followup was 4.5 years (range, 4.5–6.9 years).
Results
Among the lengthening group, the average gain in length was 10.21 ± 2.39 cm for the femur and 9.13 ± 2.12 cm for the tibia. A total of 123 complications occurred in these 88 segments. The surgical group had higher Rosenberg self-esteem scores than the nonsurgical group although there were no differences in the AAOS and the SF-36 scores. The self-esteem scores decreased with the increase in the number of complications.
Conclusions
Our data suggest that despite frequent complications, bilateral lower limb lengthening increases patients’ QOL. We believe lengthening is a reasonable option in selected patients.
Level of Evidence
Level IV, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.
doi:10.1007/s11999-011-1983-y
PMCID: PMC3254769  PMID: 21785895
2.  Congenital pseudarthrosis of the tibia treated with a combination of Ilizarov’s technique and intramedullary rodding 
Acta Orthopaedica  2012;83(5):515-522.
Background and purpose
Ilizarov’s technique and intramedullary rodding have often been used individually in congenital pseudarthrosis of the tibia. In this series, we attempted to combine the advantages of both methods while minimizing the complications.
Methods
We reviewed 15 cases of congenital pseudoarthrosis of the tibia (CPT) who were treated with a combination of Ilizarov’s apparatus and antegrade intramedullary nailing between 2003 and 2008. The mean age at surgery was 7.5 (3–12) years and the mean limb length discrepancy was 2.5 (1.5–5) cm. At a mean follow-up time of 4.5 (1.6–7.2) years after the index surgery, the patients were evaluated clinically and radiographically for ankle function (AOFAS score) and for malalignment, signs of union, limb length discrepancy, and complications.
Results
14 patients achieved union, in 6 patients primary union and in 8 patients after secondary procedures. The AOFAS score improved from a preoperative mean of 40 (20–57) to 64 (47–75). The main complication was refracture in 1 patient, and non-union in 1 patient.
Interpretation
The combination of the Ilizarov technique and conventional antegrade intramedullary nailing was successful in achieving union with few complications, though this should be shown in long-term studies lasting until skeletal maturity.
doi:10.3109/17453674.2012.736170
PMCID: PMC3488180  PMID: 23043268
3.  Fibula-related complications during bilateral tibial lengthening 
Acta Orthopaedica  2012;83(3):271-275.
Background and purpose
Complications related to the fibula during distraction osteogenesis could cause malalignment. Most published studies have analyzed only migration of the fibula during lengthening, with few studies examining the effects of fibular complications.
Patients and methods
We retrospectively reviewed 120 segments (in 60 patients) between 2002 and 2009. All patients underwent bilateral tibial lengthening of more than 5 cm. The mean follow-up time was 4.9 (2.5–6.9) years.
Results
The average lengthening percentage was 34% (21–65). The ratio of mean fibular length to tibial length was 1.05 (0.91–1.11) preoperatively and 0.83 (0.65–0.95) postoperatively. The mean proximal fibular migration (PFM) was 15 (4–31) mm and mean distal fibular migration (DFM) was 9.7 (0–24) mm. Premature consolidation occurred in 10 segments, nonunion occurred in 12, and angulation of fibula occurred in 8 segments after lengthening. Valgus deformities of the knee occurred in 10 segments.
Interpretation
PFM induced valgus deformity of the knee, and premature consolidation of the fibula was associated with the distal migration of the proximal fibula. These mechanical malalignments could sometimes be serious enough to warrant surgical correction. Thus, during lengthening repeated radiographic examinations of the fibula are necessary to avoid complications.
doi:10.3109/17453674.2012.665328
PMCID: PMC3369154  PMID: 22329670
4.  Physeal growth arrest after tibial lengthening in achondroplasia 
Acta Orthopaedica  2012;83(3):282-287.
Background and purpose
Bilateral tibial lengthening has become one of the standard treatments for upper segment-lower segment disproportion and to improve quality of life in achondroplasia. We determined the effect of tibial lengthening on the tibial physis and compared tibial growth that occurred at the physis with that in non-operated patients with acondroplasia.
Methods
We performed a retrospective analysis of serial radiographs until skeletal maturity in 23 achondroplasia patients who underwent bilateral tibial lengthening before skeletal maturity (lengthening group L) and 12 achondroplasia patients of similar height and age who did not undergo tibial lengthening (control group C). The mean amount of lengthening of tibia in group L was 9.2 cm (lengthening percentage: 60%) and the mean age at the time of lengthening was 8.2 years. The mean duration of follow-up was 9.8 years.
Results
Skeletal maturity (fusion of physis) occurred at 15.2 years in group L and at 16.0 years in group C. The actual length of tibia (without distraction) at skeletal maturity was 238 mm in group L and 277 mm in group C (p = 0.03). The mean growth rates showed a decrease in group L relative to group C from about 2 years after surgery. Physeal closure was most pronounced on the anterolateral proximal tibial physis, with relative preservation of the distal physis.
Interpretation
Our findings indicate that physeal growth rate can be disturbed after tibial lengthening in achondroplasia, and a close watch should be kept for such an occurrence—especially when lengthening of more than 50% is attempted.
doi:10.3109/17453674.2012.678802
PMCID: PMC3369156  PMID: 22489887

Results 1-4 (4)