Yonsei Med J.  2015 Nov;56(6):1656-1662. 10.3349/ymj.2015.56.6.1656.

Limb Lengthening in Patients with Achondroplasia

• Park KW
• Garcia RA
• Rejuso CA
• Choi JW
• Song HR

• KMID: 2345897
• DOI: http://doi.org/10.3349/ymj.2015.56.6.1656

Abstract

PURPOSE
Although bilateral lower-limb lengthening has been performed on patients with achondroplasia, the outcomes for the tibia and femur in terms of radiographic parameters, clinical results, and complications have not been compared with each other. We proposed 1) to compare the radiological outcomes of femoral and tibial lengthening and 2) to investigate the differences of complications related to lengthening.
MATERIALS AND METHODS
We retrospectively reviewed 28 patients (average age, 14 years 4 months) with achondroplasia who underwent bilateral limb lengthening between 2004 and 2012. All patients first underwent bilateral tibial lengthening, and at 9-48 months (average, 17.8 months) after this procedure, bilateral femoral lengthening was performed. We analyzed the pixel value ratio (PVR) and characteristics of the callus of the lengthened area on serial radiographs. The external fixation index (EFI) and healing index (HI) were computed to compare tibial and femoral lengthening. The complications related to lengthening were assessed.
RESULTS
The average gain in length was 8.4 cm for the femur and 9.8 cm for the tibia. The PVR, EFI, and HI of the tibia were significantly better than those of the femur. Fewer complications were found during the lengthening of the tibia than during the lengthening of the femur.
CONCLUSION
Tibial lengthening had a significantly lower complication rate and a higher callus formation rate than femoral lengthening. Our findings suggest that bilateral limb lengthening (tibia, followed by femur) remains a reasonable option; however, we should be more cautious when performing femoral lengthening in selected patients.

Keyword

Achondroplasia; bone lengthening

MeSH Terms

Achondroplasia/*surgery
Adolescent
Bone Lengthening/*methods
Child
Child, Preschool
Female
Femur/radiography/*surgery
Humans
Male
Retrospective Studies
Tibia/radiography/*surgery
Treatment Outcome
Young Adult

Figure

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  Fig. 1 Free line range of interest drawn at each segment. The value M (encircled in white) indicates the mean pixel value for that segment, and it was used in the computation of the pixel value ratio.

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  Fig. 2 Serial graph of the pixel value ratio measurements for the tibia and femur during the lengthening period. PVR, pixel value ratio.

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  Fig. 3 Radiographs of a 7-year-old male who underwent bilateral femoral lengthening. (A) Prior to external fixator removal (note the lateral shape of the callus), (B) bilateral femoral re-fracture after fixator removal, (C) treated with bilateral Ilizarov external fixator application with Nancy nail supplementation and autologous iliac bone grafting, and (D) at the last follow-up (17 months after surgery), showing complete union and consolidation.

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