Outcomes of Telescopic Nailing of Lower Limb Deformities and Fractures in Children with Osteogenesis Imperfecta: A Prospective Study.

BACKGROUND

Osteogenesis imperfecta (OI) is a genetic disorder of bone fragility and deformity that frequently requires surgical management. The Fassier-Duval (FD) telescopic nail is a treatment of choice for the lower limb deformities and fractures in children with OI, with the aim of decreasing the number of surgeries and improving the results. This study prospectively assesses the clinical and radiological outcomes of FD telescopic nailing in lower limb deformities and fractures in children with OI.

METHODS

This prospective study included 31 children with OI treated for deformities/fractures of the femur or tibia. All had a minimum of 2 years of follow-up, subjected to FD telescopic nailing. All patients were on pre-operative bisphosphonate therapy. Functional outcome scores (BAMF, PODCI), ambulatory ability, radiographic correction (CORA angles), union rates, complication and revision surgery rates were assessed.

RESULTS

The mean age was 5.7±2.1 years; 18 males, 13 females. Most patients had moderate/severe OI. BAMF score improved from 5.8±1.6 to 8.2±1.3 (p<0.001). PODCI score increased from 42.5±7.2 to 46.2±7.5 (p<0.001). The mean angular coronal/sagittal deformity preoperatively was 33.5°/35.6° and was corrected to ~1° postoperatively (p<0.001). Mean time of callus formation was 5.6 weeks. Union was achieved within 6.5 months in all patients. The overall complication rate was 22.5%. Revision surgery was done in 7.5%.

CONCLUSIONS

FD telescopic nailing with osteotomy is a useful method for short-term treatment of lower limb deformities and fractures in children with OI, with significant functional and radiological improvements, increased mobility, and an acceptable complication/revision rate.