Limb lengthening for deformities in Ollier's disease: a systematic review

The management of limb deformity, shortening, and bone defects in treatment of Ollier's disease is a major challenge. This study aims to summarize and compare the different surgical treatments, and to evaluate the outcome and possible prognostic factors of leg lengthening in these patients.

A systematic review of the literature from 1993 to 2017 was performed. Nineteen articles were found including a total of 121 patients with limb deformities because of Ollier's disease. The mean patients' age at the time of first surgery was 12 years. A total of 272 segments were surgically treated (14 segments in the upper limbs) with variable techniques including osteotomies and external fixation, intramedullary nails, as well as epiphysiodesis and lengthening over nail. We studied the bone healing index (BHI), distraction index (DI), distraction time, gained length, total treatment time, and complications.

Available implants and techniques allowed correction of patients' deformities (lengthening and correction of angular defects) in most cases. External fixators, circular or monolateral frames were the most commonly used technique. The Ilizarov external fixator was the most commonly used frame (196 segments). The BHI was significantly better when the external fixation was combined with intramedullary nails. Epiphysiodesis was most likely to be more associated with the past than to the present. Joint stiffness, infection, early consolidation, pathological fracture, deformity recurrence, delayed union, non-union, neurapraxia, and overlengthening were the reported complications with an overall rate of 27.9%.

There is no consensus for the optimal surgical technique and implants for correction of limbs deformities in patients with Ollier's disease. External fixators most commonly circular are the most commonly used implants; however, complications do occur.