Limb reconstruction in Ollier\'s disease

Abstract

We present our experience of lengthening and correction of complex deformities in the management of patients with Ollier’s dysplasia (multiple enchondromatosis) from 1985 and 2002. All patients were under 18 years with a minimum follow-up time of 2 years (mean 9.6 years, range 2–15 years). There were a total of ten patients of which seven were male and three female. The mean age at presentation was 10.7 years (range 5–17 years; SD 3.7 years). The total length gain was 42.3 mm (range 30–110 mm; SD 28.9 mm). The number of days in external fixation was 164.8 days (range 76–244 days; SD 42.9 days). The bone healing index was 32.5 days/cm (18–50 days/cm; SD 10.3 days/cm). Patients with Ollier’s disease have limb length inequality and angular deformities and require multiple reconstructive procedures owing to a high incidence of recurrence. We identified a tendency for the osteotomy to prematurely consolidate and advise the latency period after surgery to be 4–5 days and for distraction to proceed at a faster rate.

Outcomes of intralesional osteotomy and distraction osteogenesis for limb length equalization in Ollier’s disease

“…Two clinical studies [6,9] used Ilizarov circular external fixator to treat Ollier's disease limb length discrepancy, advocating the safety of intralesional osteotomy. Jesus-Garcia et al (a-e) Recurrence of the valgus deformity happened after 6 months; guided growth was used on the medial side of the distal femur and the proximal tibia.…”

“…Fractures after external fixator removal in patients with Ollier's disease have been reported as one case in each study [6,7,9,14]. An incidence of 3.3% has been calculated in the systematic review discussed earlier [2].…”

“…Intralesional osteotomy has been debated; histological evidence from two cases in two studies [9,10] proved that the regeneration after distraction osteogenesis had a normal lamellar bone formation. Clinical studies [6,7,9–12] also reported normal radiological bone at the distraction site. Possible malignant transformation due to the stimulant effect of the distraction has been discussed, with no clinical reports of any incidence up to date [3].…”

“…Nevertheless, the osteotomy site has been controversial in Ollier's disease patients. Intralesional osteotomy was reported with success in some studies [6,7].…”

Limb Length Discrepancy and Angular Deformity due to Benign Bone Tumors and Tumor-like Lesions

“… 32 , 33 The lengthening over a nail technique uses the versatility of external fixator–driven lengthening and deformity correction while improving stability in the diseased bone. Given the severity of shortening in patients with Ollier disease, several repetitions of distraction osteogenesis may be required if growth was inhibited from a young age 34 (Figure 1 ).…”