Abstract
The purpose of this study was to review the outcomes of four children with genu valgum secondary to Hurler Syndrome treated with circular external fixators (frames) for angular correction.
We retrospectively reviewed the medical and radiographic records of four children with Hurler Syndrome and genu valgum treated with frames. Three children had simultaneous bilateral tibial corrections. The fourth child had unilateral femoral correction. The mean age of the children was 14 years at application of frame. Mean duration of frame was 113 days for the tibial frames, and the femoral frame remained in-situ for 150 days. Correction was assessed clinically, and radiologically with x-rays and CT scannograms, with excellent results in all four cases. The complexities of each individual case necessitated specific and individualised treatment for each child. Complications included further deformities arising in treated and un-treated long-bones both during and after application of frame.
Prior to the introduction of bone marrow transplantation, the average life expectancy of children with Hurler Syndrome was seven years. With bone marrow transplantation, affected children are now living much longer, and many develop characteristic long bone deformities in their lower limbs. These deformities are progressive and can be multifocal and polyostotic. Managament can be extremely challenging, and prior reports of management with hemiepiphysiodesis with staples and 8-plates have been mixed. We believe that this is the first series of circular frame lower limb reconstruction in children with Hurler Syndrome. The flexibility and adaptability of frames confers a unique advantage in the management of these complex deformities.
