Early Results of the Bernese Periacetabular Osteotomy for Symptomatic Dysplasia in Charcot-Marie-Tooth Disease

Abstract

Introduction Charcot-Marie-Tooth disease (CMTD) is one of the most common inherited neurologic disorders and can be associated with hip dysplasia. Little is known regarding outcomes of the PAO for patients with CMTD. Our purpose is to document the early results and complications of the PAO for hip dysplasia associated with CMTD. Methods A two centre, retrospective clinical and radiographic review was performed. Demographic and surgical data were recorded. Pre- and postoperative lateral centre edge angle (LCEA), acetabular index (AI), ventral centre edge angle (VCEA), and Tönnis osteoarthritis grade were compared. Hips were classified according to Severin. The Harris Hip Score (HHS) and the Western Ontario and McMasters University (WOMAC) index documented self-reported function. Results Nineteen hips in 14 patients underwent PAO, mean age 16.2 (range 11.2–21 years). Thirteen concomitant procedures were performed, including seven proximal femoral osteotomies. Average follow-up was 3.4 years (range 0.9–8.5). Postoperative radiographic measurements significantly improved. Complications included femoral head AVN ( 1 ), transient complete bilateral peroneal nerve palsy ( 1 ), inferior rami fractures ( 4 ), and heterotopic ossification (Brooker stage 3) ( 1 ). The HHS significantly improved from a mean 49.6 pre-operatively to 82.2 at final follow-up of four patients. Seven subjects reported a mean postoperative WOMAC score of 94 (range 58.3–100). Conclusions Most patients presented with severe dysplasia in the second decade of life. The PAO successfully corrected the radiographic abnormalities. Complications were common. The majority of patients reported improved outcomes, although seven showed signs of radiographic progression of osteoarthritis.