Hip Dysplasia in Mucopolysaccharidosis Type IVA (morquio A Syndrome) Treated by Proximal Femoral Valgization Osteotomy: A Case Report.

INTRODUCTION:Morquio A syndrome or mucopolysaccharidosis type IVA (MPS IVA) is a progressive lysosomal storage disorder caused by an N-acetylgalactosamine-6-sulfatase deficiency. The abnormal metabolism of glycosaminoglycans among other medical problems leads to various skeletal disorders caused by a dysfunction of endochondral ossification of epiphyseal cartilage. Severe hip dysplasia is common and can lead to pain and impaired mobility.CASE REPORT:We report on a 15-year-old girl suffering from MPS IVA. At the age of 5 years, hip pain and a reduced walking distance were described for the 1sttime. At the age of 9 years, acetabulofemoral dysplasia associated with genuavalga was diagnosed. After pre-operative assessment of the hips including plain radiographs, magnetic resonance imaging, and arthrography with dynamic testing a valgization osteotomy of the proximal femur in combination with a shelf acetabuloplasty was performed. The patient was followed for 6 years with a stable hip joint and without any sign of redislocation.CONCLUSION:Some treatment strategies of hip dysplasia in patients with MPS IVA are described in the literature. The techniques used for congenital hip dysplasia, varisation of the femur in combination with Pemberton, Salter, or shelf acetabuloplasty, are widely reported. Nevertheless, resubluxations were described in some cases. The well-known surgical procedure with valgization of the proximal femur is not reported in literature for MPS IVA patients. In our opinion, dynamic testing with arthrography should strongly be considered for this particular problem before surgical intervention. Pathology-related decisions should be made under consideration of the different surgical techniques.