Hip in arthrogryposis multiplex congenita

PURPOSE OF THE STUDY: In arthrogryposis Multiplex Congenita (AMC) multiple joint contractures and deformities are present. Hips are often affected. In this follow-up study the outcome after primary treatment and secondary surgical procedures was analyzed with clinical and radiological features. MATERIAL AND METHODS: 52 patients with AMC were treated at Children's Hospital, Helsinki. 34 patients had Hip joint involvement. The most common type of Hip joint involvement was a flexion contracture (40 per cent). Thirty-five per cent of patients had rigid dislocations. Ten hips with rigid dislocations and four hips with flexion contractures was primarily operated on. Two failures of the primary operative procedures were noted. Thirty-one dislocated hips were treated non-operatively. RESULTS: In fourteen cases, reduction could not be achieved. Nineteen delayed surgical procedures were done. Acetabuloplasty was made in 6 hips, femoral osteotomy in 8 hips and total hip replacement in 3 hips. Aseptic necrosis of the femoral head encountered for 4 poor results in two cases of acetabuloplasty and 3 hips had a poor range of motion. In flexion contractures of the hips, 6 corrective osteotomies were done and in four cases the results were good. In all cases of total hip replacement the results were good and no complications were seen. DISCUSSION: The inefficiency to achieve reduction of dislocated hips in AMC-patients by conservative methods was confirmed in this study. Although it has been claimed that the majority of patients with flexion contractures can be managed by conservative treatment there were ten patients in this series who did not respond to this form of treatment. CONCLUSION: The primary form of treatment should be operative. Only in selected cases with bilateral dislocations should it be considered to leave the hips untreated. Double femoral osteotomy in recurrent flexion contractures or resistant flexion contractures and total hip replacement in adult patients with hip dislocations are useful methods.