Long-term outcomes following the medial approach for open reduction of the hip in children with developmental dysplasia.

Aims Open reduction is required following failed conservative treatment of developmental dysplasia of the hip (DDH). The Ludloff medial approach is commonly used, but poor results have been reported, with rates of the development of avascular necrosis (AVN) varying between 8% and 54%. This retrospective cohort study evaluates the long-term radiographic and clinical outcome of dislocated hips treated using this approach. Patients and Methods Children with a dislocated hip, younger than one year of age at the time of surgery, who were treated using a medial approach were eligible for the study. Radiographs were evaluated for the degree of dislocation and the presence of an ossific nucleus preoperatively, and for the degree of AVN and residual dysplasia at one and five years and at a mean of 12.7 years (4.6 to 20.8) postoperatively. Radiographic outcome was assessed using the Severin classification, after five years of age. Further surgical procedures were recorded. Functional outcome was assessed using the Pediatric Outcomes Data Collection Instrument (PODCI) or the Hip Disability and Osteoarthritis Outcome Score (HOOS), depending on the patient's age. Results A total of 52 children (58 hips) were included. At the latest follow-up, 11 hips (19%) showed signs of AVN. Further surgery was undertaken in 13 hips (22%). A total of 13 hips had a poor radiological outcome with Severin type III or higher. Of these, the age at the time of surgery was significantly higher (p < 0.05) than in those with a good Severin type (I or II). The patient-reported outcomes were significantly worse (p < 0.05) in children with a poor Severin classification. Conclusion This retrospective long-term follow-up study shows that one in five children with DDH who undergo open reduction using a medial surgical approach has poor clinical and/or radiological outcome. The poor outcome is not related to the presence of AVN (19%), but due to residual dysplasia. Cite this article: Bone Joint J 2018;100-B:822-7.

Prevalence and development of orthopaedic symptoms in the dutch hurler patient population after haematopoietic stem cell transplantation.

Hurler syndrome (MPS-IH) is a rare autosomal recessive lysosomal storage disease. Besides a variety of other features, Hurler syndrome is characterized by a range of skeletal abnormalities known as dysostosis multiplex. Despite the successful effect of haematopoietic stem cell transplantation on the other features, dysostosis remains a disabling symptom of the disease. This study analyzed the status and development of the orthopaedic manifestations of 14 Dutch Hurler patients after stem cell transplantation.Data were obtained retrospectively by reviewing patients' charts, radiographs and MRIs. Existing methods to measure the deficiencies were modified to optimally address the dysostosis. These measurements were done by two of the authors independently. The odontoïd/body ratio, kyphotic angle, scoliotic angle and parameters for hip dysplasia and genu valgum were measured and plotted against age. The degree of progression was determined. The intraclass correlation coefficient (ICC) was calculated to determine the reliability of the measurements.All patients showed hypoplasia of the odontoïd, which significantly improved during growth. Kyphosis in the thoracolumbar area was present in 13 patients and proved to be progressive. Scoliosis was observed in eight patients. Hip dysplasia was present in all patients and showed no tendency of improvement. In all but one patient, knee valgus remained more than two standard deviations above normal.Dysostosis remains a major problem after haematopoietic stem cell transplantation in Hurler patients. Moreover, except for dens hypoplasia, it appears to be progressive and therefore surgical interventions may be necessary in the majority of these patients.