Hip pathology in Hutchinson-Gilford progeria syndrome: a report of two children.

Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder. The estimated incidence is one in 4 million births. Orthopaedic manifestations include abnormality of the hips occurring early in the disease process. Severe coxa valga can be apparent by the age of 2 years. We report two cases of HGPS, one in a 7-year-old girl with avascular necrosis of the left hip and the second in a 13-year-old girl with recurrent traumatic hip dislocations. We demonstrate the pathoanatomical changes in the hip with HGPS using a combination of imaging modalities including radiographic, computed tomographic and MRI scans. These include coxa magna, coxa valga and acetabular dysplasia. We also comment on how these would affect the surgical management of this high-risk group of patients.

Long-term follow-up of a hydroxyapatite ceramic-coated threaded cup: an analysis of survival and fixation at up to 15 years.

We evaluated the survival of 112 consecutive JRI hydroxyapatite ceramic-coated threaded acetabular components (JRI Ltd, London, UK). Follow-up was for a minimum of 13 years (range, 13-15 years). Patients were assessed clinically and radiographically using the Merle d'Aubigné and Postel scoring systems and the De Lee and Charnley zones. Cup angle, migration, and signs of radiolucency were used to assess loosening. The criteria for failure were revision or impending revision due to pain and septic or aseptic loosening. The mean results of the Merle d'Aubigné and Postel score for pain, motion, and ability to walk were 2.2/2.7/2.2 preoperatively and 5.6/4.3/5.1 postoperatively. There was 100% endosteal bone formation in De Lee and Charnley zones 1 and 3 and 87% in zone 2. No reactive lines were seen in any zone in any cup. There were no cases of migration, change in cup angle, or revision for loosening. Our survival was 99% at 13 years (95% confidence interval, 96-100).