title: Hip pathology in Hutchinson-Gilford progeria syndrome:  a report of two children.
abstract: 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.
authors.affiliation: Department of Trauma and Orthopaedics, Conquest Hospital, East Sussex Hospitals Trust, East Sussex, UK. pakhbari@nhs.net
authors.forename: Pouya
authors.initials: P
authors.lastname: Akhbari
authors.forename: Shilpa
authors.initials: S
authors.lastname: Jha
authors.forename: Kyle D
authors.initials: KD
authors.lastname: James
authors.forename: Barry L
authors.initials: BL
authors.lastname: Hinves
authors.forename: Jamie A F
authors.initials: JA
authors.lastname: Buchanan
mesh_terms: D000077:Acetabulum / Q000473:pathology; D000293:Adolescent; D002648:Child; D060906:Coxa Valga / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D005271:Femur Head Necrosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D006617:Hip Dislocation / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology*; D006621:Hip Joint / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D011371:Progeria / Q000175:diagnosis* / Q000000981:diagnostic imaging; D012008:Recurrence; D014057:Tomography, X-Ray Computed