Hip pathology in Hutchinson–Gilford progeria syndrome

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.

Radiological Diagnosis of a Rare Premature Aging Genetic Disorder: Progeria (Hutchinson-Gilford Syndrome)

“…Bilateral coxa valga deformity with bizarre greater trochanters and shallow acetabulum with bilateral hip dislocation were seen in our patient. Coxa magna, coxa valga, acetabular dysplasia, hip subluxation, and dislocation are the common orthopaedic hip manifestations which have been previously reported [ 11 ]. Avascular necrosis of the femoral head, flared long bone metaphysis/epiphysis, enlarged capitulum of the distal humerus have also been reported.…”

The Use of Peripheral Nerve Block and Intra-articular Steroid Injection for Pain Management in an Adolescent with Hutchinson-Gilford Progeria Syndrome: A Case Report

“…2,3 They follow a normal psychomotor development but later develop multisystem comorbidities associated with aging such as coronary artery disease, cerebrovascular disease, hypertension, and atherosclerosis. [2][3][4] There have been reports on the anesthetic management in patients with HGPS, however, there is little to no literature on the use of pure locoregional anesthesia for pain management in these patients.…”