Hutchinson-Gilford Progeria Syndrome

Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare syndrome of segmental premature aging. Children appear healthy at birth and within the first year of life develop severe failure to thrive with sclerodermatous skin changes. Classic craniofacial features develop with age and underlying disease culminates in death from myocardial infarction or stroke.

Thinning and mottling of the calvarium, prominent vascular markings, delayed closure of the anterior and posterior fontanels, widening of calvarial sutures, and a J-shaped sella, optic nerve kinking and hypotelorism, short mandibular rami in combination with flattened mandibular condyles are observed.