Hallermann-Streiff Syndrome: Magnetic Resonance Imaging of a Rare Disorder

摘要

Hallermann-Streiff syndrome (HSS) was completely described in 1948 by Hallermann and then in 1950 by Streiff1 who delineated clinical criteria for the diagnosis, separating the syndrome from progeria and mandibulofacial dysostosis. The clinical diagnostic criteria of this entity include seven essential signs: dyscephalia, bird faces, dental anomalies, proportionate short stature, hypotrichosis, atrophy of the skin, bilateral microphthalmia, and congenital cataract. The bird face and the congenital cataract are especially important to differentiate oculomandibular dyscephaly from disorders with a superficial resemblance to it, such as progeria. Virtually, all cases of the HSS are sporadic and there is no sex predilection.