ABSTRACT
Hallermann-Streiff syndrome (HSS) is a rare congenital disorder characterized by significant craniofacial findings. Dyscephaly, microphthalmia, cataracts, hypotrichosis, cutaneous atrophy, thin pinched nose, and a typical bird-like face are the main features of the syndrome. Additional features of the syndrome include dental anomalies, micrognathia, skeletal defects, and short stature. What follows is a case report of a 10-year-old girl with HSS with special consideration on orodental findings. Clinical, radiographic, and cephalometric analysis revealed hypoplasia of the mandible, high arched palate, Class II malocclusion due to mandibular retrognathia, open bite, posterior crossbite, crowding, malformed teeth, and oligodontia. Magnetic resonance images (MRI) of the temporomandibular joints showed abnormal disks flattened with uniform thickness and deformed condyles bilaterally. Also discussed are the features of HSS with the differential diagnosis, and the dental management of the case is described.
