Otodental syndrome, oculo-facio-cardio-dental (OFCD) syndrome, and lobodontia: dental disorders of interest to the pediatric radiologist.
Three unusual dental anomalies are presented which should be of interest to the pediatric radiologist: otodental syndrome - an autosomal dominant syndrome of bizarre, greatly enlarged teeth with bulbous crowns (globodontia) that spares the incisors, in combination with sensorineural hearing loss; oculo-facio-cardio-dental syndrome - an X-linked dominant syndrome that is lethal in males, characterized by congenital cataracts, unusual facies, atrial septal defect (ASD), ventricular septal defect (VSD), and canine teeth that cease to grow only when they cut off their own blood supply by growing through the orbit and lower border of mandible; and lobodontia - a dominant disorder characterized by teeth whose crowns resemble those of a wolf.
Gorlin
R J
RJ
University of Minnesota Schools of Dentistry and Medicine, 515 Delaware St S. E., 16-127, Minneapolis, MN 55455 USA.
D000015:Abnormalities, Multiple; D005124:Eye Abnormalities / Q000235:genetics; D005145:Face / Q000002:abnormalities; D005804:Genes, Lethal; D006319:Hearing Loss, Sensorineural / Q000151:congenital; D006330:Heart Defects, Congenital / Q000235:genetics; D006801:Humans; D009811:Odontometry; D011859:Radiography; D013577:Syndrome; D014071:Tooth Abnormalities / Q000000981:diagnostic imaging / Q000235:genetics; D019228:Tooth Crown / Q000002:abnormalities*; D014960:X Chromosome