Lacrimo-auriculo-dento-digital syndrome. Case report, review of the literature, and clinical spectrum.
Lacrimo-auriculo-dento-digital (LADD) syndrome, also known as Levy-Hollister syndrome, is characterized by pronounced dysplasias in various organ systems. The resulting disturbances affect primarily the lacrimal glands, the inner and outer ear, the salivary glands, and the osseous framework. Although the degree to which the different organs are involved varies considerably, dental anomalies are regularly reported. An autosomal-dominant trait has been recognized for this syndrome, but the majority of cases described in the literature concerned sporadic new mutations. We report on this unusual and relatively unknown syndrome with reference to a 13-year-old girl suffering from LADD syndrome presenting symptoms of severe xerostomia and various craniofacial malformations, hearing loss, enamel dysplasia, microdontia, and hypodontia.
Lehotay
Michaela
M
Department of Orthodontics, University of Mainz, Germany. m.lehotay@web.de
Kunkel
Martin
M
Wehrbein
Heinrich
H
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D000293:Adolescent; D019465:Craniofacial Abnormalities / Q000175:diagnosis; D003878:Dermatoglyphics; D004431:Ear, External / Q000002:abnormalities*; D005260:Female; D005385:Fingers / Q000002:abnormalities*; D006314:Hearing Loss, Conductive / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D007569:Jaw Abnormalities / Q000175:diagnosis*; D007765:Lacrimal Apparatus / Q000002:abnormalities*; D013577:Syndrome; D014071:Tooth Abnormalities / Q000209:etiology*