Nance-Horan syndrome-The oral perspective on a rare disease.
The present study describes seven patients with Nance-Horan syndrome, all referred to a specialized oral care unit in the Central Denmark Region. A literature search on "Nance Horan Syndrome" resulted in 53 publications among which 29 reported on dental findings. Findings reported in these papers have been systematized to obtain an overview of the reported findings and the terminology on dental morphology. All seven patients included in the present study showed deviations of crown morphology on incisors and/or molars. The only consistent and very clear dental aberration was alterations in the tooth morphology that is screwdriver-shaped incisors and bud molars being most pronounced in the permanent dentition, but were also present in the primary dentition. In addition, three patients had supernumerary teeth, and three had dental agenesis. In conclusion, a dental examination as a part of the diagnostic process may reveal distinct characteristics of the dental morphology, which could be of diagnostic value and facilitate an early diagnosis. In the description of molar morphology in NHS patients, it is recommended to use the term "bud molar." The combination of congenital cataract, screwdriwer-shaped incisors and bud-shaped molars is a strong clinical indication of Nance-Horan syndrome. © 2016 Wiley Periodicals, Inc.
Section of Oral Health in Rare Diseases, Department of Maxillofacial Surgery, Aarhus University Hospital, Aarhus, Denmark.
Hans
H
Gjørup
Section for Pediatric Dentistry, Department of Dentistry, Health, Aarhus University, Aarhus, Denmark.
Dorte
D
Haubek
Department of Specialized Oral Health Care, Viborg Regional Hospital, Central Jutland, Viborg, Denmark.
Pernille
P
Jacobsen
Center for Rare Diseases, Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark.
John R
JR
Ostergaard
D000293:Adolescent; D000328:Adult; D002386:Cataract / Q000151:congenital* / Q000175:diagnosis / Q000235:genetics; D002648:Child; D003718:Denmark; D019066:Facies; D040181:Genetic Diseases, X-Linked / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D008297:Male; D009056:Mouth Abnormalities / Q000175:diagnosis / Q000235:genetics; D010641:Phenotype; D011859:Radiography; D035583:Rare Diseases; D014071:Tooth Abnormalities / Q000175:diagnosis* / Q000235:genetics; D055815:Young Adult
Nance-Horan syndrome; dental morphology; dentition; screwdriver-shaped; tooth anomaly