Oral manifestations of Nance Horan syndrome: A systematic review of case reports

Nance Horan Syndrome (NHS) is a rare congenital genetic condition. However, due to the sporadicity of cases and the similarity in manifestations of mild NHS with X-linked cataracts, it is difficult for clinicians and researchers to diagnose and treat NHS. Therefore, we undertake a comprehensive revi...

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Main Authors: Ankita Mathur, Sapna Negi, Snehasish Tripathy, Shalini Aggarwal, Felix Amekpor, Vini Mehta
Format: Article
Language:English
Published: Elsevier 2024-09-01
Series:Oral Oncology Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S2772906024004588
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author Ankita Mathur
Sapna Negi
Snehasish Tripathy
Shalini Aggarwal
Felix Amekpor
Vini Mehta
author_facet Ankita Mathur
Sapna Negi
Snehasish Tripathy
Shalini Aggarwal
Felix Amekpor
Vini Mehta
author_sort Ankita Mathur
collection DOAJ
description Nance Horan Syndrome (NHS) is a rare congenital genetic condition. However, due to the sporadicity of cases and the similarity in manifestations of mild NHS with X-linked cataracts, it is difficult for clinicians and researchers to diagnose and treat NHS. Therefore, we undertake a comprehensive review of the existing literature on the oral manifestations of NHS and, its definitive diagnosis based on oral and craniofacial symptoms. An electronic search of PubMed-MEDLINE, Embase, Scopus, and Google Scholar databases was done to retrieve case reports documenting oral anomalies related to NHS at the time of presentation. Genetic studies or studies focussing on the genetic analysis of the case were excluded. Descriptive statistics were used to calculate simple frequency, percentages, and proportions to identify trends and commonalities in presenting symptoms and oral assessment findings. Out of 2086 identified studies, 12 studies that met the inclusion criteria were reviewed. Most patients' ages ranged from between 4 and 12 years of age. Out of 31 affected individuals, 11 were females and 20 were males. Screwdriver-shaped incisors (75 %) often accompanied by the presence of supernumerary teeth and diastema (66 % studies), Hutchinson incisors (50 % studies), Mulberry molar (41.6 %) malocclusion (58.3 % studies) were commonly seen. Besides that, talon cusps, agenesis/non-eruption of teeth, premolars, and Taurodontism were also observed. Facial dysmorphism was common in all the studies. An in-depth understanding of these anomalies will enable dental practitioners to provide specialised care, including focused interventions and preventive measures to address the oral health issues associated with NHS.
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spelling doaj-art-0aa701cf29cc4e29bfef603bc766c7182025-01-09T06:16:55ZengElsevierOral Oncology Reports2772-90602024-09-0111100612Oral manifestations of Nance Horan syndrome: A systematic review of case reportsAnkita Mathur0Sapna Negi1Snehasish Tripathy2Shalini Aggarwal3Felix Amekpor4Vini Mehta5Department of Dental Research Cell, Dr. D. Y. Patil Dental College and Hospital, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, 411018, Maharashtra, IndiaDepartment of Dental Research Cell, Dr. D. Y. Patil Dental College and Hospital, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, 411018, Maharashtra, IndiaDepartment of Dental Research Cell, Dr. D. Y. Patil Dental College and Hospital, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, 411018, Maharashtra, IndiaDepartment of Conservative Dentistry and Endodontics, Dr. D. Y. Patil Dental College and Hospital, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, 411018, Maharashtra, IndiaDepartment of Clinical Pathology, Noguchi Memorial Institute for Medical Research, University of Ghana, Accra, GhanaDepartment of Dental Research Cell, Dr. D. Y. Patil Dental College and Hospital, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, 411018, Maharashtra, India; Corresponding author.Nance Horan Syndrome (NHS) is a rare congenital genetic condition. However, due to the sporadicity of cases and the similarity in manifestations of mild NHS with X-linked cataracts, it is difficult for clinicians and researchers to diagnose and treat NHS. Therefore, we undertake a comprehensive review of the existing literature on the oral manifestations of NHS and, its definitive diagnosis based on oral and craniofacial symptoms. An electronic search of PubMed-MEDLINE, Embase, Scopus, and Google Scholar databases was done to retrieve case reports documenting oral anomalies related to NHS at the time of presentation. Genetic studies or studies focussing on the genetic analysis of the case were excluded. Descriptive statistics were used to calculate simple frequency, percentages, and proportions to identify trends and commonalities in presenting symptoms and oral assessment findings. Out of 2086 identified studies, 12 studies that met the inclusion criteria were reviewed. Most patients' ages ranged from between 4 and 12 years of age. Out of 31 affected individuals, 11 were females and 20 were males. Screwdriver-shaped incisors (75 %) often accompanied by the presence of supernumerary teeth and diastema (66 % studies), Hutchinson incisors (50 % studies), Mulberry molar (41.6 %) malocclusion (58.3 % studies) were commonly seen. Besides that, talon cusps, agenesis/non-eruption of teeth, premolars, and Taurodontism were also observed. Facial dysmorphism was common in all the studies. An in-depth understanding of these anomalies will enable dental practitioners to provide specialised care, including focused interventions and preventive measures to address the oral health issues associated with NHS.http://www.sciencedirect.com/science/article/pii/S2772906024004588Nance-Horan syndromeDental anomaliesFacial dysmorphismRare X-linked genetic disorder
spellingShingle Ankita Mathur
Sapna Negi
Snehasish Tripathy
Shalini Aggarwal
Felix Amekpor
Vini Mehta
Oral manifestations of Nance Horan syndrome: A systematic review of case reports
Oral Oncology Reports
Nance-Horan syndrome
Dental anomalies
Facial dysmorphism
Rare X-linked genetic disorder
title Oral manifestations of Nance Horan syndrome: A systematic review of case reports
title_full Oral manifestations of Nance Horan syndrome: A systematic review of case reports
title_fullStr Oral manifestations of Nance Horan syndrome: A systematic review of case reports
title_full_unstemmed Oral manifestations of Nance Horan syndrome: A systematic review of case reports
title_short Oral manifestations of Nance Horan syndrome: A systematic review of case reports
title_sort oral manifestations of nance horan syndrome a systematic review of case reports
topic Nance-Horan syndrome
Dental anomalies
Facial dysmorphism
Rare X-linked genetic disorder
url http://www.sciencedirect.com/science/article/pii/S2772906024004588
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