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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Elsevier
2024-09-01
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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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