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PEDODONTICS NON SYNDROMIC FAMILIAL HYPODONTIA - A CASE SERIES Halah Binladen 1a* , Amal Mahmoud 1b , Neeta Chandwani 1c , Manal El-Halabi 1d Department of Pediatric Dentistry, MBRU Hamdan Bin Mohammed College of Dental Medicine, The Mohammed Bin Rashid University of Medicine and Healthcare Sciences (MBRU), Dubai, UAE 1 DDS, MSc, Specialist Pediatric Dentist DDS, MSc, Specialist Pediatric Dentist c DDS, MScD, Specialist Pediatric Dentist, Visiting Faculty d DDS, MScD, Associate Professor, Chair, Program Director a b ABSTRACT DOI: 10.25241/stomaeduj.2018.5(1).art.6 Aim: The aim of this case report is to discuss the features of non-syndromic familial hypodontia, which was noted in three siblings who had an unremarkable medical history. Summary: Congenital absence of teeth is a common dental anomaly referring to teeth that fail to erupt in the oral cavity and remain invisible in radiographs. Congenital tooth agenesis is defined by the number of missing teeth, excluding the third molars; hypodontia refers to the absence of less than six teeth, while oligodontia is the absence of six or more teeth. Tooth agenesis can occur as part of a genetic syndrome such as the cleft lip and palate, ectodermal dysplasia and Trisomy 21 (syndromic hypodontia) or an isolated trait (nonsyndromic hypodontia). Non-syndromic hypodontia is the most common form and can be familial or sporadic. The most supported etiological theory suggests that tooth agenesis may be due to a combination of genetic and environmental factors, which lead to disturbances in the tooth germ during the initial formation stages. This case series reports bilateral agenesis of the maxillary canines in siblings, which is quite rare in occurrence. The management of these anomalies involves a multidisciplinary approach in order to restore esthetics and function. An early diagnosis can lead to effective treatment planning and satisfactory results. Key learning points: The clinical manifestations as well as the short and long term management of this condition are discussed. Keywords: hypodontia, congenital, nonsyndromic, management. 1. Introduction Hypodontia is a very common dental anomaly that occurs mainly in the induction and proliferation stage of tooth development, leading to tooth agenesis, posing a significant clinical problem. It can occur in both the primary and permanent dentition and its classification is based on the number of missing teeth, excluding the third molars [1]. Anodontia is defined as the complete absence of teeth either in one or both arches [1,2]. Oligodontia is a severe and rare form of tooth agenesis which is defined as the absence of six or more teeth, while the congenital absence of less than six teeth is referred to as hypodontia. Hypodontia can be inherited as an isolated trait or is usually associated with several syndromes such as ectodermal dysplasia, Down Syndrome and cleft lip and palate [2,3]. Non-syndromic hypodontia is more common, with varying numbers of teeth that can be involved; however, the incisor-premolar type predominates [3]. Various studies report that hypodontia is more commonly seen in the maxillary arch and the permanent dentition is much more affected than the primary dentition [3,4]. A review of the literature indicates that the etiology of hypodontia is multifactorial and has been reported to be due to genetic as well as environmental factors [5]. Recent studies indicate the role of the genes in the tooth morphogenesis from the initiation, patterning and histogenesis of dental tissues. Mutations in some of 52 OPEN ACCESS This is an Open Access article under the CC BY-NC 4.0 license. Peer-Reviewed Article Citation: Binladen H, Mahmoud A, Chandwani N, El-Halabi M. Non syndromic familial hypodontia - a case series. Stoma Edu J. 2018;5(1):52-57. Academic Editor: Rodica Luca, DDS, PhD, Professor, “Carol Davila” University of Medicine and Pharmacy Bucharest, Bucharest, Romania Received: January 31, 2018 Revised: February 26, 2018 Acccepted: March 20, 2018 Published: March 26, 2018 *Corresponding author: Dr. Halah Binladen, DDS, MSc, Specialist Pediatric Dentist, Department of Pediatric Dentistry, MBRU Hamdan Bin Mohammed College of Dental Medicine, The Mohammed Bin Rashid University of Medicine and Healthcare Sci- ences (MBRU), PO Box 71696, Dubai, UAE, Tel: 800-MBRU (6278), Direct: [phone], e-mail: [email protected] Copyright: © 2018 the Editorial Council for the Stomatology Edu Journal. these genes, mainly MSX1, PAX9 and AXIN1 may result in tooth agenesis, although the exact mechanism is not known [6-9]. The environmental factors implicated in tooth agenesis include trauma to the dental region, chemotherapy or radiotherapy and maternal infections during tooth development [6,7]. Prevalence of hypodontia in the permanent dentition, excluding the third molars, is about 4.5 – 7.4% in Caucasians; while including the third molars is about 9-30% [2,4]. A 2016 systematic review and meta-analysis found that there was no difference between the prevalence of hypodontia among the different ethnic groups with each affected Negroid, Mongoloid, and Caucasian having 1.871, 1.900, and 1.889 missing teeth respectively [10]. The most frequently affected teeth are the mandibular second premolar and the maxillary lateral incisor [4]. The congenital absence of canines in the permanent dentition is reportedly a very rare occurrence with prevalence rates varying from 0.06% to 0.18% [11]. The present cases report familial non-syndromic hypodontia in three siblings, of which two cases show bilateral maxillary canine agenesis. 2. Case report 2.1. Case 1 A 7.9-year-old girl (ZA) presented with her father at the department of pediatric dentistry for an initial dental Stoma Edu J. 2018;5(1): 52-57 http://www.stomaeduj.com