Rare and Multiple Hypodontia in Van der Woude Syndrome: Case Report

2021 ◽  
pp. 105566562110582
Author(s):  
Aline Cristina da Silva Trevizan ◽  
Andréa Guedes Barreto Gonçales ◽  
Bruna Stuchi Centurion Pagin ◽  
Otávio Pagin ◽  
Lucimara Teixeira das Neves

Van der Woude syndrome (VWS) is a rare syndrome of genetic etiology, commonly occasioned by mutations in the IRF6 gene and that causes disorders in craniofacial development. VWS is characterized by the presence of paramedian fistulas in the lower lip and cleft lip and / or cleft palate. Although some dental phenotypes have been reported in this syndrome, multiple and rare hypodontias were not described. Through this case report, we present a case of Van der Woude Syndrome (VWS) with rare and multiple hypodontia in which clinical data and radiographic exams were evaluated. The patient presented hypodontia of eight permanent teeth (lateral incisors, second premolars and second molars). So, when the dentist recognizes multiple and/or rare hypodontias, for an accurate diagnosis, detailed examination of the lower lip is indicated, as well as a survey of the family history and referral for genetic counseling, since the syndrome presents high penetrance. The patient is expected to be rehabilitated to have a good quality of life. Rehabilitation in these cases requires alveolar bone graft, orthodontics and prosthesis to replace missing teeth.

1996 ◽  
Vol 33 (5) ◽  
pp. 436-439 ◽  
Author(s):  
Peter J. Anderson ◽  
Anthony L.H., Moss

The incidence of dental abnormalities in the cleft lip and palate population has been reported to be much higher than in the normal population. The role of genes in the production of a cleft lip and palate, and dental anomalies is thought to be complex, with autosomal dominant, recessive, and x-linked genes all playing a role. Noncleft parents can carry some of the cleft lip and palate genes, which produce clinically subtle manifestations in their facial skeleton. The purpose of this study was to look for evidence of increased dental anomalies in the non-cleft parents of cleft lip and palate children. The dentitions of the parents of 60 children with different types of cleft lip and palate were examined prospectively to see whether or not they exhibited features found more readily in the cleft lip and palate rather than did the normal population. Their dentitions were studied to record the following dental features: congenitally missing teeth, supernumerary teeth, or morphologic changes of the crowns of the permanent teeth. The number and position of any frenal attachments were also recorded. The results of this study did not show any differences in incidence of dental anomalies from the noncleft population. There was no evidence to support the hypothesis that congenital absence of lateral incisors is a microform of cleft lip and palate. Further, these results also failed to reveal any consistent pattern in the number and position of frenal attachments.


2020 ◽  
Vol 13 (1) ◽  
pp. 6-9 ◽  
Author(s):  
Mustafa Elhussein ◽  
Jonathan J O'Dwyer ◽  
Jonathan Sandler

Fractured and missing teeth within the aesthetic zone have always posed a clinical challenge for the dental team. Management and treatment requires the input from a number of dental specialists to guarantee a high quality result. A case report is presented to demonstrate an effective combined orthodontic/restorative approach that fully restored aesthetics and function. The report is of a 14-year-old girl who traumatized her maxillary central incisors, in a dentition that was already compromised as she had congenital absence of her maxillary left lateral incisor. Using fixed orthodontic appliances, the fractured teeth were extruded to bring the fracture line above the level of the alveolar bone. This movement allowed an immediate aesthetic improvement, using stainless steel prefabricated posts and composite resin buildups, which also facilitated further orthodontic tooth movement. Teeth, that would otherwise have been extracted, were salvaged and, utilizing a multidisciplinary team approach, a reasonable long-term prognosis has resulted. CPD/Clinical Relevance: This case report aims to demonstrate how a multidisciplinary team approach in Orthodontics can be utilized for management of severely handicapped dentitions that have sustained dental injury. It is not the aim of this case report to discuss the treatment of dental injuries but to demonstrate and provide an overview of the possible treatment modalities that can be effective in restoring function and aesthetics, with special consideration given to missing teeth and subgingival crown fractures within the aesthetic zone.


2016 ◽  
Vol 5 (1) ◽  
pp. 61-63
Author(s):  
Karla Ferreres García ◽  
Beatriz Berenguer ◽  
Luis Ortiz Quintana ◽  
Elena De Tomás Vicente ◽  
Ricardo Fernández Pérez-Pacheco ◽  
...  

Abstract We report the postnatal diagnosis of Van der Woude syndrome (VWS) in a foetus found to have an isolated right cleft lip and palate by ultrasound examination. After prenatal genetic counselling, the parents declined further evaluation by amniocentesis. At delivery, the infant was also found to have labial pits in the lower lip in addition to the cleft lip and palate identified by ultrasound consistent with VWS. Although VWS is rare, its autosomal dominant inheritance and variable penetrance should prompt additional modalities to more thoroughly evaluate the extent of other organ system and more extensive craniofacial anomalies.


2021 ◽  
Vol 2 (1) ◽  
pp. 61-64
Author(s):  
Andrian Fajar Kusumadewi

Introduction : Organic mental disorders are diseases we need to put more attentionon because they are related to systemic disorders or disorders of the brain and cancause high mortality. Organic mental disorders often manifest in the form ofpsychiatric symptoms so that they can be treated too late because the physician isnot able to recognize the symptoms which can be fatal. Sequelae can be found inorganic mental disorders and may affect the patient’s quality of life, so a fast andproper management is needed to get a better outcome. Case presentation: A caseof organic hallucinosis in viral encephalitis had been reported in a 18 year-old malewith a history of sudden changes in behaviour. The symptoms appeared after thepatient had problems during OSPEK and was threatened by someone. The patientwas the only child in the family and often spoiled by his parents. Laboratory andimaging studies showed that there was a cerebritis in the CT scan result, a decreasein CD4 count, and an increase in anti-Rubella IgG titers in which the patient wasfinally diagnosed with viral encephalitis. Conclusion: The diagnosis of organicmental disorders can easily be overlooked in daily clinical practice so that patientsdo not receive proper management


2017 ◽  
Vol 158 (33) ◽  
pp. 1314-1318
Author(s):  
Katalin Vajda ◽  
Gergő Fekecs ◽  
Zsófia Muzsek

Abstract: Avulsion of the permanent teeth is one of the most serious forms of traumatic dental injuries, which endangers the patients’ quality of life. Therefore, the fast and proper treatment is crucial. In this case report we will discuss the supportive and definitive functional treatment and correct rehabilitation. The traumatic force can cause complex oral and maxillofacial injuries, which comprehensive treatment constitutes a major challenge for the dentist. Here we review functional restoration of dental injuries and their definitive aesthetic solution. Orv Hetil. 2017; 158(33): 1314–1318.


2021 ◽  
Vol 10 (3) ◽  
pp. 1-6
Author(s):  
Daysi Morocho-Monteros ◽  
◽  
Juan Marcos Parise-Vasco ◽  
Danela Cisneros-Boada ◽  
Sabela Manzano-Flores ◽  
...  

Introduction: Hereditary gingival fibromatosis is a rare disorder with a genetic component that may appear during tooth replacement. This condition can cause functional and aesthetic pro-blems such as malocclusions, diastemas, pain when chewing, dental caries, periodontal disease, delayed eruption, among others. Objective: To report the multidisciplinary treatment provided to a patient with hereditary gingival fibromatosis. Case Report: This report describes the treatment carried out in a thirteen-year-old male patient presenting generalized increase in gingival volume associated with functional and aesthetic compromise and delayed eruption of permanent teeth. After diagnosis, a multidisciplinary intervention was proposed, involving perio-dontal and pediatric dentistry procedures, which improved the quality of life of the patient both functionally and aesthetically. Conclusion: Hereditary gingival fibromatosis not only affects the dental eruption process, but also causes aesthetic and emotional alterations in the patient. The periodontal procedures significantly im-proved the appearance, function, and the psychological state of the patient.


2020 ◽  
Vol 4 (7) ◽  
pp. CR3-CR6
Author(s):  
Parul Uppal Malhotra ◽  
Yagyeshwar Malhotra ◽  
Neera Ohri ◽  
Anindita Mallik

Hypodontia is the most common dentofacial anomaly observed in humans.  It can be syndromic or an isolated trait. Missing teeth not only affects functionality of dentition but also aesthetically looks unpleasing. In this case report, a 12 year old girl is presented with agenesis of four permanent teeth. On examination, patient was found to be suffering from non syndromic hypodontia. Restorative and Prosthetic treatment was done to rehabilitate the case


Author(s):  
Shakun Kanjani

ABSTRACT Hypodontia is the term used to describe the developmental absence of one or more primary or permanent teeth, excluding the third molars. It is the most commonly occurring developmental dental anomaly and can be a challenge to manage clinically. Hypodontia can occur in association with syndrome or it may occur in nonsyndromic patient. Bilateral occurrence is common but it is very rare to see two bilateral congenital missing teeth in a nonsyndromic patient. This case report presents a rare occurrence of congenital bilateral missing mandibular second premolars and mandibular lateral incisors in a nonsyndromic patient. How to cite this article Agarwal N, Chaturvedy S, Marwah N, Mishra P, Kanjani S. Bilateral Hypodontia of Mandibular Second Premolars and Lateral Incisors in a Nonsyndromic Patient: A Rare Case Report. J Mahatma Gandhi Univ Med Sci Tech 2016;1(1):24-26.


2021 ◽  
Vol 98 ◽  
pp. 01014
Author(s):  
Viktoria Agaeva ◽  
Galina Butko ◽  
Natalia Bal

This article discusses important factors of quality of life of children with oral and maxillofacial pathologies: the issues of socialization and social and psychological adaptation. Cleft lip and cleft palate are common congenital malformations, having impact not only on somatic status but also on socialization, which is determined, in particular, by appearance and speech quality. As a child gets older, the risk of formation of secondary disorders of emotional and personal, behavioral and communicative spheres increases, which negatively affects psychoemotional background of the family. This work is aimed at substantiation of necessity of integrated assistance to families with children with appearance defects as the main subject of their socialization. The research was based on the methods of evaluation of social situation of a child with cleft lip and cleft palate and its influence on successful speech rehabilitation as an important constituent of quality of life. Analysis of the obtained data about opportunities of social adaptation of children with oral and maxillofacial pathologies gives confidence in necessity to maximum involvement of the family of such child into rehabilitation. It is important that all experts of interdisciplinary team understand the most important role of family in rehabilitation success, assist to parents and the child to resolve complicated situations related both with operative intrusion and with subsequent remedial actions. Early terms of integrated assistance allow to eliminate esthetic and functional violations of maxillofacial region, providing opportunities for full socialization of the child. Peculiar attention should be paid to the issue of tolerance of society regarding the persons with maxillofacial abnormalities.


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