Orthodontic Treatment in a Patient With Kniest Dysplasia: A Case Study and Review of Literature

2019 ◽  
Vol 56 (10) ◽  
pp. 1393-1403
Author(s):  
Tania Jhamb ◽  
Hayat Masood ◽  
Jeffrey Arigo ◽  
P. Emile Rossouw

Kniest dysplasia is a rare autosomal dominant chondrodysplasia that is characterized by distinct musculoskeletal and craniofacial irregularities. These craniofacial abnormalities include cleft palate, midface anomalies, tracheomalacia, and hearing loss. This article illustrates a case of Kniest dysplasia that presented for orthodontic treatment. The purpose of this literature review is to describe clinical manifestations, radiographic features, histopathological features, genetic mutation, and management of Kniest dysplasia.

Author(s):  
Shweta ◽  
Vijetha Rai ◽  
Kuladeepa Ananda Vaidhya ◽  
Sukesh

Hailey-Hailey disease (HHD), also called as familial benign chronic pemphigus, is a rare autosomal dominant blistering skin disease with waxing and waning in its clinical course. It is characterized by the presence of flaccid vesiculo-pustules, crusted erosions or expanding plaques in the areas of friction such as neck, axilla, groins, and perineum. His to pathologically shows suprabasal separations, inconspicuous dyskeratosis, acantholytic cells within the epidermis, giving a dilapidated brick wall appearance. Here we have discussed about the clinical and histopathological features of HHD and various differential diagnosis for this disease.


2020 ◽  
Vol 8 (11) ◽  
Author(s):  
Breno dos Reis Fernandes ◽  
Darah Ligia Marchiori ◽  
Daniel de Lima e Sá Medronho ◽  
Gabriel Mulinari-Santos ◽  
Patrick Peloso Pereira Figueira ◽  
...  

Odontoma refere-se a tumores de origem odontogênica. Sua etiologia envolve distúrbios de desenvolvimento, traumatismos e infecções. Embora o crescimento seja lento e geralmente assintomático, complicações de ordem estética e funcional podem advir da permanência desta lesão. Os odontomas podem ser classificados como complexos e compostos, e o seu tratamento envolve a remoção cirúrgica conservadora com posterior exame histopatológico. Neste artigo é relatado um caso clínico de uma remoção de um canino incluso no palato associado a um odontoma com posterior enxertia utilizando enxerto do ramo mandibular particulado.Descritores: Odontoma; Cirurgia Bucal; Dente Canino.ReferênciasTeruhisa U, Murakami J, Hitasomi M, Yanagi Y, Asaumi J. A case of unerupted lower primary second molar associated with compound odontoma. Open Dent J. 2009;3:173-76.Ladeinde AL, Ajayi OF, Ogunlewe MO, Adeyemo WL, Arotiba GT, Bamgbose BO et al. Odontogenic tumors: a review of 319 cases in a Nigerian teaching hospital. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005;99(2):191-95.Hidalgo-Sánchez O, Leco-Berrocal MI, Martínez-Gonzáles JM. Metaanalysis of the epidemiology and clinical manifestations of odontomas. Med Oral Patol Oral Cir Bucal. 2008;13(11):730-34.Pires LD, Krüger MLB, Viana ES, Kramer PF, Ferreira SL. Odontoma: estado da arte e relato de caso clínico. Stomatos. 2007;13(24):21-9.Chang JY, Wang JT, Wang YP, Liu BY, Sun A, Chiang CP. Odontoma: a clinicopathologic study of 81 cases. J Formos Med Assoc. 2003;102(12):876-82.Hisatomi M, Asaumi JI, Konouchi H, Honda Y, Wakasa T, Kishi K. A case of complex odontoma associated with an impacted lower deciduous second molar and analysis of the 107 odontomas. Oral Dis. 2002;8(2):100-5.Sheehy EC, Odell EW, Al-Jaddir G. Odontomas in the primary dentition: literature review and case report. J Dent Child (Chic). 2004;71(1):73-6.Sasaki PS, Biancalana H, Duarte DA. Odontoma em pacientes odontopediátricos: repercussöes clínicas e proposiçäo de tratamento Rev Assoc Paul Cir Dent. 2002;56(5):382-86.Serra-Serra G, Berini-Aytés L, Gay-Escoda C. Erupted odontomas: a report of three cases and review of the literature. Med Oral Patol Oral Cir Bucal. 2009;14(6):299-303.Cardoso LC, Miyahara GI, Magro Filho O, Garcia Junior IR, Soubhia AMP. Odontoma combinado associado a dentes não-irrompidos: relato de casos clínicos. Rev Odontol Araçatuba. 2003;24:47-51.Lukes SM, Wachter KM. Compound odontoma: a case study. J Dent Hyg. 2003;77(1):47-9.Chrcanovic RB, Jaeger F, Freire-Maya B. Two-stage surgical removal of large complex odontoma. Oral Maxillofac Surg. 2010;14(4):247-52.


2021 ◽  
Vol 21 (1) ◽  
pp. 44-54
Author(s):  
Sabah Nuri Mizel ◽  
◽  
Ali F Al-Zubaidee

Cherubism is an autosomal dominant, very rare benign fibro-osseous lesion, with an estimation of only 300 cases have been reported worldwide. Cherubism is characterized clinically by bilateral symmetrical self-limiting jaw enlargement starts in the childhood or early teens.Mandible is mostly affected although maxilla and zygomatic bones may be involved but to a lower extent), and is associated with tooth displacement and severe malocclusion. Histopathologically, it is indistinguishable from giant cell lesions therefore the clinical manifestations of the disease remain the Gold Stone. Keywords: Cherubism, mandible, maxilla, bilateral palatal swelling


2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Dorota Kustrzycka ◽  
Marcin Mikulewicz ◽  
Anna Pelc ◽  
Piotr Kosior ◽  
Maciej Dobrzyński

The aim of this article was to present a characteristic clinical image of Melnick–Needles syndrome using an example of an 11.5-year-old female patient treated at the Facial Congenital Disorders Outpatient Clinic as well as to present the actual literature review of the surgical treatment. The patient was diagnosed with several characteristics typical for Melnick–Needles syndrome: single-sided hearing loss, malocclusion, and facial dysmorphism, among others. Due to malocclusion and facial dysmorphism, the patient with Melnick–Needles syndrome requires orthodontic treatment with surgical intervention. Mandibular distraction with fixed appliance treatment is a recommended treatment protocol.


Author(s):  
Zumbi Musiba

Background: Noise induced hearing loss (NIHL) is a major contributor to disabling hearing loss. Engineering controls are superior to hearing protection devices (HPDs) in prevention of occupational noise induced hearing loss (ONIHL), although the latter are more commonly used. Effective use of audiometry requires quick categorization of audiograms. The UK Health and Safety Executive (UKHSE) scheme for the categorization of audiograms is a tool that accomplishes this.Objectives: The objective of this paper is to provide an overview of the classification of audiograms and build a case for the preferential use of the UKHSE’s scheme to achieve this.Method: The author provides a literature review of methods of classification for audiograms and uses a case study in a Tanzanian mining company to demonstrate how the UKHSE scheme was successfully used to enhance the existing hearing protection program.Results: The literature review identified several methods of classification based on a variation of threshold shifts from baseline. The difference was in the frequency and level of threshold shift used to determine hearing loss, and the recommended course of action once hearing loss is detected. The UKHSE scheme is simple and provides guidance on steps to be taken thereafter. This was demonstrated in a case study among miners in a mining company in Tanzania.Conclusion: The UKHSE audiogram classification scheme has the advantage of providing a straightforward, easy to determine classification that allows for intervention appropriate to the findings.


2020 ◽  
Vol 12 (6) ◽  
pp. 132-135
Author(s):  
Sharique M ◽  
Yasir Salam Siddiqui, ◽  
Mazhar Abbas Julfiqar ◽  
Sherwani MKA

Fibrodysplasia ossificans progressiva (FOP) is an exceptionally uncommon autosomal dominant genetic disorder characterized by defects in skeletogenesis manifesting as congenital malformations of the great toes and progressive postnatal induction of disabling ectopic endochondral osteogenesis. During early course of disease patients of FOP are often misdiagnosed as having soft tissue sarcoma or aggressive juvenile fibromatosis and hence sometimes undergo invasive procedures that usually lead to the speeding up of disease process. Therefore early correct diagnosis of fibrodysplasia ossificans progressiva is necessary to prevent additional iatrogenic insult. The case study is being presented to highlight the clinical features of fibrodysplasia ossificans progressiva, difficult aspect of diagnosis and management with brief literature review.


2016 ◽  
Vol 130 (12) ◽  
pp. 1137-1141 ◽  
Author(s):  
K G Effat

AbstractObjective:Otological symptoms (otalgia, subjective hearing loss, blocked ear sensation, tinnitus and vertigo) associated with temporomandibular disorders are documented features of Costen's syndrome. However, the origin of these symptoms and the causes of hearing loss are unknown. This study aimed to characterise hearing loss in a large number of patients with temporomandibular disorders. The causes of these symptoms were explored in patients with otological symptoms and normal audiometric findings.Methods:A prospective case study and literature review were performed. The audiometric features of 104 temporomandibular disorder patients were compared with those of 110 control participants.Results:A large proportion of temporomandibular disorder patients had several otological symptoms. Twenty-five per cent of unilateral or bilateral temporomandibular disorder patients had either unilateral (ipsilateral) or bilateral hearing loss; respectively, which was usually mild (p = 0.001). Hearing loss was predominantly sensorineural.Conclusion:The main cause of otological symptoms (apart from otalgia) and of audiometric findings in temporomandibular disorder patients is postulated to be an altered middle-ear to inner-ear pressure equilibrium.


2019 ◽  
Vol 50 (1) ◽  
pp. 16-33 ◽  
Author(s):  
Elizabeth Runnion ◽  
Shelley Gray

PurposeChildren with hearing loss may not reach the same level of reading proficiency as their peers with typical development. Audiologists and speech-language pathologists (SLPs) have important roles to play in preventing this problem early in children's development. In this tutorial, we aim to communicate how the habilitation practices of audiologists and intervention services of SLPs can support early literacy skill development in children with hearing loss.MethodWe describe key findings from peer-reviewed research articles to provide a review of early literacy skill development, to explain the relationship between early literacy skills and conventional reading skills, and to highlight findings from early literacy skill intervention studies that included children with hearing loss who use spoken language. We conclude with a hypothetical case study to illustrate how audiologists and SLPs can support early literacy acquisition in children with hearing loss.ConclusionFindings from studies of young children with hearing loss suggest that a promising approach to improving reading outcomes is to provide explicit early literacy instruction and intervention.


2013 ◽  
Vol 20 (3) ◽  
pp. 91-106 ◽  
Author(s):  
Rachel Pizarek ◽  
Valeriy Shafiro ◽  
Patricia McCarthy

Computerized auditory training (CAT) is a convenient, low-cost approach to improving communication of individuals with hearing loss or other communicative disorders. A number of CAT programs are being marketed to patients and audiologists. The present literature review is an examination of evidence for the effectiveness of CAT in improving speech perception in adults with hearing impairments. Six current CAT programs, used in 9 published studies, were reviewed. In all 9 studies, some benefit of CAT for speech perception was demonstrated. Although these results are encouraging, the overall quality of available evidence remains low, and many programs currently on the market have not yet been evaluated. Thus, caution is needed when selecting CAT programs for specific patients. It is hoped that future researchers will (a) examine a greater number of CAT programs using more rigorous experimental designs, (b) determine which program features and training regimens are most effective, and (c) indicate which patients may benefit from CAT the most.


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