scholarly journals Noninvasive Multi-Disciplinary Management of Ectodermal Dysplasia with Hyposalivation – A Case Report

2021 ◽  
Vol 14 (4) ◽  
pp. 1409-1412
Author(s):  
Sana Alhamed
Keyword(s):  
Ectodermal Dysplasia ◽  
Dental Treatment ◽  
Treatment Plan ◽  
Well Being ◽  
Management Plan ◽  
Child Growth ◽  
Sweat Glands ◽  
Interdisciplinary Management ◽  
Early Assessment ◽  
Facial Height

Ectodermal dysplasia (ED) is a rare genetic condition with nearly 200 documented traits. As the name states, ED targets tissues derived from ectoderms, such as hair, skin, nails, sweat glands, and teeth. Other orofacial structures might be affected, such as salivary glands and hard palate. Lack of teeth and diminished facial height can impact negatively on child growth and psychological well-being. Therefore, assessment and an interdisciplinary management plan of orofacial components of ED children should be installed as early as possible. Here we report an early assessment and multi-disciplinary management of ED child’s orofacial structures, which allow restoration of facial height and dental function and saliva reduction by the least invasive restorative approach in the form of the composite build-up of microdont and overdentures. It also highlights the importance of periodic evaluation of growth and treatment plan adjustment as an integral part of the transitional management until the age of a definite dental treatment.

Full Mouth Fixed Rehabilitation of a Young Adult with Ectodermal Dysplasia: Making Mountain Out of a Molehill in The Literal Sense

2020 ◽  
Author(s):  
Vaishnavi Rajaraman
Keyword(s):  
Ectodermal Dysplasia ◽  
Treatment Plan ◽  
Sweat Glands ◽  
Congenital Diseases ◽  
Facial Esthetics ◽  
Key Factor ◽  
Full Mouth Rehabilitation ◽  
Fixed Prosthesis ◽  
Multiple Abnormalities ◽  
Hereditary Condition

The personality of an individual is often judged by his looks. A beautiful smile brings immense pleasure, not only to the viewer but also to the wearer of the smile. Time and again in dentistry, esthetics has been the prime area of focus. Prosthodontic rehabilitation also involves esthetics as its key factor, which is challenging especially in patients with congenital diseases or syndromes. Ectodermal dysplasia is one such hereditary condition associated with failure of the development of ectoderm in the embryonic stage and can cause multiple abnormalities. The ectodermal structures like skin, nails, hair, sweat glands, and teeth are particularly affected. The management of ectodermal dysplasia is quite complex and multidisciplinary owing to the abnormal morphology in craniofacial structures, a wide array of dentofacial defects, and age of the affected individuals, as most of them are very young when they report for or are evaluated for treatment. The deciduous as well as the permanent dentition in these patients may present with anodontia, hypodontia or oligodontia or combination of these. Hypodontia is one of the most common intraoral finding among these scenarios. Therefore, the affected patients obviously require absolute attention towards prosthodontic treatments throughout their developmental years till their adulthood. This report presents a case of an adult diagnosed with having ectodermal dysplasia with hypodontia. By appropriate modification and customization, the prosthodontic management was meticulously planned for the young patient which involved a full mouth rehabilitation with tooth supported fixed prosthesis in the maxillary and mandibular arch. The devised treatment plan had a key impact on the psychological, the masticatory function, speech and facial esthetics of the patient.

Treatment strategy for patients with ectodermal dysplasia

2005 ◽  
Vol 29 (2) ◽  
pp. 113-118 ◽  
Author(s):  
Jack W. Martin ◽  
Nicholas Tselios ◽  
Mark S. Chambers
Keyword(s):  
Ectodermal Dysplasia ◽  
Treatment Plan ◽  
Abnormal Development ◽  
Sweat Glands ◽  
Clinical Report ◽  
Stomatognathic System ◽  
Ectodermal Dysplasias ◽  
Different Types ◽  
Anhidrotic Ectodermal Dysplasia ◽  
Hereditary Condition

Ectodermal dysplasia (ED) is a hereditary condition characterized by abnormal development of the skin, hair, nails, sweat glands, and the stomatognathic system. There are many different types of ectodermal dysplasia of which X-linked anhidrotic ectodermal dysplasia is the most common. Multiple genes have been discovered to cause ectodermal dysplasias. With any form of ED, children may display a range of symptoms and challenging rehabilitation. This clinical report presents the treatment plan for a young patient with ED and anodontia requiring prosthetic restoration. J Clin Pediatr Dent 29(2): 113-118,2005

Interdisciplinary Management of Patients With Ectodermal Dysplasia

2010 ◽  
Vol 36 (3) ◽  
pp. 239-245 ◽  
Author(s):  
Joseph E. Van Sickels ◽  
Ted P. Raybould ◽  
E. Preston Hicks
Keyword(s):  
Ectodermal Dysplasia ◽  
Treatment Plan ◽  
Maxillofacial Surgery ◽  
Current Therapy ◽  
Interdisciplinary Management ◽  
Oral And Maxillofacial Surgery ◽  
Coordinated Care ◽  
University Of Kentucky ◽  
Staged Treatment ◽  
The University

Abstract A 15-year-old girl with ectodermal dysplasia who had been treated previously with minimal orthodontic intervention and removal prosthesis was seen and evaluated at the General Practice Program at the University of Kentucky. After consultations with orthodontics and oral and maxillofacial surgery, an interdisciplinary staged treatment plan was developed to address her skeletal and dental issues. The authors briefly review the literature with respect to current therapy for patients with ectodermal dysplasia and present the case as an example of the coordinated care provided for a patient with complex skeletal and dental issues.

Rapp-Hodgkin Syndrome: Clinical and Dental Findings

2009 ◽  
Vol 34 (1) ◽  
pp. 71-75 ◽  
Author(s):  
Gul Tosun ◽  
Ulku Elbay
Keyword(s):  
Cleft Palate ◽  
Ectodermal Dysplasia ◽  
Dental Treatment ◽  
Sweat Glands ◽  
Oral Manifestations ◽  
Steel Wool ◽  
Rare Form ◽  
Dental Rehabilitation ◽  
Delayed Eruption ◽  
Sparse Hair

Rapp-Hodgkin syndrome is a rare form of ectodermal dysplasia involving the hair, eyes, sweat glands, nails,teeth and palate. This syndrome is characterized by stiff, sparse hair with the appearance of steel wool,sparse eyebrows and lashes, cleft palate, absence of lacrimal punctae, epiphora, a decreased number of sweat glands, and dystrophic nails. Cleft palate, hypodontia, abnormal tooth shape, multiple caries, delayed eruption of teeth are the main oral manifestations. In this paper we describe the clinical and dental findings of this syndrome in a 7 year old girl referred to the dental clinic because of severe tooth ache. The dental treatment included root canal treatment, composite restorations, extractions and removable prostheses. This paper highlights features of Rapp-Hodgkin Syndrome and its dental rehabilitation.

scholarly journals Direct Composite Resin Restoration of Anterior Maxillary Teeth and Esthetic Smile Design: A Case Report and Esthetic Smile Design: A Case Report

2020 ◽  
Vol 27 (2) ◽  
Author(s):  
Christina Widya Nugrohowati ◽  
◽  
Wignyo Hadriyanto ◽  
Tunjung Nugraheni ◽  
◽  
...  
Keyword(s):  
Case Report ◽  
Male Patient ◽  
Composite Resin ◽  
Dental Treatment ◽  
Treatment Plan ◽  
Well Being ◽  
Composite Resins ◽  
Minimally Invasive Dentistry ◽  
Anterior Teeth ◽  
Composite Resin Restoration

Dental esthetic complex involves more than one caries with malformed shape and malposition. Dental trauma most commonly affects maxillary anterior teeth, and subsequently affects the esthetics, functions, and psychological well-being of the individual. Composite resins have become an integral part of contemporary restorative and minimally invasive dentistry. Objective: To summarize the successful use of direct composite resin restoration in the management of a 29-year old male patient. Case Report: A 29-year old male patient presenting with multiple secondary caries and crown fractures in the anterior maxillary teeth, resulting in an unaesthetic smile. Comprehensive esthetic dental treatment was carried out. The patient was highly satisfied with the results even after eleven months of follow-up. Conclusion: restorative success with composite resins, the dental treatment plan should be carefully executed with attention to factors such as dental convexities and contours, re‑establishment of function, and esthetics. Smile design should always be a multifactorial decision-making process that allows the clinician to treat patients with an individualized and interdisciplinary approach.

scholarly journals Prosthetic rehabilitation of a hypohidrotic ectodermal dysplasia patient: A case report

2021 ◽  
Vol 11 (Suppl. 1) ◽  
pp. 292-298
Author(s):  
Dersim Gökçe ◽  
Emrah Ayna ◽  
Zelal Seyfioğlu Polat
Keyword(s):  
Case Report ◽  
Ectodermal Dysplasia ◽  
Dental Treatment ◽  
Sweat Glands ◽  
Prosthetic Rehabilitation ◽  
Hypohidrotic Ectodermal Dysplasia ◽  
Invasive Treatment ◽  
Homozygous Carrier ◽  
School Of Dentistry ◽  
Sparse Hair

Aim: Ectodermal dysplasia is a rare hereditary disease that arises from a developmental disorder of 2 or more ectoderm-derived tissues. Ectodermal dysplasia is seen in 3 different types: anhidrotic, hypohidrotic, and hidrotic. Its anhidrotic and hypohidrotic types are the most common. This study presents the intraoral findings and dental treatment approach of a case diagnosed with hidrotic ectodermal dysplasia that demonstrates the typical characteristics of the disease, such as anodontia, hypohydrosis (reduced sweating), hypotrichosis (sparse hair), and loss of vertical dimension. Methodology: A 5-year-old male patient presented to the clinic of the Prosthetic Dental Treatment Department of the School of Dentistry at Dicle University on 25.10.2020 with complaint of missing teeth. A genetic analysis conducted in 2016 showed that he was a homozygous carrier of the p.Cys148Arg (c.442 T>C) mutation on the 5th exon of the ectodysplasin-A receptor (EDAR) gene. The mutation detected in the patient was associated with ectodermal dysplasia. An extraoral clinical examination revealed sparse hair, eyebrows, and eyelashes; soft, smooth, and dry skin; thin, linear wrinkles around the eyes and the lips; drooping, thickened lips; a sunken nose; fractured nails; hyperthermia due to lack of sweat glands; hyperkeratosis in the skin and soles of the feet; 2 nipples on one side of the chest; and reduced vertical facial height. An intraoral examination revealed anodontia; there were no teeth on the maxilla or the mandible and no radiographically identified tooth germ. Dry mouth due to a lack of sufficient saliva was another finding. Conclusion: In this case report, in the presence of anodontia, a removable total prosthesis, which is a non-invasive treatment option, was applied. Production of endosseous implants was postponed for a later time following the patient’s growth and development.   How to cite this article: Gökçe D, Ayna E, Seyfioğlu Polat Z. Prosthetic rehabilitation of a hypohidrotic ectodermal dysplasia patient: A case report. Int Dent Res 2021;11(Suppl.1):292-8. https://doi.org/10.5577/intdentres.2021.vol11.suppl1.43   Linguistic Revision: The English in this manuscript has been checked by at least two professional editors, both native speakers of English.

Possibilities and prospects in early orthopaedic rehabilitation of children with ectodermal dysplasia and congenital edentulism. clinical features and practical experience

2020 ◽  
pp. 40-50
Author(s):  
V.G. Galonsky ◽  
N.V. Tarasova ◽  
E.S. Surdo ◽  
A.V. Gradoboev
Keyword(s):  
Preschool Children ◽  
Clinical Features ◽  
Ectodermal Dysplasia ◽  
Dental Treatment ◽  
Practical Experience ◽  
Dental Clinic ◽  
Pedagogical Tools ◽  
Psychological Types ◽  
Clinical Cases

The article is devoted to the issue of early orthopaedic rehabilitation of youngest preschool children with ectodermal dysplasia and congenital edentulism. The essence and details of children’s psychophysiological development at this age are revealed, as well as main pedagogical tools making it possible to adapt the children to the specific environment of the dental clinic and motivate them for undergoing long-term multistage orthopaedic dental treatment. Clinical approaches and peculiarities in the dentist’s work with children of different psychological types as well as particular aspects in their adaptation to removable dentures are described. Two clinical cases of effective orthopaedic rehabilitation of 3-years-old children with ectodermal dysplasia and congenital edentulism using full removable lamellar dentures.

scholarly journals Risk Management in nursing homes: an Italian experience in 2019

2020 ◽  
Vol 30 (Supplement_5) ◽  
Author(s):  
M F Furmenti ◽  
F Bert ◽  
M Rucci ◽  
U Fiandra ◽  
A Scarmozzino ◽  
...  
Keyword(s):  
Risk Management ◽  
Nursing Homes ◽  
Management Practices ◽  
Well Being ◽  
The Elderly ◽  
Management Plan ◽  
Management Policy ◽  
Insurance Company ◽  
Term Care ◽  
Starting Point

Abstract Background The ageing of the European population leads to an increasing demand for Long-Term Care services. The security and well-being of the elderly population hosted in nursing homes (NHs) needs an effective Risk Management policy, officially sanctioned in Italy by the so-called “Legge Gelli” n.24 (March 8th, 2017) and the Directive 2011/24/EU on the application of patients' rights in cross-border healthcare. In order to verify the effective application of common “best practices” in terms of Risk Management in NHs, a tool useful to analyse risk management attitudes in Northern Italy was conceived and applied in a sample of NHs. Methods The tool, developed in collaboration with the health insurance company SHAM Italia, is composed of 124 items (with a dichotomous answer -YES/NO) on topics related to various Risk Management practices. This tool was submitted in a face-to-face interview to several Directors (Health Directors or Nursing Coordinators) of NHs in the Piedmont Region. A list of randomly-chosen NHs was contacted: 4 of them were selected for the pilot study and compiled the questionnaire. Answers were gathered and analyzed through Microsoft Excel. Results Only the 25% of NHs has a Risk Management plan with objectives and indicators of effectiveness and uses Risk Analysis instruments for a pre- and post-” risk detection. Only one has employees working mainly on Risk Management alone. The 75% of the reported events were “Adverse Events”, and all the NHs (100%) have a protocol for a patient voluntary departure or for fall prevention or for bedsores prevention; while 50% have a protocol for prevention of aggressions towards operators or for patients' suicide prevention. Conclusions This work provides a starting point to face new challenges that are looming on the European Health-care Systems: the care for the elderlies needs to be perfected to reduce inefficiencies, cut useless costs and improve safety of patients in the NHs setting. Key messages Despite safety of older patients in nursing homes is not only important but mandatory in Italy, risk management tools for this setting are lacking in literature. A new tool applied in Italian nursing homes showed that risk management needs to be implemented in practice and these results can be extended to European context.

Diagnostic accuracy and clinical management by realtime teledermatology. Results from the Northern Ireland arms of the UK Multicentre Teledermatology Trial

1998 ◽  
Vol 4 (2) ◽  
pp. 95-100 ◽  
Author(s):  
M A Loane ◽  
R Corbett ◽  
S E Bloomer ◽  
D J Eedy ◽  
H E Gore ◽  
...  
Keyword(s):  
Diagnostic Accuracy ◽  
Clinical Management ◽  
Low Cost ◽  
Treatment Plan ◽  
Management Plan ◽  
Face To Face ◽  
Management Plans ◽  
Video Link ◽  
The Uk ◽  
Management Recommendations

Diagnostic accuracy and management recommendations of realtime teledermatology consultations using low-cost telemedicine equipment were evaluated. Patients were seen by a dermatologist over a video-link and a diagnosis and treatment plan were recorded. This was followed by a face-to-face consultation on the same day to confirm the earlier diagnosis and management plan. A total of 351 patients with 427 diagnoses participated. Sixty-seven per cent of the diagnoses made over the video-link agreed with the face-to-face diagnosis. Clinical management plans were recorded for 214 patients with 252 diagnoses. For this cohort, 44 of the patients were seen by the same dermatologist at both consultations, while 56 were seen by a different dermatologist. In 64 of cases the same management plan was recommended at both consultations; a sub-optimum treatment plan was recommended in 8 of cases; and in 9 of cases the video-link management plans were judged to be inappropriate. In 20 of cases the dermatologist was unable to recommend a suitable management plan by video-link. There were significant differences in the ability to recommend an optimum management plan by video-link when a different dermatologist made the reference management plan. The results indicate that a high proportion of dermatological conditions can be successfully managed by realtime teledermatology.

Caring for Gravely Ill Children

PEDIATRICS ◽  
1994 ◽  
Vol 94 (4) ◽  
pp. 433-439 ◽  
Author(s):  
Alan R. Fleischman ◽  
Kathleen Nolan ◽  
Nancy N. Dubler ◽  
Michael F. Epstein ◽  
Mary Ann Gerben ◽  
...  
Keyword(s):  
Health Care ◽  
Decision Making ◽  
Health Care Professionals ◽  
Treatment Plan ◽  
Ethics Committees ◽  
Well Being ◽  
Pediatric Health Care ◽  
Spiritual Well Being ◽  
Primary Focus

Background. Much has been written about the care of the hopelessly ill adult, but there is little guidance for pediatric health care professionals in the management of children who are critically or terminally ill. Methods. Through a 3-day meeting in Tarrytown, NY, attended by a group of pediatricians and others directly involved in these issues, a principled approach was developed for the treatment of, and health care decision-making for, children who are gravely ill. Results. The group agreed that the needs and interests of the child must be the central focus of any treatment plan and that the child should be involved to as great extent possible, consistent with developmental maturity, in the decision-making process. Quality of future life should be viewed as being relevant in all decisions. Parents are believed to be the natural guardians of children and ought to have great latitude in making decisions for them. However, parental discretion is not absolute and professionals must maintain an independent obligation to protect the child's interests. Conclusions. Decision-making should be collaborative among patient, parents, and professionals. When conflict arises, consultation and ethics committees may assist in resolution. When cure or restoration of function is no longer possible, or reasonable, promotion of comfort becomes the primary goal of management. Optimal use of pain medication and compassionate concern for the physical, psychological, and spiritual well-being of the child and family should be the primary focus of the professionals caring for the dying child.

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