Prosthetic management of an 11-year-old patient with hereditary ectodermal dysplasia and partial anodontia – a case report.

Objective: To illustrate the problems of partial anodontia and replacement of missing teeth and bone in patients with ectodermal dysplasia.Method: The illustrative case is an 11year old boy with tuberculoid shaped 11, 21 and 16 as the teeth present in the upper arch with anodontia of lower arch. Result: Upper removable partial denture and lower removable complete denture was fabricated and fitted satisfactory. Conclusion: The psychological and social embarrassment suffered by children with ectodermal dysplasia associated with missing teeth can be greatly improved with early prosthetic rehabilitation with removable prostheses. Further management with fixed prosthesis may be undertaken as they grow older.Key words: Ectodermal dysplasia, Prosthetic treatment

Anhidrotic ectodermal dysplasia: a case report in a Nigerian child and literature review

This report of Hereditary anhidrotic ectodermal dysplasia (HAED), a genetic disorder characterized by abnormalities of structures of ectodermal origin, was informed by its rarity, and its import for survival in a tropical environment. The five-year old male was first seen on account of inability to cut the front teeth, and a persistent offensive nasal discharge. He had heat intolerance and inability to perspire from early infancy. Pedigree evaluation revealed that both parents are Nigerians and unrelated, but the maternal front dentition was visibly defective. A 19-year old female sibling needed dentures at 10 years of age, while the father was one of two survivors out of 12 children, eight of whom were males. Findings included hypotrichosis; “saddle-nose” deformity and an offensive nasal discharge; the skin was thin, warm and dry; he had no incisors and canines, but had a single erupted premolar on either side and radiographic evidence of unerupted premolars was found. Genetic counseling and parental anticipatory guidance were offered, as was antimicrobial treatment for the co-morbid atrophic rhinitis. Dentures were deferred on the dentist's advice. This case report of HAED in a Nigerian was aimed at raising the local index of clinical suspicion by highlighting the reality of rarities, even with inadequate diagnostic support. The diagnostic parameters, literature review and the management strategies are discussed.Key words: Anhidrotic ectodermal dysplasia; hypotrichosis; oligodontia;Nigeri

Dental Management of Ectodermal dysplasia: A case report

A variety of syndromes/disorders (genetic/acquired) are encountered in our day-to-day life. Among them, ectodermal dysplasia is a rare syndrome which is transmitted as x-linked recessive/dominant disorder and is known to majorly affect males as compared to females. Clinically, it is observed that there is presence of partial or complete anodontia with conical teeth along with maxillary retrusion, prominent supraorbital ridges or frontal bossing, fine and blond, scanty hair (resembling lanuogo), deformed or absent nails, facial physiognomy (fairy-like face) and reduced sweating. Early diagnosis remains the key for the management of ectodermal dysplasia. Since patient suffers from unpleasant appearance (due to partial or complete anodontia); hence, dental management includes replacement of teeth by either fixed/removable partial or complete dentures /implant- supported dentures. We present a case of ectodermal dysplasia with features of oligodontia, severely deficient alveolar processes and maxillary retrusion which was managed with tooth-supported complete overdentures

Hypohidrotic (anhidrotic) ectodermal dysplasia in female twins

Autosomal recessive hereditary ectodermal dysplasia (HED) has not been described in sub‑Saharan Africa. It is acknowledged to be rarer than the occasionally reported x‑linked and autosomal dominant variants. We report a pair of Nigerian female twins with family history and clinical features suggestive of recessive HED, thereby showing the existence of this rare form in sub‑Saharan Africa.Keywords: Anhidrotic, dysplasia, ectodermal, female, hypohidroticNigerian Journal of Clinical Practice •Jan-Mar 2012 • Vol 15 • Issue

Prosthodontic management of hypohidrotic ectodermal dysplasia: a case report

Introduction: Ectodermal dysplasia (ED) is a hereditary disorder associated with developmental disorders of two or more structures of ectodermal embryonic origin. Hypodontia or anodontia of the primary and permanent dentition, poorly developed alveolar ridges and improper maxillo – mandibular relations, are the most common oral manifestations. Management of patients with ectodermal dysplasia requires a multidisciplinary team approach. Case presentation: A 6.5 year-old boy with hypohidrotic ectodermal dysplasia (HED) presented in this article, had typical features of HED: hypohidrosis, hypotrichosis, severe hypodontia, atrophic alveolar ridges, old-looking facial expression. According to the patient’s age and clinical findings, removable complete over-denture prosthesis in both arches was the treatment of choice. Conclusion: In patients with ED, it is important to establish correct maxillo – mandibular relations and normal function of the dento-facial system (chewing, swallowing, and speaking). Prosthodontic treatment has a major impact on aesthetics and functions,facilitates psychological development and improves emotional condition and social life of the patient

Displasias Ectodérmicas

Ectodermal dysplasias are a heterogeneous group of rare inherited disorders. Molecular findings and clarification of cell sig naling processes and ectodermal-mesenchyme interaction enabled the development of a clinical-functional model, which in turn helps to explain clinical signs, with variability in severity, associated non-ectodermal abnormalities and overlap seen in many patients. We herein review the current state of knowledge regarding this distinct entity and illustrate with an elucidative case report. The need for early multidisciplinary intervention is highlighted, and further studies will focus on genetically-target therapeutic approaches.As displasias ectodérmicas representam um grupo heterogéneo de doenças hereditárias raras. Os achados moleculares e o esclarecimento dos processos de sinalização celular e da interação ectoderme-mesênquima permitiram compreender os sinais clínicos. Estes caracterizam-se por gravidade variável, observando-se associação a anomalias não ectodérmicas e sobreposição clínica em muitos pacientes. No presente trabalho resumimos o estado atual do conhecimento sobre as displasias ectodérmicas e apresentamos ainda um relato de caso ilustrativo. Salientamos a necessidade de intervenção multidisciplinar precoce, sendo necessários estudos futuros com enfoque em abordagens terapêuticas geneticamente direcionadas.info:eu-repo/semantics/publishedVersio

Removable Partial Dentures vs Overdentures in Children with Ectodermal Dysplasia: Two Case Reports

Ectodermal dysplasia (ED) represents a disorder group characterised by abnormal development of the ectodermal derivatives. Removable partial dentures (RPD), complete dentures (CD) or overdentures (OD) are most often the treatment of choice for young affected patients. Prosthetic intervention is of utmost importance in the management of ED patients, as it resolves problems associated with functional, aesthetic, and psychological issues, and improves a patient’s quality of life. However, few studies present the principles and guidelines that can assist in the decision-making process of the most appropriate removable prosthesis. The purpose of this study was to suggest a simple treatment decision-making algorithm for selecting an effective and individualised rehabilitative treatment plan, considering different parameters

Diffuse palmoplantar keratoderma, onychodystrophy, universal hypotrichosis and cysts

No abstract availabl