Prosthetic rehabilitation with fixed prosthesis of a 5-year-old child with Hypohidrotic Ectodermal Dysplasia and Oligodontia: a case report.

BACKGROUND: Ectodermal dysplasia is a rare genetic disorder that affects ectodermally derived structures, including teeth, nails, hair, and sweat glands. Hypohidrotic ectodermal dysplasia is the most common type, with oligodontia being the most striking dental feature. Prosthetic rehabilitation in children with ectodermal dysplasia is an important step toward improving their overall quality of life. The fixed prosthesis has the advantages of being more stable in the mouth with good child compliance and a good aesthetic outcome. CASE PRESENTATION: Our patient was a 5-year-old Middle Eastern boy with oligodontia caused by ectodermal dysplasia. He was managed by fabrication of an upper functional space maintainer and a lower fixed partial denture to restore occlusion, masticatory function, aesthetics, and overall quality of life. CONCLUSIONS: The use of the fixed prosthesis in children is a new and evolving treatment modality that resolves many of the issues caused by removable prostheses. It accommodates jaw growth in the mandible, reduces the need to remake the prosthesis, and has an overall better aesthetic outcome.

Polymer-Infiltrated-Ceramic-Network, CAD/CAM Restorations for Oral Rehabilitation of Pediatric Patients with X-Linked Ectodermal Dysplasia.

Functional and esthetic oral rehabilitation of young patients affected by ectodermal dysplasia is traditionally performed with direct composite restorations, which encounter various limitations. However, recent advances in computer-aided design/computer-assisted manufacturing (CAD/CAM) composites have led to the introduction of high-performance materials. In the present case report, a 9-year-old patient was treated with 20 CAD/CAM partial and peripheral restorations in polymer-infiltrated-ceramic-network material to restore deciduous and permanent teeth. The restorations were minimally invasive, since they did not require any tooth tissue preparation. Appropriate occlusal relationships with increased vertical dimension of occlusion were obtained. Tooth anatomy and esthetics were significantly improved, and chairtime was reduced.

Prosthetic rehabilitation of patients with hypohidrotic ectodermal dysplasia: A systematic review.

Hypohidrotic ectodermal dysplasia (HED) comprises a large group of inherited disorders of ectodermal structures, characterised by hypo- or anhidrosis, hypotrichosis and hypo- or oligo- or anodontia. We aimed to systematically assess the spectrum of prosthodontic approaches with regard to the patients' age and to provide clinical implications for practicing dentists. An electronic and manual search was conducted in four databases (Medline, LIVIVO, Cochrane Library, Web of Science Core Collection). Publications of multiple study designs written in English or German without data restrictions, reporting on prosthodontic treatment of patients diagnosed with HED and afflicted with oligo- or anodontia, were included. In total, 75 articles on 146 patients were analysed according to the patients' age. In children aged 2-17 years, removable full or partial (over)dentures represented standard treatment. In the mandible, implant-supported removable dentures on two interforaminal implants presented an alternative, already in young childhood. In cases with more than six teeth per jaw, also fixed (resin) bridges were used, frequently after orthodontic treatment. In adults, fixed or removable reconstructions with the help of up to eight implants per jaw, usually placed after bone augmentation procedures, were standard. Ten case reports/series with long-term follow-up illustrated the need for consistent maintenance including denture renewals. Prosthodontic rehabilitation should start in early childhood and needs to be revised in accordance with the patients' growth. Treatment should be carried out by a multidisciplinary team addressing variable demands in different age groups.

Speech prosthesis in child patient with ectodermal dysplasia. Case report.

Fabrication of several esthetic speech prostheses in a 4-year-old child patient with ectodermal dysplasia is reported. This report details, clinically and radiographically, effects in a child diagnosed with hypohidrotic ectodermal dysplasia, as well as speech device construction.

Oral rehabilitation of children with ectodermal dysplasia.

The aim of this study was to describe the clinical treatment of young patients, affected by ectodermal dysplasia (ED), and to possibly establish clinical guidelines. The study design was case series. ED syndromes (EDs) are a heterogeneous group of inherited diseases characterised by abnormal development of tissues of ectodermal origin. The most common form of EDs is X linked hypohidrotic ED (HED). Characteristic triad of HED is oligo-anodontia, hypotricosis, hypo-anhydrosis. Oligo-anodontia is one of the most severe impairment, since it affects chewing, swallowing, speech, esthetics and social relation. Early prosthetic rehabilitation (at 2-3 years of age), with partial or complete dentures, is essential to improve oral function and reduce the social impairment.

Immediate loading in ectodermal dysplasia: clinical case.

The aim of the present report is to describe a clinical case of ectodermal dysplasia (ED) treated with an immediate implant-loading protocol. Six implants were placed in freshly extracted sockets of a 55-year-old patient with ED and immediately loaded in 48 hours. No immediate implant-loading protocol in a patient with ED has been reported. Implant management is difficult because of knife-edge ridges and pathologic 3-dimensional relation of the jaws. In the present case, implant non-parallelism caused by alveolar ridge atrophy was managed using a specific prosthetic connection device that simplified the surgical and prosthetic procedure and allowed a low-risk immediate-loading protocol.

Rehabilitación odontológica en la displasia ectodérmica hipohidrótica / Dental rehabilitation in hypohidrotic ectodermal dysplasia

La displasia ectodérmica pertenece a un grupo heterogéneo de trastornos congénitos. La más frecuente es la displasia ectodérmica hipoidrótica, hipotricosis y alteraciones dentarias. La disminución de la cantidad de piezas dentarias, al igual que las anomalías en la forma de las mismas(dientes conoides), son relevantes para el diagnóstico y tratamiento odontológico. La reheabilitación debería comenzar a edades tempranas, alrededor de los 3 años. Consiste en reponer las piezas dentarias ausentes con prótesis de acrílico, las cuales deben renovarse periódicamente. De esta manera, se logra aumenttar la dimensión vertical, regular las funciones foniátricas y masticatorias, mejorando así la dicción y la autoestima de los pacientes. Para ello, de fundamental importancia el rol del dentista pediatra en el manejo interdisciplinario del niño afectado. Cuando finalice la etapa de crecimiento, se optará por el tratamiento definitivo más adecuado.

Ectodermal dysplasia is a heterogeneous group of congenital disorders. Hypohidrotic ectodermal dysplasia is the most common type. It is characterized by the triad of signs comprising hypohidrosis, hypotrichosis and dental abnormalities. The lack of teeth and conical or pointed teeth are important for diagnosis and clinical management. Treatment should be started as soon as possible. Early placement of partial or full dentures is commonly recommended from the age of three years. Removable protheses must be periodically modified. Prosthodontic rehabilitation is fundamental in these situations, attempting to provide a functional and aesthetic solution and to control the vertical dimension, which will improve patient’s diction and self-steem. For these reasons it is important to consider the role of the paediatric dentist in interdisciplinary management of the affected child patient. At the time the patient finishes growing, the possibility of definite treatment should be considered.

Hypohidrotic ectodermal dysplasia: a unique approach to esthetic and prosthetic management: a case report.

Hypohidrotic ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures. The condition is usually transmitted as an x-linked recessive trait, in which gene is carried by the females and manifested in males. Manifestations of the disease differ in severity and involve teeth, skin, hair, nails and sweat and sebaceous gland. Ectodermal dysplasia is usually a difficult condition to manage. Prosthodontically, because of the typical oral deficiencies, and afflicted individuals are quite young to receive extensive prosthodontic treatment, which restores their appearance and helps them, for the development of positive self-image. This case report describes the management of upper jaw with over denture with copings on existing teeth i.e. two permanent peg shaped centrals as well as lateral incisors. However with adequate of retainer lower denture was provided with a new treatment modality.