Prosthodontic Management of Children with Ectodermal Dysplasia: Review of Literature

Abstract

Prosthodontic Management of Children with Ectodermal Dysplasia: Review of LiteratureRashu Grover and Manjul Mehra* Department of Pedodontics and Preventive Dentistry, Sri Guru Ram Das Institute of Dental Sciences and Research, Amritsar, India gene was located to Xq 12-13 by Zonana et al. [20]. Autosomal forms of HED are due to mutation in the EDA receptor (EDAR). EDAR binds specifically the A1 isoform of EDA (EDA-A1) but not the EDA-A2 isoform that utilizes a distinct receptor. Autosomal HED may also be caused by mutation in a cytosolic, EDAR-specific adapter molecule named EDAR-associated death domain (EDARADD) [17,18,21,22].The EDA, EDAR and EDARADD genes provide instructions for making proteins that work together during embryonic development. These proteins form part of a signaling pathway that is critical for the interaction between two cell layers, the ectoderm and the mesoderm. It is essential for the formation of several structures that arises from ectoderm, including the skin, hair, nails teeth and sweat glands. Mutation in these genes prevents normal interaction between the ectoderm and mesoderm and impairs the normal development of hair, sweat glands and the teeth [7,8]. The improper formation of these ectodermal structures leads to the characteristic features of HED. In rare cases, HED is associated with immune deficiency caused by mutations in further downstream components of the EDA pathway that are necessary for the activation of the transcription factor NFkappa b [18][19][20]23,24]. Clinical ManifestationsHypohidrosis is possibly the most remarkable characteristic of ED because it may not be the apparent in the first year of life but present later as a fever of unknown origin. The inability to sweat results in intolerance to heat, occasionally causing hyperpyrexia after mild exertion or even after a simple meal [25]. Resultant high fevers may lead to seizures and other neurological sequelae. This is one of the most common causes of mortality with a 30% rate in infancy and early childhood [26]. Other problems associated are pharyngitis, rhinitis, cheilitis and dysphagia may result from decreased number of mucous glands in the respiratory and gastrointestinal tracts [27] ClassificationThere are several classifications given by different authors. Some are based on clinical features and others on genetic component of the disorder [7,8]. Clinically there are two major types of ED namely hidrotic and anhidrotic (hypohidrotic form). The hidrotic form, inherited as an autosomal trait, affects teeth, hair and nails but usually spares the sweat glands and was first described by Clouston in 1929 [9]. Whereas the hypohidrotic form (Christ-Siemens-Touraine Syndrome) is most common type seems to be an X-linked recessive trait, with an incidence of this syndrome estimated to be 1 to 7 per 10,000 live births [10,11]. Hypodontia, hypotrichosis and hypohidrosis which form a triad are the characteristic feature of the hypohidrotic form [12,13].Other attempts at classifying EDs were proposed later [2,4,7,14,15]. ...

Tissue Malformations, Characteristics and Occurrence Rates in Ectodermal Dysplasia

“…ED vakalarda sık rastlanan karakteristik intraoral bulgular; süt ve daimi dişlerde konik şekil bozukluğu, oligodonti, hipodonti, anadonti, dişsiz bölgelerde az gelişmiş alveolar kret, maksiller retrüzyon, mandibular protrüzyon, dişlerde sürme gecikmesi olup klinik tanıda oldukça önemlidir (7,11,18,26). Yaptığımız çalışmada, klinik muayene ve radyolojik incelemelerde vakaların yaş ortalaması 13,6 olarak tespit edildi, 21 kadın, 24 erkek olmak üzere toplam 45 vaka üzerinde yürütülen incelemede 29 ebeveyn arasında akrabalık, 38 vakada terleme azlığı, 29 vakada semer burun, 34 vakada ileri çıkık dolgun dudak tespit edildi.…”

“…Şimdiye kadar 170-200 farklı ED formu tarif edilmesine rağmen, moleküler düzeyde neden olan 30 civarında gen türü tanımlanıp açıklanabilmiştir (5,6,8,9,(11)(12)(13)(14)(15)(16). Kalıtsal olarak ED, yeni mutasyonlar sonucu veya otozomal dominat, otozomal resesif, X'e bağlı resesif ya da X'e bağlı dominant genetik geçiş gösterebilir (12)(13)(14)(16)(17)(18)(19)(20)(21)(22). X-linked hipohidrotik ektodermal displaziye (XLHED) genetik olarak ectodisplasin A1 (EDA1) molekül eksikliğinin sebep olduğu ve bu molekülün eksikliğinin prenatal dönemde giderilmesinin deri ve diş eksiklikleri gibi klinik bulguları engellediği ile ilgili çalışmalar yayınlanmıştır (23,24).…”

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

“…FPDs with rigid connectors are usually avoided in young, actively growing patients because they could prevent jaw growth, especially if the prosthesis crosses the midline [4]. However, transverse skeletal and alveolodental changes are less pronounced in the mandible than in the maxilla, and it is widely accepted that the lateral growth of the anterior mandible is completed by the age of 3 [13].…”

“…The National Foundation for Ectodermal Dysplasias recommends the placement of dental implants in the anterior mandible of children older than school age (7 years and older) [15]. However, the transverse growth of the maxilla continues until the age of 17 in boys when the midpalatine suture fuses, which contraindicates the use of maxillary dental implants in young patients [4]. Placement of dental implants in a growing patient carries the risk of growth cessation, implant submergence, or ankylosis.…”

“…Compared with traditional removable appliances, fixed prostheses are patient-friendly and provide a more stable and better hygienic and aesthetic result. Fixed prostheses improve speech and masticatory function and have fewer negative sequelae than other prosthetic replacements, which makes them the ideal option for young patients with multiple missing teeth [4].…”