The Early Prosthodontics Treatment For Patients With Hpohidrotic Ectodermal Dysplasia

Ectodermal dysplasias(EDs)is a general term for a complex disease with more than 200 kinds of different pathological damages,mainly involving the development defects of ectodermal tissues,such as sweat glands,hair,toenail,teeth and nervous system.According to the development of sweat glands,EDs are divided into hypohidrotic ectodermal dysplasia and hidrotic ectodermal dysplasia,among which the former is more common.Hypohidrotic ectodermal dysplasia(HED),also known as anhidrotic ectodermal dyslasia(AED),or Christ-SiemensTouraine syndrome,is a rare congenital genetic disease with the birth incidence rate about 1/ 100000,which can be sporadic or familial.Patients usually have thin hair,sweat gland hypoplasia,craniofacial abnormalities,prominent forehead,saddle nose,dry skin,hyperkeratosis of the finger or toes.Oral presentation includes hypoplasia,dysplasia and conical teeth,which seriously affecting the mastication,pronunciation and appearance of patients and is not conducive to physical and mental health of patients.The early prosthetic treatment of HED patients can not only reshape the teeth,reconstruct the occlusion,restore the mastication and pronunciation,but also promote the development of the jaw and temporomandibular joint,prevent the malocclusion,improve the facial appearance,establish a harmonious craniofacial relationship,enhance the physical and mental health and improve the quality of life.The main difficulties of the treatment of HED patient include excessive loss teeth,low alveolar ridge,poor retention the residual teeth.This study recruited four HED families through clinical examination,taken early prosthetic treatment for probands,improves the retention and stability of the denture with stainless?steel?crown,key-way attachment and fixed-removable prostheses,and observed the long-term clinical efficacy,aiming to provide reference for the clinical treatment of such patients.Part One Collection of HED families1 Materials and Methods: Four unrelated HED families who came to the department of stomatology in the Affiliated Stomatology Hospital of Nanchang University from 2016 June to 2018 April were enrolled in this study.Through detailed inquiry and clinical examination,we acquired their family histories and drawn the pedigree map respectively to understand their pathogenic characteristics.2 Results: All of these 4 families were Chinese Han population.All these 4probands were born at term with no history of consanguineous marriage in their families.However,in family one,the proband's mother has a history of abortion and pregnancy medication.A brother of proband one was died due to the recurrent fever in the first year after his birth.Another brother of proband one was died because of malignant lymphoma at the age of 4.None of other members in family one has any similar manifestation.The permanent teeth of proband one,two and three are totally missing and the number of missing deciduous teeth in the upper jaw were more than those in lower jaw,mostly concentrated in the incisor area and the first molar area.The number of permanent tooth loss of proband four was 23.The missing teeth in the mandible is more than that in the maxilla,most of which are concentrated in the anterior molar area.3 Conclusion: Combined with common genetic patterns of related diseases,we speculated the genetic characteristics of these four HED families may be recessive inheritance.The affected teeth are mainly concentrated in the incisors and premolars.The loss of the same tooth is more easier to occur in the mandible than that in the maxilla.Part Two Early prosthodontic treatment of HED patients1 Materials and Methods: Oral and facial examination,routine oral panoramic/CBCT examination,facial and intraoral image collection were acquired for HED patients.Through radiological examination,we can clearly identify the development of probands' teeth and jaws.We communicated with children and their families,andformulated corresponding treatment plans combined with their own conditions respectively.The procedures are as follows: making individual pallets and analysis models,determining the intermaxillary distance preliminary using facial proportion method,making constant base resin,obtaining and verifying the centric relation by bimanual manipulation,determining the vertical distance and occlusal relation,reforming malformed teeth,completing with 4 pairs of removable partial denture(RPD)for temporary repair treatment.Every patient should visit us every 3 months regularly to examine teeth,denture and alveolar bone,occlusal changes,and the whole body including hair growth,height and weight which can reflect the nutritional status of the body,and observe the psychologic status of the patients at the same time.The purpose is to modify or replace the denture in time when necesary.2 Results: After early prosthodontic treatment,HED patients achieved occlusal relationship,improved masticatory efficiency,appearance and pronunciation significantly.3 Conclusion: For HED patients,early prosthodontic treatment should be taken to improve the masticatory efficiency,appearance and pronunciation,stimulate alveolar bone growth,promote physical and mental health.Nevertheless,considering the young age,growth stage and unstable occlusal relationship,we need to take clinical observation continually and combine with multiple disciplines to provide such children more effective treatment.