Nejabi Mohammad Bashir, Anwari Abdurrahman, Shadab Hassina, Mtawakel Nargis, Omarzad Fariha, Ahmadi Mohammad Eissa
Prosthodontics Department, Kabul University of Medical Sciences (KUMS), Kabul, Afghanistan.
Operative/ Restorative Dentistry and Endodontics Department, Kabul University of Medical Sciences (KUMS), Kabul, Afghanistan.
Clin Cosmet Investig Dent. 2023 Aug 7;15:133-141. doi: 10.2147/CCIDE.S419939. eCollection 2023.
Ectodermal dysplasia (ED) is an inherited disorder that affects the ectoderm of a developing embryo and impacts structures that originate from it. It typically presents as a triad of missing teeth (anodontia/hypodontia), sparse hair (atrichosis/hypotrichosis), and lack of sweat glands (hypohidrosis), often accompanied by nail dystrophy and palmoplantar hyperkeratosis. There are two main types of this condition: X-linked anhidrotic or hypohidrotic and hidrotic (autosomal type). The oral manifestation of ED may include anodontia or hypodontia, with or without cleft lip and palate. Tooth loss leads to a decrease in the height of the alveolar ridges, resulting in a reduction of the vertical dimension of the lower face, disappearance of the vermilion border, and prominent lips. As a result, the affected person's face may resemble that of an elderly individual. The current case report aims to illustrate the prosthodontic rehabilitation of 16-year-old male patient with ectodermal dysplasia and complete anodontia, visiting Dentistry Teaching Hospital, Kabul, Afghanistan. Fortunately, this patient was acceptably managed with the collaboration of several disciplines.
外胚层发育不全(ED)是一种遗传性疾病,会影响发育中胚胎的外胚层,并影响起源于该外胚层的结构。它通常表现为三联征,即牙齿缺失(无牙症/少牙症)、头发稀疏(无毛症/少毛症)和汗腺缺乏(少汗症),常伴有指甲营养不良和掌跖角化过度。这种疾病主要有两种类型:X连锁无汗型或少汗型和有汗型(常染色体型)。ED的口腔表现可能包括无牙症或少牙症,伴有或不伴有唇腭裂。牙齿缺失导致牙槽嵴高度降低,进而导致下脸垂直尺寸减小、唇红缘消失和嘴唇突出。因此,患者的面部可能类似老年人。本病例报告旨在说明一名16岁患有外胚层发育不全和完全无牙症的男性患者在阿富汗喀布尔牙科教学医院接受的口腔修复治疗。幸运的是,通过多学科合作,该患者得到了妥善治疗。