Vol. 9, Issue 3, Part F (2023)

Background: Ectodermal dysplasia (ED) is a rare group of hereditary disorders affecting tissues derived from the ectoderm, which includes teeth, hair, nails, and sweat glands. Many pediatric patients with ED often present with notable oral and maxillofacial abnormalities, such as hypodontia, oligodontia, abnormal tooth morphology, and craniofacial deformities. Despite this rising interest in these manifestations, a systematic synthesis of the evidence regarding their prevalence, characteristics, and clinical implications is lacking.

Methods: This systematic review was carried out in accordance with PRISMA guidelines. The search was performed in six databases by applying Boolean operators and MeSH terms. Oral and maxillofacial findings in children diagnosed with ED were considered for the study, which covered case series and observational studies. The data were extracted regarding demographic characteristics, dental phenotypes, craniofacial abnormalities, and treatment outcomes, which were analyzed. The ROBINS-I tool was used to assess the bias.

Results: A total of eight studies was included, with patients from different geographic locations and subtypes of ED. Dental findings included hypodontia (82.4%), oligodontia (61.8%), and anodontia (37.6%). Morphological anomalies like conical teeth (67.2%), paurodontids (29.3%), and enamel hypoplasia (48.1%) were present frequently. Craniofacial features included reduced alveolar bone height (54.6%), vertical facial deficits (43.7%), and narrow palates (38.5%). Salivary dysfunction was present in 26.8% of the cases. Radiographic assessments showed widespread dental agenesis and delayed eruption in more than 80% of the patients. Improved functional and aesthetic outcomes were associated with early prosthetic and orthodontic rehabilitation.

Conclusion: These findings reemphasize the heterogeneity of oral and craniofacial manifestations in children with ED. There were large variations seen among subtypes and population groups. In many children, early intervention with an individually tailored approach could prevent most functional and psychosocial impairment. These findings will offer a basis for enhanced diagnostic and therapeutic strategies in handling ED.