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Dental Rehabilitation of a Child with Ectodermal Dysplasia: A Case Report.

ABSTRACT: ABSTRACTA nine-year-old girl was presented to the dental clinic at King Abdulaziz Medical City in Jeddah with carious posterior primary teeth and discolored teeth. She was referred to a pediatric geneticist to rule out any medical conditions. She was suspected to have ectodermal dysplasia (ED). DNA testing was requested and sent to Germany to confirm the clinical findings. Afterward, full-mouth rehabilitation was done including prophy and fluoride application, composite restorations, stainless steel crowns, and pulp therapy. The treatment was planned to be carried out under local anesthesia due to the cooperative behavior of the child and the parents’ request. Treatment objectives were oriented to achieve esthetic and functional rehabilitation of all carious teeth, maintain occlusion, in addition to maintaining spaces for the missing permanent teeth for future replacement. The patient became caries-free. Oral hygiene was improved which resulted in gingival inflammation resolution. Spaces were maintained and the behavior of the child was improved during recall visits from positive to definitely positive. Multidisciplinary approach in the management of oral and dental manifestations associated with ED including pediatric dentists, prosthodontists, and orthodontists is of paramount importance.How to cite this articleGholman RR, Kassar WM, et al. Dental Rehabilitation of a Child with Ectodermal Dysplasia: A Case Report. Int J Clin Pediatr Dent 2019;12(4):362–365.

SUBMITTER: Gholman RR

PROVIDER: S-EPMC6898874 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Project description:IntroductionAn analysis was made of three different syndromes associated with p63 gene mutations, known as ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC), ankyloblepharon-ectodermal dysplasia clefting syndrome (AEC or Hay-Wells) and Rapp-Hodgkin syndrome (RHS). The postoperative complications associated with their cleft reconstructions were also evaluated.Materials and methodsExtensive demographic information, in particular of the clinical appearances, associated malformations, and the types and complications of the reconstructive surgical procedures, were recorded of these syndromic cases occurring in a database of 3621 facial cleft deformity patients. The data was analyzed using the Microsoft Excel program.ResultsA total of 10 (0.28%) cases of p63 associated syndromes were recorded: EEC (6), RHS (3), and AEC (1). The following clinical cleft appearances were noted - EEC = 6: CLA 1 -right side unilateral (female); CLAP 4 - right side (1) + left side (1) unilateral (male + female); bilateral (2) (males); hPsP 1 (female) (divided in 3 Black, 2 White, 1 Indian); RHS = 3: CLAP 2 (White males); hPsP 1 (White female); AEC = 1: CLAP bilateral (White male). Other features of the syndromes were: skin, hand, foot, tooth, hair and nail involvement, and light sensitivity. Postoperative complications included: (i) stenosis of nasal opening, especially after reconstruction of the bilateral cleft lip and the columella lengthening (2 cases), (ii) premaxilla-prolabium fusion (2 cases), (iii) repeated occurrence of oro-nasal fistula in the hard palate (4 cases), and (iv) dysgnathial development of midfacial structures (3 cases).DiscussionThree different p63 associated syndromes (EEC, AEC, and RHS) were diagnosed (0.27% of the total facial cleft deformities database). The majority of the cases presented with a bilateral CLAP in males only. A number of females and males had unilateral CLA. The hPsP-cleft was recorded in females only. The associated ectodermal component most probably had a profoundly negative influence on postoperatively wound healing, which was observed in particular at the nasal openings, the premaxilla sulcus and in the hard palate mucosa. The reconstruction of p63 associated syndromes is a greater challenge than the usual cleft reconstruction to the surgeon.

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Dental Rehabilitation of a Child with Ectodermal Dysplasia: A Case Report.

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