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구개열을 동반한 저한성 외배엽형성이상 환자의악교정수술을 포함한 치료: A Clinical report

Treatment including orthognathic surgery of a patient with Hypohidrotic ectodermal dysplasia with cleft palate: A Clinical report

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초록

Ectodermal dysplasia is a genetic disorder in which various clinical manifestations involve two or more of the differentiated tissues of the ectoderm. Facial deformity, which is frequently associated with ectodermal dysplasia, appears in the form of cleft lip or cleft palate, especially in the middle facial area.Cleft and tooth defects result in decreased alveolar bone development.This leads to severe skeletal incongruity. Facial features include frontal protrusion, malar bone hypoplasia, flat nose, mandibular prominence and long lower facial height. This clinical report presents treatment including orthognathic surgery of a patient with Hypohidrotic Ectodermal dysplasia with cleft palate.

Abstract

Ectodermal dysplasia is a genetic disorder in which various clinical manifestations involve two or more of the differentiated tissues of the ectoderm. Facial deformity, which is frequently associated with ectodermal dysplasia, appears in the form of cleft lip or cleft palate, especially in the middle facial area.Cleft and tooth defects result in decreased alveolar bone development.This leads to severe skeletal incongruity. Facial features include frontal protrusion, malar bone hypoplasia, flat nose, mandibular prominence and long lower facial height. This clinical report presents treatment including orthognathic surgery of a patient with Hypohidrotic Ectodermal dysplasia with cleft palate.

참고문헌 (11)

  1. Vo Truong N. Ngoc, "Clinical, radiographic, and genetic characteristics of hypohidrotic ectodermal dysplasia: A cross-sectional study", Clinical Genetics, 94(5), pp.484~486 (2018), Wiley[DOI]
  2. Liu Y, "Mutation Screening of the EDA Gene in Seven Chinese Families with X-Linked Hypohidrotic Ectodermal Dysplasia", Genet Test Mol Biomarkers, 22(8), pp.487~491 (2018)
  3. Meshram GG, "A case report of Hypohidrotic ectodermal dysplasia: A mini-review with latest updates", J Family Med Prim Care, 7(1), pp.264~266 (2018)
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  5. Drew SJ, "Clinical controversies in oral and maxillofacial surgery: Part one. Maxillary distraction osteogenesis for advancement in cleft patients, internal devices", J Oral Maxillofac Surg, 66(12), pp.2592~2597 (2008)
  6. Kuroda S, "Maxillary distraction osteogenesis to treat maxillary hypoplasia:comparison of an internal and an external system", Am J Orthod Dentofacial Orthop, 127(4), pp.493~498 (2005)
  7. Rachmieal A, "Long-term results in maxillary deficiency using intraoral devices", Int J Oral Maxillofac Surg, 34(5), pp.473~479 (2005)
  8. Rachmiel A, "Management of Severely Atrophic Maxilla in Ectrodactyly Ectodermal Dysplasia-cleft Syndrome", Plast Reconstr Surg Glov Open, 6(2), pp.e1678~ (2018)
  9. Mohammad Bayat, "Full mouth implant rehabilitation of a patient with ectodermal dysplasia after orthognathic surgery, sinus and ridge augmentation: a clinical report", The Journal of Advanced of Prosthodontics, 3(2), pp.96~100 (2011), 대한치과보철학회[DOI]
  10. Van Sickels JE, "Interdisciplinary management of patients with ectodermal dysplasia", J Oral Implantol, 36(3), pp.239~245 (2010)
  11. Sclar AG, "Treatment plannig and surgical considerations in implant therapy for patients with agenesis, oligodontia, and ectodermal dysplasia:review and case presentation", J Oral Maxillofac Surg, 67(11 Suppl), pp.2~12 (2009)

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