Suspected Gardner’s syndrome in orthodontic practice – a case report
Journal Title: Forum Ortodontyczne - Year 2017, Vol 13, Issue 4
Abstract
Gardner syndrome (GS, MIM#175100) is a genetic disease inherited in an autosomal dominant manner that belongs to the spectrum of familial adenomatous polyposis, FAP1). Familial adenomatous polyposis is characterised by the presence of as many as up to several thousand polyps especially in the colon, as well as their early malignant transformation that reaches the level of one hundred percent in untreated patients. Other non-gastrointestinal symptoms of GS include benign tumours of the epidermis, dermis and hypodermis, osseous lesions (osteomas and diffuse osteosclerosis), dental abnormalities (supernumerary teeth, compound odontomas, unerupted permanent teeth, overretained deciduous teeth, hypercementosis, or root anomalies) and multiple (> 4) foci of congenital hypertrophy of the retinal pigment epithelium (CHRPE). The risk of neoplastic growth, including that occurring also outside of the colon, is observed in FAP. Patients with FAP are exposed to a higher degree of occurrence of thyroid and pancreatic carcinomas, gallbladder cancer and cholangiocarcinoma, duodenal carcinomas, adrenal adenoma, or hepatoblastoma.<b> Aim.</b> Presentation of the case of a patient suspected of suffering from GS based on characteristic dental and osseous lesions, and collection of available information on GS in terms of orthodontic diagnostic and therapeutic management conducted on the grounds of related literature.<b> Case report. </b> The description embraced a clinical and radiological picture of an orthodontically treated 16-year-old female patient. The data bases of PubMed, Embase and Google Scholar were reviewed searching the following key words, “Gardner syndrome”, “familial adenomatous polyposis”, ”osteoma”, “dental abnormalities”. From among 87 publications from the years 1987–2017, 32 were used. <b>Results</b>. As a nosological entity, Gardner syndrome is characterised by genetic and phenotypic variability. Patients with GS may require multidirectional dental treatment, inclusive of orthodontic therapy. <b>Conclusions</b>. For a patient suspected to be affected by GS, it is crucial to be diagnosed early in order to save his or her health and life. A dentist can be the first person to suspect that the patient suffers from GS due to characteristic lesions in the stomatognathic system that may be concomitant with GS. <b>(Kuśmierczyk D, Jurek A, Szałwińska M. Suspected Gardner’s syndrome in orthodontic practice – a case report. Orthod Forum 2017; 13: 371-82). </b>
Authors and Affiliations
Dorota Kuśmierczyk, Agnieszka Jurek, Magdalena Szałwińska
Keywords
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