| Case report: Rutherfurd syndrome associated with Marfan syndrome. | |
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MedLine Citation:
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PMID: 18793596 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Rutherfurd syndrome is a rare genetic disorder that is primarily characterised by the classical triad of gingival fibromatosis, delayed tooth eruption and corneal dystrophy. Associated features of the condition include abnormally shaped teeth, mental retardation and aggressive behaviour. CASE REPORT: His Consultant Clinical Geneticist referred a 2-year-old boy to the Dept. of Paediatric Dentistry at Manchester Dental Hospital. The child and his father had a diagnosis of Rutherfurd Syndrome; a rare autosomal-dominant condition featuring corneal dystrophy, gingival hypertrophy, abnormally shaped teeth and delayed eruption. The only erupted teeth were 52, 71 and 81. The patient also suffers from features of Marfan syndrome, a condition he has inherited from his mother. CLINICAL MANAGEMENT: Preventive advice was provided and the patient was placed on regular review. By the age of 4 years, 52, 61, 72, 71 and 81 were the only teeth present clinically. Maxillary and mandibular dentures were provided, which he refused to wear. FOLLOW-UP: At age 8 years, 31 and 41 were erupted lingual to the primary incisors. No other permanent teeth had erupted. Radiographs revealed failure of eruption of multiple primary and permanent teeth. The second premolars had formed above the unerupted mandibular primary second molars. The patient continued to refuse any intervention. Treatment options discussed included overdentures and /or removal of primary teeth and exposure of permanent teeth. CONCLUSION: This case has presented a rare syndrome with two important dental features, namely gingival hyperplasia and failure of eruption. The case has a long term follow up of 6 years and has a first time association to Marfan syndrome. It also highlights the importance of family history and how it can affect the attitude of the child towards dental treatment. |
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Authors:
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T A Raja; S Albadri; C Hood |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: European archives of paediatric dentistry : official journal of the European Academy of Paediatric Dentistry Volume: 9 ISSN: 1818-6300 ISO Abbreviation: - Publication Date: 2008 Sep |
Date Detail:
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Created Date: 2008-09-16 Completed Date: 2009-01-09 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101277157 Medline TA: Eur Arch Paediatr Dent Country: England |
Other Details:
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Languages: eng Pagination: 138-41 Citation Subset: D; IM |
Affiliation:
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Dept. Paediatric Dentistry, University Dental Hospital of Manchester, England. taiyub_raja@hotmail.com |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple
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diagnosis*,
therapy Anodontia / radiography* Child, Preschool Corneal Dystrophies, Hereditary / diagnosis Denture, Partial Fibromatosis, Gingival / diagnosis* Follow-Up Studies Humans Jaw, Edentulous, Partially / radiography, rehabilitation* Male Marfan Syndrome Syndrome Tooth Abnormalities / radiography, surgery Tooth, Unerupted / radiography, surgery* Treatment Refusal |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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