Advertisement

European Archives of Paediatric Dentistry

, Volume 9, Issue 3, pp 138–141 | Cite as

Rutherfurd syndrome associated with Marfan syndrome

  • T. A. RajaEmail author
  • S. Albadri
  • C. Hood
Case Report

Abstract

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: A 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; an uncommon 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 had 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.

Key words

Rutherfurd delayed eruption gingival fibromatosis treatment 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Ahmad S, Bister D, Cobourne MT. The clinical features and aetiological basis of primary eruption failure. Europ J Ortho 2006;28:535–540.CrossRefGoogle Scholar
  2. Baptista IP. Hereditary gingival fibromatosis: A case report. J Clin Periodontol 2002;29:871–874.PubMedCrossRefGoogle Scholar
  3. Bittencourt LP, Campos V, Moliterno LFM, Ribeiro DPB, Sampaio RK. Hereditary gingival fibromatosis: Review of the literature and a case report. Quintessence Int 2000;31:415–418.PubMedGoogle Scholar
  4. Bozzo L, Machado MA, de Almeida OP, Lopes MA, Coletta RD. Herditary gingival fibromatosis: Report of three cases. J Clin Pediatr Dent 2000;25:41–46.PubMedGoogle Scholar
  5. Coletta RD, Graner E. Hereditary gingival fibromatosis: A systematic review. J Periodont 2006;77:753–764.PubMedCrossRefGoogle Scholar
  6. Cuestas-Carnero R, Bornancini CA. Hereditary generalised gingival fibromatosis associated with hypertrichosis: Report of five cases in one family. J Oral Maxillofac Surg 1988;46:415–420.PubMedCrossRefGoogle Scholar
  7. De Coster PJA, Martens LCM, De Paepe A. Oral manifestations of patients with Marfan syndrome: A case control study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;93:564–572.PubMedCrossRefGoogle Scholar
  8. Doufexi A, Mina M, Ioannidou E. Gingival overgrowth in children: Epidemiology, pathogenesis, and complications. A literature review. J Periodontol 2005;76:3–10.Google Scholar
  9. Haytac MC, Ozcelik O. The phenotypic overlap of syndromes associated with hereditary gingival fibromatosis: follow-up of a family for five years. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;103:521–7.PubMedCrossRefGoogle Scholar
  10. Houston IB, Shotts N. Rutherfurd’s Syndrome: A familial oculo-dental disorder. Acta Paed Scan 1966;55:233–238.CrossRefGoogle Scholar
  11. Kavvadia K, Pepelassi E, Alexandridis C, et al. Gingival fibromatosis and significant tooth eruption delay in an 11-year old male: A 30 month follow-up. Int J Paediatr Dent 2005;15:294–302.PubMedCrossRefGoogle Scholar
  12. Rutherfurd ME. Three generations of inherited dental defect. Brit Med J 1931;2:9–11.PubMedCrossRefGoogle Scholar
  13. Shah N, Gupta YK, Ghose S. Zimmermann-Laband syndrome with bilateral developmental cataract — a new association? Int J Paed Dent 2004;14:78–85.CrossRefGoogle Scholar

Copyright information

© Adis International 2008

Authors and Affiliations

  1. 1.Dept. Paediatric DentistryUniversity Dental Hospital of ManchesterManchesterEngland

Personalised recommendations