Advertisement

European Archives of Paediatric Dentistry

, Volume 17, Issue 2, pp 137–140 | Cite as

Case report: Dental management of Berardinelli-Seip congenital lipodystrophy

  • N. BhujelEmail author
  • H. Clark
Case Report
  • 163 Downloads

Abstract

Background

Berardinelli-Seip congenital lipodystrophy (BSCL) is a very rare autosomal recessive condition caused by mutations of the AGPAT2 gene or the BSCL2 gene. BSCL is associated with a number of dental manifestations. There are no published case reports of dental treatment under general anaesthesia (GA) in patients with this condition.

Case report

A 6-year-old girl with BSCL characterised by classical features of the condition including a growth disorder, precocious puberty, endocrine disturbances, hypertrophic cardiomyopathy and fatty infiltration of the liver was referred for treatment under GA. Dental manifestations included aberrant tooth morphology, macrodontia and generalised severe crowding.

Treatment

Dental treatment was carried out under GA following consideration of various options. In conjunction with other medical specialists, a number of peri-operative precautions were taken. Comprehensive dental care was provided which included restorations, extractions and fissure sealants.

Follow-up

The patient was reviewed post-operatively and at regular intervals between 3 and 6 months for review and prevention.

Conclusion

The patient had a number of dental manifestations associated with BSCL. Peri-operative planning is essential for some patients who may have a number of medical conditions.

Keywords

Berardinelli-Seip congenital lipodystrophy Dental management 

References

  1. Bennett T, Allford M. Delayed emergence from anesthesia in a child with congenital generalized lipodystrophy (Berardinelli-Seip syndrome). Paediatr Anaesth. 2012;22:299–300.CrossRefPubMedGoogle Scholar
  2. Garg A. Acquired and inherited lipodystrophies. N Engl J Med. 2004;350:1220–34.CrossRefPubMedGoogle Scholar
  3. Gomes KB, Pardini VC, Fernandes AP. Clinical and molecular aspects of Berardinelli-Seip congenital lipodystrophy (BSCL). Clin Chim Acta. 2009;402:1–6.CrossRefPubMedGoogle Scholar
  4. Lima DL, Montenegro Junior RM, Fernandes VO, Barros AI, Rego DM. Dental and periodontal alterations in Berardinelli-Seip syndrome. J Int Acad Periodontol. 2007;2007(9):63–7.Google Scholar
  5. Poliac LC, Barron ME, Maron BJ. Hypertrophic cardiomyopathy. Anesthesiology. 2006;104:183–92.CrossRefPubMedGoogle Scholar
  6. Solanki M, Patil SS, Baweja DK, Noorani H, Shivaprakash PK. Talon cusps, macrodontia, and aberrant tooth morphology in Berardinelli-Seip syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008;105:e41–7.CrossRefPubMedGoogle Scholar
  7. Van Maldergem L. Berardinelli-Seip congenital lipodystrophy. In: Pagon RA, Adam MP, Bird TD, Dolan CR, Fong CT, Smith RJH, Stephens K, editors. GeneReviews. Seattle: University of Washington; 1993.Google Scholar

Copyright information

© European Academy of Paediatric Dentistry 2015

Authors and Affiliations

  1. 1.Guy’s and St Thomas’ NHS TrustLondonUK
  2. 2.Salaried Dental ServiceBradford District Care TrustBradfordUK

Personalised recommendations