Case report: Dental management of Berardinelli-Seip congenital lipodystrophy

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.

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.

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.

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

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.