Dental disease in type 3 Von Willebrand disease: a neglected problem

Type 3 Von Willebrand disease (VWD) is a rare, severe, autosomal recessive bleeding disorder. In our institution, we follow 17 children with type 3 VWD. We have observed a high prevalence of dental disease in these patients prompting us to undertake a retrospective review of our cohort of patients with type 3 VWD to catalogue the extent of their dental disease. Sixteen of these patients have been assessed by our dentistry department. Five children have undergone minor dental procedures (e.g. restorations, stainless steel crowns) and seven major procedures (e.g. dental extractions, pulpotomies and root canal treatments). These patients have collectively used 85,400 (ristocetin cofactor) IU of Humate-P on dental procedures alone. In addition to the considerable costs of factor are the cost of operating room time, dentists' costs, and the cost of other topical haemostatic agents (e.g. Tisseel) used during their dental procedures. As such there is considerable morbidity and cost from dental disease in these patients that is much higher than what is seen in patients with haemophilia or in the normal paediatric population. We speculate that the combination of these patients having a significant mucosal bleeding disorder together with various socioeconomic factors contribute to the significant degree of dental disease seen in this group of patients. We would suggest that better preventive dental care needs to be provided to these patients to avoid the considerable morbidity and very high burden of dental disease in type 3 VWD.