Abstract
Background
von Willebrand disease (VWD) is a bleeding disorder caused by an abnormality of the von Willebrand factor protein.
Case presentation
Two brothers diagnosed with VWD came to our clinic, the older for caries treatment and the younger for extraction of primary teeth. To control intraoperative bleeding and postoperative hemostasis, the older brother received plasma-derived/factor VIII concentrate intravenously the day before dental treatment, while the younger brother received desmopressin preoperatively, as that was found to be effective.
Conclusion
For invasive dental treatment of patients with VWD, it is important to choose a plan according to patient symptoms.
1
Introduction
von Willebrand disease (VWD) is an inherited bleeding disorder caused by quantitative or qualitative abnormalities of the von Willebrand factor (VWF) protein [ ]. This protein has two primary roles; binding of platelets to exposed collagen at a vascular injury site, and binding and stabilization of factor VIII [ ]. For patients with or a family history of significant bleeding symptoms, hemostatic screening tests including complete blood count (CBC), prothrombin time (PT), activated partial thromboplastin time (ATPP), and fibrinogen measurements are performed [ ]. When APTT is prolonged despite normal CBC, PT, and fibrinogen findings, VWD is suspected, then bleeding symptoms, and low VWF activity or a VWF antigen level below 30% leads to diagnosis of VWF [ ]. However, when significant bleeding symptoms are observed even with normal VWF activity or a VWF antigen level greater than 30%, VWD cannot be excluded. For cases in which it is difficult to diagnose or classify disease type by quantifying VWF activity or VWF antigen levels, genetic testing may provide definitive information [ ]. VWD is classified into seven clinical types; type 1, 2A, 2B, 2 M, 2 N, and pseudo-type [ ]. Manifestations of the disease are primarily repeated mucosal and skin bleeding, including epistaxis, purpura, hematoma, oral bleeding, abnormal menstrual bleeding, gastrointestinal bleeding, and hematuria, as well as difficulty with hemostasis following tooth extraction, surgery, or trauma [ ]. Children with VWD may have difficulty obtaining adequate assessment based on severity of bleeding due to lack of history of trauma and/or invasive treatments [ ].
For treatment or prevention of bleeding related to dental procedures, tranexamic acid is often used alone or in combination with desmopressin (1-deamino-8- d -arginine vasopressin; DDAVP), or plasma-derived VWF VIII (pdVWF/FVIII) concentrate [ , ]. However, response to DDAVP varies among individuals, thus its effects should be tested early after diagnosis during a non-bleeding time, except for children under two years old, elderly patients with atherosclerotic disease, and patients with ineffective disease types, for whom DDAVP adverse reactions are a concern [ ]. When DDAVP is ineffective or contraindicated, use of pdVWF/FVIII concentrate is recommended for hemostatic management [ , ]. Therefore, it is necessary to understand the condition of individual VWD patients and consider an appropriate management plan prior to performing invasive dental treatment.
Herein, results of two brothers with VWD who underwent different dental invasive treatments are presented. Informed consent was obtained from their parents for publication of these findings and the accompanying images.
2
Case report
Two Japanese brothers came to the Department of Pediatric Dentistry, Osaka University Dental Hospital. The older brother had iron deficiency anemia and the younger brother arthrogryposis multiplex congenita. Family history findings showed that their father and the younger brother had previously been diagnosed with VWD. Thus, the older brother was suspected to also have VWD and underwent medical examinations, which led to that diagnosis. Blood test results for the brothers showed low levels of VWF (<10% in both, normal >50%) and FVIII (47.3% in older and 36.1% in younger brother, normal >50%) activities. Both were prone to epistaxis, though had no previous history of injury with massive bleeding. In addition, the dental history for each included no experience with local anesthesia or tooth extraction, and there was no persistent bleeding at the time of spontaneous exfoliation of their primary teeth. Only the younger brother had undergone genetic testing and was found to be VWD type 2A.
The older brother came to our clinic in July 2022 at the age of 11 years 0 months (11Y0M) for caries treatment. Intraoral examination findings at the first visit showed dental caries on the occlusal surface of the maxillary left first molar, with the caries cavity covered by a gingival valve ( Fig. 1 A). A dental radiographic examination revealed that the caries had extended close to the pulp ( Fig. 1 B). The treatment plan was a gingivectomy under local anesthesia, then atraumatic indirect pulp capping. The primary physician was consulted, who advised administration of DDAVP or pdVWF/FVIII concentrate to control intraoperative bleeding and postoperative hemostasis. Since the caries was near the pulp, treatment was considered urgent. An examination to determine the effects of DDAVP is required before use, though that had not been conducted for the patient. Therefore, a pdVWF/FVIII concentrate was selected after obtaining consent from his parents and intravenously administered at a medical hospital one day before the dental treatment, which was then performed in August 2022. Postoperatively, tranexamic acid oral internal medicine was prescribed and a dental splint was placed. Intraoperative bleeding and postoperative hemostatic control were both satisfactory. Additionally, the wound in the gingivectomy area showed adequate healing ( Fig. 2 ). Preparations were made for treatment at the medical institution of the primary physician should there be difficulty with stopping the bleeding, though that was unnecessary.
