FACT SHEET: Von Willebrand Disease (also known as “von Willebrand’s disease”, “vWD”, and “VWD”)
Is the initiation of non-invasive dental hygiene procedures* contra-indicated?
Is medical consult advised?
- Yes, liaison with the patient/client’s hematologist (blood specialist) is advisable regarding the type, severity, and management of the patient/client’s vWD before undertaking dental hygiene treatment for the first time. Medical consult is also warranted for suspicious, but as yet undiagnosed, bleeding disorder, and if known disease is poorly controlled.
Is the initiation of invasive dental hygiene procedures contra-indicated?**
- Yes. This is a blood disorder that may affect appropriateness or safety, and scaling and root planing, including curetting surrounding tissue, are contraindicated until the patient/client is medically cleared.
Is medical consult advised?
- See above. Additionally:
- The patient/client’s hematologist should be consulted before any invasive dental hygiene procedures are performed. The diagnosis needs to be confirmed; the type of vWD variant established; and the need for desmopressin [DDVAP1] (i.e., non-replacement therapy to elevate von Willebrand factor — vWF — levels), vWF with coagulation factor VIII (i.e., replacement therapy), and aminocaproic acid or tranexamic acid (i.e., antifibrinolytic therapy to preserve clot) determined.
Is medical clearance required?
- Patients/clients with type 1 vWD (70% to 80% of cases, which tend to be mild) can often safely receive dental hygiene care (including supra- and subgingival scaling, periodontal probing, root planing, curetting, and polishing) in a general dental/dental hygiene practice with desmopressin (which transiently increases vWF, as well as factor VIII, levels) cover. Consultation will identify the relatively few patients/clients who require vWF with factor VIII.
- Some patients/clients with type 2 vWD (20% to 30% of cases) can also usually be managed with desmopressin. However, in others, vWF with factor VIII is also required, which likely necessitates being seen in a specialist/hospital/hemophilia (i.e., bleeding disorder) treatment centre setting.
- Type 3 vWD (severe disease) is rare and requires vWF with factor VIII in all cases. Patients/clients with type 3 vWD and some with type 2 vWD may require hospitalization for surgical/invasive procedures.
Is antibiotic prophylaxis required?
Is postponing treatment advised?
- Yes, until the patient/client has been medically cleared and possibly pre-treated to avoid excessive bleeding after dental hygiene procedures. See above.
Oral management implications
- Patients/clients should be asked about any previous unusual bleeding episode after surgery or trauma, spontaneous bleeding, easy or frequent bruising, and bleeding problems in relatives. A history of prolonged bleeding after tooth extraction is more suggestive of von Willebrand disease (or other platelet disorders) than hemophilia A or hemophilia B.
- For the purpose of history-taking, a clinically significant bleeding episode is one that:
- continues beyond 12 hours;
- causes the patient/client to call or return to dental practitioner or seek medical treatment/emergency care;
- results in development of hematoma or ecchymosis with soft tissues; or
- requires blood product support.
- History taking should include enquiries about herbal supplements (e.g., garlic, ginseng, ginger, gingko, and kava) that may potentiate bleeding tendency.
- The dental hygienist should impress upon patients/clients with vWD the importance of good oral hygiene. Appropriate self-care, coupled with regular dental hygiene and dental visits, will reduce the chances of future problems such as needing extractions or oral infections, which can lead to bleeding complications.
- Some persons with vWD fear that brushing and flossing will result in bleeding. The dental hygienist should inform patients/clients that these activities are important for everyone, and neglect of these basics has a more profound impact on persons with hemophilia than others. Healthy gums do not generally bleed during brushing and flossing — bleeding gums are a sign of dental disease. If bleeding continues for more than 20 minutes — or stops and then starts again — the patient/client should be advised to contact his/her hemophilia/bleeding disorder treatment centre for potential treatment to stop the bleeding.
- A soft brush or extra-soft should be used for cleaning teeth in order to minimize bleeding tendency.
- Medicated mouthwashes (e.g., chlorhexidine and fluoride) may be recommended to decrease oral disease.
- Mouth guards should always be worn when playing sports — particularly contact sports. Oral piercing should be avoided.
- Mouth bleed emergencies include:
- bleeding on the tongue, cheek, or floor of the mouth that does not stop;
- swelling or bruising of the tongue, throat, or neck; and
- trouble breathing or swallowing.
The patient/client should be advised to go to the emergency department (and contact his/her treatment centre) if these occur.
- Children with vWD may experience bleeding or oozing when teeth erupt. The dental hygienist should advise parents to apply sustained, gentle pressure to the area. A prescribed antifibrinolytic agent may additionally be used. Soft, cool foods such as yogurt and the avoidance of hot foods and straws can help preserve the blood clot until healing occurs.
- Children with vWD can wear braces (following special care to avoid cutting the gums when bands and wires are placed on the teeth). Dental hygienists can advise patients/clients that application of dental wax over rough edges protects gum tissue, cheeks, and lips.
- During the dental hygiene/dental visit, care should be taken with the soft tissues, particularly the use of impression trays, aspirators, and x-ray films in the floor of the mouth. Trauma to mandibular lingual tissues increases the risk of hemorrhage, which can lead to airway obstruction.
- For severely inflamed tissues, prior treatment with chlorhexidine mouthwashes and gross debridement to reduce inflammation is recommended before deep scaling. Ultrasonic instrumentation may result in less tissue trauma.
- Tranexamic acid mouth rinses significantly reduce bleeding following invasive dental hygiene procedures; the mouthwash can be applied to gauze for direct application. It may be used in combination with oral tranexamic acid tablets. Patients/clients are often advised to medicate pre-procedure and for 5 to 10 days post-procedure.
- Dental hygienists should be alert for intraoral, tongue, and throat swelling/bleeding that could block the patient/client’s airway. Difficulty breathing or swallowing should prompt initiation of emergency protocol, and prompt transfer to an emergency department is indicated.
- Patients/clients placed on replacement therapy (i.e., von Willebrand factor plus coagulation factor VIII) should be observed for signs and symptoms of allergy (i.e., shortness of breath, urticaria [hives], etc.).
- Aspirin and nonsteroidal inflammatory drugs (e.g., ibuprofen, indomethacin, and naproxen) should be avoided in persons with vWD, because they can adversely affect platelet function. Acetaminophen and codeine-based pain relievers are more appropriate, because they do not exacerbate bleeding tendency.
- Persons who undergo invasive procedures should generally be seen 24 to 48 hours afterwards by the dental hygienist or dentist to check on control of bleeding.
- Canada has 26 specialized hemophilia/bleeding disorder treatment centres (9 of which are located in Ontario), which are listed at https://www.hemophilia.ca/find-a-treatment-centre-near-you/. These centres may provide the patient/client with an Information Sheet for the Dentist that describes the type and severity of the bleeding disorder, as well as whom to contact for advice.
- The incidence of dental caries and periodontal disease is higher in patients/clients with bleeding disorders, which may be related to lack of effective oral hygiene and professional oral care due to fear of bleeding. Gingival bleeding from gingivitis may be exacerbated by vWD.
- Prolonged bleeding following invasive dental hygiene procedures or tooth extraction may be the first indication of von Willebrand disease variants with factor VIII deficiency.
- Petechiae (pinpoint, round red spots which are caused by capillary bleeding in the skin), ecchymoses (bruises), and hemorrhagic blisters may occur in the oral cavity, depending on the severity of vWD. In severe cases, spontaneous oral mucosal bleeding — particularly gingival — occurs.
Related signs and symptoms
- Von Willebrand disease (vWD) is the most common inherited bleeding disorder. A lifelong condition, it is caused by a deficiency or defect in the clotting protein known as von Willebrand factor, which binds factor VIII (a key coagulation protein) to platelets in blood vessel walls, thereby forming a platelet plug during the clotting process.
- This disorder of platelet adhesion is usually transmitted in an autosomal dominant fashion (types 1 and 2), thereby equally affecting males and females (unlike male preponderance in hemophilia). There are also several autosomal recessive variants (type 3) with male predilection.
- VWD is a form of nonthrombocytopenic purpura2. It affects at least 0.1% of the Canadian population; i.e., about 37,000 Canadians although many others remain undiagnosed. (With up to 9 out of 10 persons with vWD being undiagnosed, prevalence estimates are up to 1% of the world’s population.)
- Type 1 (70% to 80% of vWD) and type 2A (15% to 20%) variants tend to result in mild to moderate bleeding problems. The greater the quantitative deficiency of von Willebrand factor in type 1 disease or the qualitative deficiency in type 2 disease (i.e., vWF unable to bind coagulation factor VIII), the more likely that coagulation signs and symptoms of hemophilia A will also be found, in addition to those associated with defective platelet adhesion.
- Type 3 vWD, which is rare (5% to 10% of cases), manifests as a severe bleeding disorder due to absence of vWF and concomitant reduction in circulating factor VIII.
- Acquired vWD, which is less common than congenital vWD, can occur in adults secondary to autoimmune disease (such as lupus), certain types of heart disease or cancer, or complications of certain medications.
- Patients/clients with severe forms of vWD may describe a family history of bleeding problems, whereas those with mild disease may have a negative family history.
- Laboratory testing may show prolonged activated partial thromboplastin time (aPTT), normal or slightly reduced platelet count, and normal prothrombin time (PT). Additional specialized tests comprising the “vWD disease panel” are required to establish the diagnosis and type of vWD.
- Easy bruising is a sign commonly seen in patients/clients with vWD.
- In mild vWD (the majority of cases), cutaneous and mucosal bleeding usually occurs only after surgery or trauma. In more severe cases, spontaneous epistaxis (nosebleed) or oral mucosal bleeding may occur. In very severe forms of the disease, hemarthroses (bleeding into joint spaces), and dissecting muscular hematomas (bleeding into muscles) are part of the picture.
- Menorrhagia (heavy menstrual periods) is common; affected women are often prescribed oral contraceptive agents to suppress menses and thus avoid excessive physiologic loss of blood. Other vWD manifestations include excessive postpartum bleeding and hematuria (blood in urine).
- Life-threatening bleeding (e.g., cerebral and gastrointestinal) can occur in persons who have type 3 vWD, in some persons who have type 2 vWD, and rarely in persons who have type 1 vWD.
References and sources of more detailed information
- College of Dental Hygienists of Ontario
- Gupta A, Epstein JB, Cabay RJ. Bleeding Disorders of Importance in Dental Care and Related Patient Management. JCDA. 2007 Feb;73(1):77-83a.
- Yawn BP, Nichols WL and Rick ME. Diagnosis and Management of Von Willebrand Disease: Guidelines for Primary Care. Am Fam Physician. 2009 Dec 1;80(11):1261–1268.
- Canadian Hemophilia Society http://www.hemophilia.ca/en/bleeding-disorders/von-willebrand-disease/
- National Hemophilia Foundation (U.S.)
- Medline Plus — U.S. National Library of Medicine https://www.nlm.nih.gov/medlineplus/ency/article/000544.htm
- National Heart, Lung and Blood Institute — National Institutes of Health
- Haemophilia and Dental Care — Information for Dentists (U.K)
- World Federation of Hemophilia www.wfh.org
- Bolivar E, Karp S and Peterson S with revised edition authors McIntosh K and Purcell S. Dental Care for People with Bleeding Disorders. Booklet of Canadian Association of Nurses in Hemophilia Care; 2012.
- Scully C, Dios PD, Giangrande P. Oral Care For People With Hemophilia Or A Hereditary Bleeding Tendency. Monograph of World Federation of Hemophilia (second edition); 2008.
- Brewer A and Correa ME. Guidelines For Dental Treatment Of Patients With Inherited Bleeding Disorders. Monograph of World Federation of Hemophilia; 2006.
- Hemophilia of Georgia. Protocols For The Treatment Of Hemophilia And Von Willebrand Disease; 2018.
- Little JW, Falace DA, Miller CS and Rhodus NL. Dental Management of the Medically Compromised Patient (8th edition). St. Louis: Elsevier Mosby; 2013.
- Ibsen OAC and Phelan JA. Oral Pathology For The Dental Hygienist (6th edition). St. Louis: Elsevier Saunders; 2014.
1 DDAVP = 1-desamino-8-D-arginine vasopressin
2 Purpura is reddish-blue or purplish discolouration of the skin or mucosa resulting from spontaneous extravasation of blood. Von Willebrand disease is the nonthrombocytopenic type, because there is usually no or only minor thrombocytopenia (i.e., no or only minor reduction in platelet numbers, unlike the case in thrombocytopenic purpura).
* Includes oral hygiene instruction, fitting a mouth guard, taking an impression, etc.
** Ontario Regulation 501/07 made under the Dental Hygiene Act, 1991. Invasive dental hygiene procedures are scaling teeth and root planing, including curetting surrounding tissue.