• Users Online: 141
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 

 Table of Contents  
Year : 2020  |  Volume : 32  |  Issue : 3  |  Page : 160-165

Endodontic management of a Von Willebrand's disease patient: A case report with short review

Department of Restorative Dental Science, College of Dentistry, University of Hail, Hail, Kingdom of Saudi Arabia

Date of Submission08-Mar-2020
Date of Decision07-Apr-2020
Date of Acceptance13-Jun-2020
Date of Web Publication28-Oct-2020

Correspondence Address:
Dr. Saad M Al-Zubaidi
Department of Restorative Dental Science, College of Dentistry, University of Hail, Hail
Kingdom of Saudi Arabia
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/endo.endo_33_20

Rights and Permissions

Von Willebrand's disease (VWD) is a lifelong bleeding disorder in which the blood does not clot well. Mutations in the VWD gene cause VWD. The von Willebrand factor as a blood-clotting protein is provided by the VWF gene and is essential for the formation of blood clots. People with inherited bleeding disorders present a particular risk with regard to dental treatment. It is that these patients are treated safely and appropriately to avoid unnecessary risk of bleeding but also to minimize the use of clotting factor concentrate in situations where alternative methods of treatment can be effective. The aim of this case report is to provide information on how to handle a patient with a probably diagnosis of VWD with symptomatic irreversible pulpitis and symptomatic apical periodontitis of mandibular left second molar #37. The endodontic therapy was performed together with nonsurgical initial cause-related therapy. Even though the patient was on medication, the bleeding was obviously during the treatment. During follow-up period, the endodontic therapy was successfully. The strategy used in this case was effective in the management of coagulopathy and allowed emergency care to be carried out without complications.

Keywords: Endodontic therapy, symptomatic apical periodontitis, symptomatic irreversible pulpitis, von Willebrand's disease

How to cite this article:
Al-Zubaidi SM. Endodontic management of a Von Willebrand's disease patient: A case report with short review. Endodontology 2020;32:160-5

How to cite this URL:
Al-Zubaidi SM. Endodontic management of a Von Willebrand's disease patient: A case report with short review. Endodontology [serial online] 2020 [cited 2022 Jan 28];32:160-5. Available from: https://www.endodontologyonweb.org/text.asp?2020/32/3/160/299286

  Introduction Top

Hematological diseases are a very significant aspect, due to they are fairly often met in dental clinics. Therefore, patients with hematological diseases need more care from dentists than other patients because they are continually exposed to the risk of bleeding during dental treatment. The dental practitioner may be the first one who suspects or recognizes one of these diseases.[1] Therefore, dentists should be aware of the impact of bleeding disorders on the management of their patients. Proper dental and medical evaluation of patients is, therefore, necessary before treatment, especially if an invasive dental procedure is planned. Patient assessment and history must start with standard medical questionnaires. Patients should be asked about any preceding unusual bleeding episode after surgery or injury, spontaneous bleeding, and easy or frequent bruising.[2]

Von Willebrand's disease (VWD) is a hereditary disease that is transferred by an autosomal dominant gene but also sometimes by an autosomal receive gene. This disease is more likely associated with females. The VWD is an inherited bleeding disorder that results from quantitative and/or qualitative abnormalities of the von Willebrand factor (VWF).[3] The patients have lack of von Willebrand's coagulation factor that is present in platelets, megakaryocytes, plasma, and endothelial cells.[4] VWF is a large glycoprotein with a broad range of physiological and pathological functions in health and disease.[5] The VWF as a blood-clotting protein was provided by the VWF gene and is essential for the formation of blood clots. In fact, 95%–97% of all coagulation deficiencies belong to the three groups of diseases: VWD, hemophilia A, and hemophilia B.[6]

The VWF has two main functions: binding to subendothelial collagen and platelets, with consequent promotion of platelet plug formation at endothelial injury sites, and binding to and transport of coagulation factor VIII, thereby protecting it from proteolytic degradation in the plasma.[7] Individuals with VWD display a high frequency of oral bleeding, particularly after surgical procedures and mucosal trauma.[7],[8]

Clinical disease is described by a tendency of bleeding in the initial period of childhood, which expresses itself with bleeding from the mucous membrane of the oral cavity (gingiva), skin (bruising with minimal trauma), nose, and mucous membrane of the digestive organs. Prolonged hemorrhage happens in children after traumatic injuries, and it needs to be occupied into concern that these children must be prepared by the hematologist before any oral surgical intervention was undergone.[2]

There are three major forms of inherited VWD as follows:

  • Type 1 is the most common form, affecting about three-quarters of people who have inherited VWD. People with this type do not make enough VWF or have abnormally fast removal of VWF from the bloodstream. About 60%–80% of people with VWD have this type, and they do not have enough VWF in their blood. Patients with Type 1 VWD have 20%–50% of normal levels of the VWF. Symptoms of Type 1 VWD are mild
  • Type 2 is the second most common form. People with this type make abnormal VWF, with abnormal function of the protein. There are subtypes of Type 2 that have different characteristics. It is caused by patients' own VWD factor which does not work well. From all patients with VWD, 15%–30% of them have a chance to have Type 2 VWD. Symptoms of VWD Type 2 range from mild to moderate
  • Type 3 is the rarest form of VWD. People with Type 3 do not make any VWF at all or have extremely fast removal of VWF from the bloodstream. This type is usually detected early in life because it leads to the most severe bleeding symptoms. Type 3 has the most severe symptoms. An acquired form of VWD is possible to appear if patients have an autoimmune disease, like lupus. An autoimmune disease occurs when patients' natural defense system (immune system) fights itself. Patients can also get acquired VWD after taking certain medications or from heart disease or some types of cancer.[9],[10]

The patients with Type 1 and 2 VWD have symptoms that range from mild to moderate. They include frequent large bruises from minor injuries, frequent or hard-to-stop nose bleeds, blood in your stool or pee (from internal bleeding), heavy bleeding after a cut, accident, or minor medical procedure, bleeding for a long period of time after major surgery, and heavy or long menstrual periods. Patients with Type 3 VWD may have all the symptoms of Type 1 and Type 2, plus episodes of severe bleeding for no reason. They also might experience severe pain and swelling in their soft tissues and joints because of bleeding. Most people with VWD cannot be cured, but with good management treatment and self-care and control, most people with this disease can have active lives. VWD as a hematologic disorder is the most frequently inherited bleeding disorder, described as a deficiency of VWF, and affects 0.8%–2% of the general population in Europe and America.[11],[12],[13] The purpose of this article was to report a case of VWD and to overview all oral health aspects of patients with this disease.

Individuals with VWD are at high risk of bleeding during dental procedures, and dentists are thus responsible for their safety, for which purpose they should work jointly with hematologists during the clinical planning of treatments.[8],[14] To provide the best care to patients with VWD, some precautions are needed relative to the prescription of medications and the use of anesthetics as well as during procedures.[4] Dentists should ensure that their offices are equipped with adequate means to control bleeding so that they are available for the treatment of patients with VWD.[15],[16]

Many studies are available in the literature regarding the endodontic therapy of patients with bleeding disorders, as shown in [Table 1].[17],[18],[19],[20],[21],[22],[23],[24] Although dental treatment is an important factor by which we can avoid surgical therapies with its associated risk of bleeding in patients with a hereditary bleeding tendency,[25],[26],[27] this is the first study done in Saudi Arabia. In this report, a case is presented in which uncontrollable gingival bleeding during emergency endodontic therapy of tooth #37 was reported. Gingival and pulpal bleeding suggested the possibility of a generalized bleeding disorder that was later confirmed by an extensive hematologic examination. In this case, we reported the endodontic and periodontal outcomes of an adult patient following the final laboratory diagnosis of VWD.
Table 1: Previous studies regarding endodontic treatment for von Willebrand's disease patient

Click here to view

  Case Report Top

A 26-year-old female patient presented to the endodontic emergency at the private clinic, Jeddah, Saudi Arabia, complaining of a spontaneous, sharp intense pain relieved with cold drinks. General medical and dental histories were reviewed, and she has VWD confirmed with hematologic analysis. Under hematologist supervision, she was kept under medication (Dicynone 500 mg). She had undergone private clinic consultations that refused endodontic treatment because of their bleeding disorder. She was given fresh frozen plasma six units before the dental procedure.

The extraoral examination was within normal limits with no relevant abnormalities, while the intraoral examination revealed generalized gingival overgrowth [Figure 1]a and spontaneous gingival bleeding upon probing (gingival index score 3) with heavy plaque accumulation (plaque index 3), as shown in [Figure 1]b. Mandibular left second molar #37 was badly decayed and diagnosed as symptomatic irreversible pulpitis with symptomatic apical periodontitis. Nonsurgical endodontic therapy was then planned together with nonsurgical initial cause-related therapy.
Figure 1: (a) Intraoral clinical picture of the patient shows generalized gingival hyperplasia and (b) continues gingival bleeding

Click here to view

The preoperative periapical radiograph of the affected tooth revealed deep occlusal carious and apical widening of periodontal ligament space [Figure 2]a. The patient was anesthetized with 1.8 mL 2% lidocaine with 1:100,000 epinephrine for inferior alveolar nerve block and 1.8 mL 4% articaine with 1:100,000 epinephrine as buccal infiltration. The rubber dam was used to isolate the tooth with care to avoid injury to hyperplastic gingival tissues by clamp placement. Occlusal carious was removed and endodontic access was gained using Endo Access Bur and Endo Z Bur (Dentsply Tulsa, Tulsa, OK, Switzerland) and irrigated with 5.25% sodium hypochlorite (NaOCl) solution. Intrapulpal supplemental injection was administered to alleviate the anesthetic resistance problem of acutely inflamed pulp tissue and to control massive bleeding that was reported during the extirpation of the pulp tissue. Following partial bleeding control, the pulp chamber tissue was removed, and canal orifices were located and negotiated. Total pulp tissue removal was associated with a complete bleeding control. To determine and measure the working length accurately, the electronic apex locator using a Root ZX (J. Morita Corp., Kyoto, Japan) was used. Chemomechanical preparation was performed using Reciproc instrument (VDW, Munich, Germany). After completing the cleaning and shaping, root canals were dried with sterile paper points (VDW, Munich, Germany). After that, the root canal was filled with calcium hydroxide (Shofu Dental Corporation, Kyoto, Japan) as an intracanal medicament to eliminate the remaining microorganisms. Finally, canal orifices was covered with sterile cotton pellet and the access cavity was restored with temporary filling [Figure 2]b. Paracetamol 1000 mg twice a day for two days was prescribed. At the second visit, after removal the temporary filling and the cotton pellets, the calcium hydroxide were removed thus the by 10 mL of 17% ethylenediaminetetraacetic acid for a minute with mechanical agitation, followed by 10 mL of 5.25% NaOCl. Then, root canals were dried with sterile paper points and then obturated using matching gutta-percha cones (VDW, Munich, Germany) and AH-plus sealer (Dentsply Maillefer, Ballaigues, Switzerland). Finally, the endodontic access opening was restored with glass-ionomer cement and composite resin materials. The postoperative periapical radiograph was taken [Figure 2]c.
Figure 2: Periapical radiographs show mandibular left second molar #37 (a), calcium hydroxide was applied as intracanal medicament (b), postobturation (c) and after 6–24 months of follow-up (d-f)

Click here to view

Initial cause-related therapy consisted of thorough full-mouth scaling and root planing performed in quadrants under local anesthesia following the first emergency endodontic visit. This procedure was performed using a combination of hand and ultrasonic instrumentation using a P10 tip. The patient was recalled in 24 hours in order to complete initial therapy and to receive detailed mechanical plaque control instructions.

Two years following therapy, she reported no signs or symptoms of clinical failure and periapical tissues showed signs of healing radiographically [Figure 2]d, [Figure 2]e, [Figure 2]f. Gingival inflammation was still above 1 score throughout the study period. The pain score approached zero throughout the follow-up period.

  Discussion Top

VWD is a chronic life-lasting bleeding disorder inherited as an autosomal dominant trait and characterized by prolonged bleeding and a decreased level of factor VIII.[28] Patients afflicted with this disease showed ability to form factor VIII following plasma or cryoprecipitate infusion. In addition, factor VIII abnormality is more complex than in hemophilia.[29] Although factor VIII procoagulant activity (antihemolytic factor, factor VIII) is decreased in both VWD and hemophilia, hemophilic plasma contains an antigen precipitated by a rabbit antibody to factor VIII, whereas patients with severe VWD lack this factor.[28] Evidence has been found that factor VIII may be essential for normal platelet function and for the primary arrest of bleeding.[30],[31]

The management of patients with bleeding disorders such as VWD depends on the severity of the condition and the invasiveness of the planned dental procedure. If the procedure has limited invasiveness and the patient has a mild bleeding disorder, only slight or no modification will be required. In patients with severe bleeding disorders, the goal is to minimize the challenge to the patient by restoring the hemostatic system to acceptable levels and maintaining hemostasis by local and adjunctive methods. The patient's physician should be consulted before invasive treatment is undertaken.[2],[3],[7],[8]

General restorative procedures do not pose a substantial risk of bleeding. Care should be taken to avoid injuring the gingiva while placing rubber dam clamps, matrices, and wedges. A rubber dam should be used to prevent laceration of soft tissues by the cutting instruments. Saliva ejectors and high-speed suction can injure the mucosa in the floor of the mouth and cause hematoma or ecchymosis; thus, they should be used carefully. Endodontic therapy is preferred overextraction whenever possible in these patients. Endodontic therapy does not usually pose any significant risk of bleeding and can be performed routinely. Endodontic surgical procedures may require factor replacement therapy.[2]

Endodontic management of patients with hereditary bleeding disorders involves a close cooperation between hematologist and endodontist. In fact, hematologists must provide the latter with the appropriate prophylactic regimen to prevent secondary local bleeding during endodontic therapy. In addition, endodontists must carry out all techniques to reduce the probability of endo-related bleeding. The lack of large published retrospective or prospective studies supporting the recommended regimens for dental treatment of hemophiliacs spurred us to present such cases of VWDs with pulp involvement together with gingival bleeding in order to through light on the feasibility of treating such cases without bleeding complications.

The case showed that patient systemic bleeding control measures do not seem to control pulp or gingival bleeding. The case also showed the ability to control profuse bleeding partially by intrapulpal injection and totally by the removal of pulp tissues during canals negotiation. The patient was anesthetized with 1.8 mL 2% lidocaine with 1:100,000 epinephrine for inferior alveolar nerve block and 1.8 mL 4% articaine with 1:100,000 epinephrine as buccal infiltration. A recent systematic review supports the use of articaine for patients with symptomatic irreversible pulpitis. They reported a significant advantage to use articaine over lidocaine for supplementary infiltration after mandibular block anesthesia but no advantage when used for mandibular block anesthesia alone or for maxillary infiltration.[32] On the contrary, optimal periodontal control of gingival bleeding could not be achieved in spite of meticulous oral hygiene performance and meticulous nonsurgical professional therapy. Initially, the gingival bleeding score was score 3 that was maximally improved to score 1 month following therapy.[33] Episodic relapse of gingival bleeding score to score 3 was also reported at three-month follow-up visit. These findings through light on the importance of meticulous systemic bleeding evaluation and control just before endodontic treatment even in case where the patient is under treatment, which situates the patient in a safe treatment level in order to prevent any complication during dental therapy. Patient pain score was 0, one day following therapy which was maintained after paracetamol tablet termination. Many studies contraindicated the use of acetylsalicylic acid and nonsteroidal anti-inflammatory drugs as pain killers in patients with bleeding disorders since it. However, this restriction is limited to the prolonged use of these drugs. They reported no restrictions regarding antibiotics.[20],[34] Follow-up of the case (24 months) revealed normal periapical tissue response to endodontic treatment, which reflects the normal regenerative capacity of the periapical area in such patients.

  Conclusion Top

Within the limitations of the present study, it can be concluded that the endodontic treatment of a VWD-affected patient is a successful therapy, provided that systemic bleeding is controlled and intrapulpal injection together with total tissue extirpation is performed. In this case, even after 24 months from follow-up, no periapical lesions were observed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Ambarkova V, Jovanovska A, Ambarkov J. Dental management of patient with Von Willebrand disease. Arch Clin Med Case Rep 2019;3:231-41.  Back to cited text no. 1
Gupta A, Epstein JB, Cabay RJ. Bleeding disorders of importance in dental care and related patient management. J Can Dent Assoc 2007;73:77-83.  Back to cited text no. 2
Veyradier A. Von Willebrand factor – A new target for TTP treatment? N Engl J Med 2016;374:583-5.  Back to cited text no. 3
Gajic M, Stevanovic R. Monograph: Handicapped Child in a Dental Office. Faculty of Dentistry, University of Belgrade Hendikepirano dete u Stomatoloskoj Ordinaciji. Stomatoloski Fakultet, Univerzitet u Beogradu. Beograd; 2002.  Back to cited text no. 4
Mehta R, Athar M, Girgis S, Hassan A, Becker RC. Acquired Von Willebrand Syndrome (AVWS) in cardiovascular disease: A state of the art review for clinicians. J Thromb Thrombolysis 2019;48:14-26.  Back to cited text no. 5
Mannucci PM. Hemophilia: Treatment options in the twenty- first century. J Thromb Haemost 2003;1:1349-55.  Back to cited text no. 6
Forés R, Lario A, Gil S, Campo-Cañaveral JL, Gomez-De-Antonio D, Laporta R, et al. Von Willebrand factor deficiency corrected by lung transplantation. Transplantation 2015;99:2663-4.  Back to cited text no. 7
Zaliuniene R, Peciuliene V, Brukiene V, Aleksejuniene J. Hemophilia and oral health. Stomatologija 2014;16:127-31.  Back to cited text no. 8
Horiuchi H, Doman T, Kokame K, Saiki Y, Matsumoto M. Acquired von Willebrand syndrome associated with cardiovascular diseases. J Atheroscler Thromb 2019;26:303-14.  Back to cited text no. 9
Kubicki R, Stiller B, Kroll J, Siepe M, Beyersdorf F, Benk C, et al. Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support. Eur J Cardiothorac Surg 2019;55:1194-201.  Back to cited text no. 10
Handin RI, Ewenstein BM. Von Willebrand's disease. In: Handin RI, Lux SE, Stossel TP. editors. Blood Principles and Practice of Hematology. 2nd ed.. Philadelphia: Lippincott Williams & Wilkins; 2003. p. 1103-30.  Back to cited text no. 11
Werner E. Willebrand disease in children and adolescents. Pediatr Clin North Am 1996;43:684-707.  Back to cited text no. 12
Werner EJ, Broxson EH, Tucker EL, Giroux DS, Shults J, Abshire TC. Prevalence of von Willebrand disease in children: A multiethnic study. J Pediatr 1993;123:893-8.  Back to cited text no. 13
Chapin J, Bamme J, Hsu F, Christos P, DeSancho M. Outcomes in patients with hemophilia and von Willebrand disease undergoing invasive or surgical procedures. Clin Appl Thromb Hemost 2017;23:148-54.  Back to cited text no. 14
Marques RV, Conde DM, Lopes FF, Alves CM. Dental care in patients with Hemophilia and von Willebrand Disease. Arquivos Odontol 2010;46:176-80.  Back to cited text no. 15
Dall'MagroAK, RibeiroAA, ShenkelA, Samuelsson M, Studzinski MS, Almeida D. Dental management of patients with bleeding - a literature review and case report: Bernard Soulier syndrome. RFO UPF 2011;16:193-9.  Back to cited text no. 16
Vire DE, Barrett KC. Endodontic Rx for the von Willebrand patient. J Endod 1982;8:514-6.  Back to cited text no. 17
Keila S, Kaufman A, Itckowitch D. Uncontrolled bleeding during endodontic treatment as the first symptoms for diagnosing von Willebrand's disease. A case report. Oral Surg Oral Med Oral Pathol 1990;69:243-6.  Back to cited text no. 18
Kumar NJ, Kumar A, Varadarajan R, Sharma N. Endodontics for the haemophiliac, a multidisciplinary perspective. J Conserv Dent 2007;10:59-63.  Back to cited text no. 19
  [Full text]  
Deonizio MDA, Kowalczuck A. Emergency endodontic care of patient with inconclusive diagnosis of von Willebrand disease. Dental Press Endod 2013;3:68-72.  Back to cited text no. 20
Atara RR, Shenoi PR, Mute WR, Makade CS, Mahajan AK. Endodontic management of patient with hemophilia. Int J Prosthodont Restor Dent 2013;3:101-4.  Back to cited text no. 21
Amer AI, Almushayt AS, El Meligy OA. Emergency endodontic treatment for von Willebrand's disease patient: A case report. J Oral Sci Health 2014;1:1-4.  Back to cited text no. 22
Kaul R, Shilpa PS, Sanjay CJ, David CM. Endodontic treatment in a patient with Hemophilia A: Case report with literature review. IJSS Case Rep Rev 2014;1:9-11.  Back to cited text no. 23
Dudeja PG, Dudeja KK, Lakhanpal M, Ali S. Endodontic management of a haemophilic patient- a clinical perspective. J Clin Diagn Res 2014;8:ZD17-8.  Back to cited text no. 24
Wilde JT, Cook RJ. von Willebrand's disease and its management in oral and maxillofacial surgery. Br J Oral Maxillofac Surg 1998;36:112-8.  Back to cited text no. 25
Vangelisti R, Pagnacco O, Ristagno G, Ruggeri M, Tosetto A, Castaman G, et al. Prevention of hemorrhage and dental treatment of patients with congenital or acquired coagulopathies. Minerva Stomatol 1997;46:621-6.  Back to cited text no. 26
Brewer AK, Roebuck EM, Donachie M, Hazard A, Gordon K, Fung D, et al. The dental management of adult patients with haemophilia and other congenital bleeding disorders. Haemophilia 2003;9:673-7.  Back to cited text no. 27
Staffileno H Jr., Giancio S. Bleeding disorders in the dental patient: Causative factors and management. Compend Contin Educ Dent 1987;8:501-7.  Back to cited text no. 28
Sonis AL, Musselman RJ. Oral bleeding in classic hemophilia. Oral Surg Oral Med Oral Pathol 1982;53:363-6.  Back to cited text no. 29
Whitrob MM. Clinical Hematology. 8th ed.. Philadelphia: Lea & Febiger Co.; 1981. p. 1167-75.  Back to cited text no. 30
Zimmerman TS, Ruggeri ZM. von Willebrand's disease. Clin Haematol 1983;12:175-200.  Back to cited text no. 31
Kung J, McDonagh M, Sedgley CM. Does articaine provide an advantage over lidocaine in patients with symptomatic irreversible pulpitis? A systematic review and meta-analysis. J Endod 2015;41:1784-94.  Back to cited text no. 32
Loe H, Silness J. Periodontal disease in pregnancy. I. Prevalence and severity. Acta Odontol Scand 1963;21:533-51.  Back to cited text no. 33
Franchini M, Rossetti G, Tagliaferri A, Pattacini C, Pozzoli D, Lorenz C, et al. Dental procedures in adult patients with hereditary bleeding disorders: 10 years experience in three Italian Hemophilia Centers. Haemophilia 2005;11:504-9.  Back to cited text no. 34


  [Figure 1], [Figure 2]

  [Table 1]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures
Article Tables

 Article Access Statistics
    PDF Downloaded186    
    Comments [Add]    

Recommend this journal