DISCUSSION
Erik Adolf von Willebrand first recognized the disorder in 1926 in a study conducted on the inhabitants of the Aland Islands. Epidemiologic studies indicate that it is the most common bleeding disorder, affecting approximately 1% of the population. However, only a fraction of people come to medical and dental attention because of bleeding symptoms.[13] Table 2 shows the revised classification of vWD.[14]
vWD is an autosomally inherited congenital bleeding disorder involving a qualitative or quantitative deficiency of vWF. vWF is a protein that is critical for proper platelet adhesion and protects against coagulant factor degradation. This results in recurrent bleeding episodes. In fact, many cases of vWD go undiagnosed, and bleeding during dental treatment may be the first sign of underlying disease.[15] In this disease, there is factor VIII deficiency, prolonged bleeding, and activated partial thromboplastin time (aPTT), but prothrombin time and platelet count are normal.[15] Table 3 shows laboratory values for vWD, according to the National Heart, Lung and Blood Institute (NHLBI), 2012 update.[16]
Table 2  Revised classification of vWD
Table 3  Laboratory values for vWD, according to National Heart, Lung and Blood Institute (NHLBI), 2012 update   The results of the specific tests performed in this case to diagnose vWD are not suggestive of type 1 vWD, since the multimeric analysis and ristocetin-induced platelet aggregation (RIPA) are normal in type 1. Also, it is not type 3 vWD because all multimers and RIPA are absent in type 3. The pattern here is suggestive of type 2A or 2B vWD, given the low vWF: Ag and vWF: Rco and the VWF: Rco to VWF: Ag ratio being < 0.7.
A consanguineous marriage is usually defined as a marriage between people who are second cousins or closer.[17] As a result of these marriages, the rare autosomal recessive disorders run in close families and tribes. Thus, due to autosomal recessive inheritance, the rare congenital coagulation bleeding disorders, congenital platelet functional disorders, and vWD become common. Family history in this case revealed consanguineous marriage between the patient's parents. Pedigree analysis of the family revealed that the patient's father, mother, maternal and paternal uncles, and one of her three brothers were also suffering from vWD.
The most commonly reported symptom among women with the diagnosis of vWD or suspected bleeding disorder is menorrhagia, but additional symptoms or signs also may be present.[810] Treatments include ways to increase endogenous plasma concentration of vWF, replace vWF, or promote hemostasis without affecting vWF. With mild vWD and menorrhagia combination, hormonal contraceptives are the first-line treatment. In a study involving women with the diagnosis of vWD, 88% reported improvement in menorrhagia with oral contraceptives alone.[818] An exaggerated response to local irritants occurs in gingival tissues on intake of oral contraceptives in the form of inflammation that ranges from mild edema and erythema to severe inflammation with hemorrhagic and hyperplastic gingival tissues.[19] Kalkwarf reported that the response may be caused by an altered microvasculature, increased gingival permeability, and increased synthesis of prostaglandin.[20] Newer hemostatic agents include antifibrinolytics such as epsilon-aminocaproic acid and tranexamic acid.[21]
Endometrial ablation is a general term for any technique that removes or destroys the endometrium (lining of the uterine cavity). Endometrial ablation is a treatment for menorrhagia (heavy menstrual bleeding). It is an alternative to hysterectomy for the treatment of menorrhagia in a younger woman who has no desire for future fertility but wishes to preserve her uterus and in a older woman with obesity or medical contraindications for hysterectomy.[22] There are many different techniques for performing endometrial ablation. The entire endometrium is ablated by electrocautery using a resectoscope, roller ball, or a combination of two.[22] In a “roller ball” endometrial ablation, a ball-shaped electrode is used to deliver energy to the endometrium, not unlike a microwave. This destroys the endometrium. Success rates of nearly 90% are obtained with about 60% of the patients reporting amenorrhea.[22]
Treatment depends on the clinical condition of the patient and the type of vWD that is diagnosed. Available treatment options include cryoprecipitate, factor VIII concentrates that retain HMW vWF multimers (Humate-P, Koate-HS), and desmopressin (DDAVP). Patients with type 1 vWD are the best candidates for desmopressin therapy. It is not effective for type 3 vWD and most variants of type 2 vWD. These patients are treated with factor VIII replacement that retains the HMW vWF multimers. In short, more severe forms of vWD require preoperative factor VIII concentrates or cryoprecipitate infusion. Patients with milder forms respond favorably to administration of DDAVP (desmopressin) before periodontal surgery or tooth extraction.[2324]

Current dental management of vWD

Preoperative
Consult the hematologist and confirm the diagnosis. Establish the variant and treatment modality. Most patients can be treated in the dental office. Those with type 3 vWD may be hospitalized. Treat any acute oral infection. Maintain good oral hygiene. Construct palatal splints for multiple extractions in patients with type 3 or type 2N variants, so that mechanical displacement of the clot in wound healing by secondary intention is prevented.[25]

Operative
Treat with DDAVP, Epsilon-aminocaproic acid or tranexamic acid, or factor VIII replacement (Humate-P or Koate-HS) prior to the procedure. Use good surgical technique. Control bleeding using local measures like gelfoam, fibrin glue, etc., Place splint (palatal).[25]

Postoperative
Examine for signs of bleeding within 24–48 h. Additional doses of DDAVP, EACA, and factor VIII are given as needed. Examine for the signs of allergy to factor VIII. Bleeding can be managed through local means; if these fail, additional systemic therapy may be needed. If infection occurs, treat by local and systemic means. Avoid aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs). Acetaminophen with or without codeine may be used.[25]