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Coagulation Disorders
Congenital coagulopathies are deficiency of the clotting factors due to inherited disorders of coagulation which are essential in the coagulation cascade or deficiency of von Willebrand factor (vWF). The clinical bleeding can be different from mild to severe depending on the specific clotting factor affected and the level of factor deficiency. There are some common examples of them like Haemophilia A, Haemophilia B and von Willebrand disease.

Haemophilia A
Patients with haemophilia A exhibit bleeds into more deep-seated spaces. Severe bleeding is seen when the F VIII level is less than 1% of normal. Common signs include hematomas, hemarthrosis, hematuria, gastrointestinal bleeding and bleeding from lacerations or head trauma or spontaneous intracranial bleeding that require factor therapy.

Dental Manifestations
The dental manifestations are characterized by frequent bleeding of multiple sites, frequently seen as gingival and postextraction haemorrhages. The symptoms depend on the severity of haemophilia. In the case of severe haemophilia, patients may complain of multiple oral bleeding episodes throughout their life. Haemophilic patients are considered to be a special group of patients as routinely done procedures may be fatal in them. It was seen that almost 14% of all haemophilia patients and 30% of cases with a mild type of haemophilia have been diagnosed early following an episode of severe oral bleeding, of which the most common sites were the labial frenum and the tongue.

Dental Considerations
The inferior alveolar nerve block should only be given after raising clotting factor levels by appropriate replacement therapy, as there is a risk of bleeding into the muscles along with potential airway compromise due to a hematoma in the retromolar or pterygoid space. The intraligamental technique or interosseous technique should be considered instead of the mandibular block. Articaine has been used as a buccal infiltration to anaesthetize the lower molar teeth. A lingual infiltration also requires appropriate factor replacement since the injection is into an area with a rich plexus of blood vessels and the needle is not adjacent to bone.

Haemophilia B
Haemophilia B involves F IX (Christmas factor) deficiency. The genetic background, factor levels and clinical symptoms are same as haemophilia A. Patients can survive only with supportive transfusions and catastrophic bleeding can occur. The two main oral diseases affecting patients with haemophilia are the same as for the rest of the population - dental caries and gingivitis/periodontitis.

Dental Manifestations
Patients with bleeding disorders show a higher incidence of periodontal disease as well as dental caries, concerning the fear of bleeding which leads to a lack of oral hygiene and oral health care. The most prominent oral manifestation of a mild haemophilia B would be gingival bleeding during exfoliation of primary dentition, or prolonged bleeding after an invasive procedure/tooth extraction; In severe haemophilia, there may be spontaneous bleeding from the oral tissues (e.g. soft palate, tongue, buccal mucosa), lips and gingiva, with ecchymoses. In rare cases, hemarthrosis of the temporomandibular joint (TMJ) may be observed.

Patients with haemophilia will experience many episodes of oral bleeding over their lifetime. Average 29.1 bleeding events per year are serious enough to require factor replacement in F VIII-deficient patients which 90% involved oral structures. Children with severe haemophilia have significant lower prevalence of dental caries and lower plaque scores compared with matched, healthy controls.

Dental Considerations
Surgical treatment, including a simple dental extraction, must be planned to minimize the risk of bleeding, excessive bruising, or hematoma formation. Soft vacuum-formed splints can be used to provide local protection following a dental extraction or prolonged post-extraction bleed.

Von Willebrand Disease
von Willebrand disease (vWD) is the most common inherited bleeding disorder which can be due to quantitative or qualitative defects in vWF, a multimeric HMW glycoprotein. Clinical features of vWD are mild and include mucosal bleeding, soft tissue haemorrhage, rare hemarthrosis and menorrhagia in women.

Dental Manifestations
In the case of severe deficiency, there may be spontaneous gingival bleeding, ecchymosis, and epistaxis. Symptoms of vWD include postoperative bleeding/ bleeding after dental extraction, gingival bleeding, epistaxis and easy bruising. The intake of oral contraceptives as the first-line treatment for menorrhagia may lead to gingival enlargement and bleeding in women.

Platelet or coagulation disorders with severely altered hemostasis can cause spontaneous gingival bleeding, as seen in conjunction with hyperplastic hyperemic gingival enlargements in leukemic patients. Deposition of hemosiderin and other blood degradation products on the tooth surfaces which turn them brown can occur in cases of continuous oral bleeding over long periods.

The location of oral bleeds is as follows: labial frenum, 60%; tongue, 23%; buccal mucosa, 17% and gingiva and palate, 0.5%. Severe haemophilia will have most frequent bleeding occurrences, followed by moderate and then mild haemophilia. They mostly occur from traumatic injuries. Bleeding will also be induced by iatrogenic factors and poor oral hygiene practices. The frequency of oral haemorrhage by location in people with deficiency of F VIII and F IX is as followed: gingiva, 64%; dental pulp, 13%; tongue, 7.5%; lip, 7%; palate, 2% and buccal mucosa, 1%.

Dental Considerations
The protocols suggest the use of factor concentrate along with the use of local hemostatic techniques, such as suturing, and local measures, like use of oxidized cellulose, for example, Surgicel or fibrin glue in conjunction with post-operatively administered antifibrinolytic agents where appropriate.

The use of any non-steroidal anti-inflammatory drug (NSAID) must be discussed beforehand with the patient's haematologist because of their effect on platelet aggregation. There are no restrictions regarding the type of local anaesthetic agent used although those with vasoconstrictors may provide additional local hemostasis.