Dental Management Of Patients With Bleeding Disorders

Give a brief account of bleeding disorders, their etiology, and management in dental practice.

Answer. Bleeding disorders: Bleeding disorders or hemorrhagic diatheses are a group of disorders characterized by defective hemostasis with abnormal bleeding.

Etiology Of Bleeding Disorders

Bleeding Disorders Vascular Defects:

Bleeding disorders caused by vascular defects may be caused by structural malformation of vessels.
Hereditary disorders of connective tissue and acquired connective tissue disorders.
Vascular defects rarely cause serious bleeding.
Bleeding into the skin or mucous membrane immediately alters trauma but ceases within 24 to 48 hours.
The vascular defects are hereditary hemorrhagic telangiectasia, Henoch-Schönlein purpura

Bleeding Disorders Platelet Disorder

Bleeding can be of two types:

Reduction in number: Thrombocytopenic purpura.
If the total number of circulating platelets falls below 50,000 per mm3 of blood the patient can have bleeding. In some cases the total platelet count is reduced by an unknown mechanism, this is called primary or idiopathic thrombocytopenic purpura (ITP).
Chemicals, radiation, and various systemic diseases, (e.g. leukemia) may have a direct effect on the bone marrow and may result in secondary thrombocytopenia.
Defect in quality: Non-thrombocytopenic purpura e.g. von Willebrand’s disease, Bernard-Soulier disease, Glanzmanns thrombasthenia.

Von-Willebrand’s disease (pseudo hemophilia) is the most common inherited bleeding disorder.

Unlike hemophilia, it can occur in females.
This is a disease of both coagulation factors and platelets.
It is caused by an inherited defect involving platelet adhesion.
Platelet adhesion is affected because of a deficiency of Von Willebrand`s factor.
Various drugs such as carbamazepine, aspirin, methyl dopa, and phenytoin can also lead to platelet disorders.

Bleeding Disorders Coagulation Defects

Hemophilia A: It is the most common coagulation defect.

Hemophilia A is inherited as an X-linked recessive trait.
The hemostatic abnormality in hemophilia A is caused by a deficiency/defect of factor VIII.
Until recently, factor VIII was thought to be produced by endothelial cells and not by the liver as most coagulation factors.
The defective gene is located on the X chromosome.
Hemophilia B (Christmas disease): Factor IX is deficient or defective. It is inherited as an X-linked recessive trait. Like
Hemophilia A, the disease primarily affects males and the clinical manifestations of the two are identical.
Disseminated intravascular coagulation (DIC): It is a condition that results when the clotting system is activated in all or a major part of the vascular system.
Despite widespread firin production, the major clinical problem is bleeding not thrombosis.
DIC is associated with many disorders such as infection, obstetric complications, cancer, and snake bites.

Management of Bleeding disorders

Hereditary Hemorrhagic telangiectasia

It is transmitted as an autosomal dominant trait and is characterized by bleeding from mucous membranes.

Bleeding Disorders Management

In patients having repeated attacks of epistaxis septal dermoplasty should be done.
In septal dermoplasty, mucosa gets removed and skin grafting is done.
If spontaneous hemorrhages are present or nasal bleeding is present, it is controlled by giving pressure packs.
Sclerosing agents i.e. sodium tetradecyl sulfate, were injected intralesionally to stop bleeding.
Electrocautery is done. It helps in arresting bleeding.

Bleeding Disorders Idiopathic Thrombocytopenic Purpura (ItP)

Bleeding Disorders Steroid Treatment Protocol

Initial steroid treatment protocol for ITP: Initial steroid treatment protocol l mg/kg/day prednisolone, PO for 2–6 weeks.
subsequent steroid treatment protocol for ITP: Prednisolone dose is individualized for every patient.

Usually, the dose of prednisolone is tapered to less than l0 mg per day for 3 months and then withdrawn.

Splenectomy is done, if discontinuation of prednisolone causes a relapse.

Follow the ’rule of twos’ for major dental treatment and provide extra steroids before surgery, if the patient is currently on steroids or has used steroids for 2 weeks longer within the past 2 years.

Minor surgery

Hemostasis after minor surgery is usually adequate if platelet levels are above 50,000 cells/mm3.
Platelets can be replaced or supplemented by platelet transfusions; though sequestration of platelets occurs rapidly.
Platelet transfusion is indicated for established thrombocytopenic bleeding.
When given prophylactically platelets should be given half before surgery to control capillary bleeding and half at the end of the operation to facilitate the placement of adequate sutures.
Platelets should be used within 6–24 hours after collection and suitable preparations include platelet-rich plasma (PRP), which contains about 90% of the platelets from a unit of fresh blood, and platelet-rich concentrate (PRC), which contains about 50% of the platelets from a unit of fresh whole blood.
PRC is thus the best source of platelets. Platelet infusions carry the risk of isoimmunization, infection with bloodborne viruses, and, rarely, graft-versus-host disease.
Where there is immune destruction of platelets (e.g. in ITP), platelet infusions are less effective.
The need for platelet transfusions can be reduced by local hemostatic measures and the use of desmopressin or tranexamic acid or topical administration of platelet concentrates.
Absorbable hemostatic agents such as oxidized regenerated cellulose (Surgicel), synthetic collagen (instant) or microcrystalline collagen (Avitene) may be put in the
socket to assist clotting in the post-extraction socket.
Drugs that affect platelet function, such as gentamicin, antihistamines, and aspirin should be avoided.

Major surgery

For major surgery, platelet levels over 75,000 cells/mm3 are desirable.

Bleeding Disorders Hemophilia

It is a hereditary disorder of blood coagulation characterized by excessive hemorrhage due to increased coagulation time. It is of two types, i.e. Hemophilia A and Hemophilia B.

Bleeding Disorders Management

Local anesthesia is contraindicated. So intrapulpal anesthesia and intraligamentary anesthesia should be used.
Sedation with diazepam or NO2–O2 sedation can be given.
Endodontic procedures should be carried out and care should be taken not to do instrumentation beyond apex.
If hemorrhage is present it should be controlled by 1:1000 aqueous epinephrine on paper point.
Restorative treatments can be carried out by proper application of rubber dam to avoid trauma to the gingiva and other soft tissues.
In case, if rubber dam is not present an epinephrine-impregnated hemostatic cord is kept in the gingival sulcus before the preparation of the crown or inlay margin.
Complete dentures and removable partial dentures can be given to hemophilic patients and are well tolerated by them.
The patient has to take care of proper maintenance of hygiene of the prosthesis.
Conservative periodontal treatment should be done rather than attempting periodontal surgeries.
In case, if oral surgical procedures are to be done local
hemostatic agents should be used, pressure surgical packs should be employed, and sutures and topical thrombin should be used.
After removal of tooth socket is packed with a mechanical splint.
Postoperative use of anti-fibrinolytic agents is used
to support clot maintenance.
In cases of Hemophilia A, Human freeze-dried factor VIII concentrate or new recombinant factor VIII is used.
In hemophilia B, human dried factor IX concentrate is supplied as a powder which is to be mixed with distilled water and administered IV

Bleeding Disorders Von Willebrand disease

It is the most common inherited bleeding disorder.
It is inherited as autosomal dominant but a severe form of disease may be inherited as a sex-linked recessive trait.
It is caused due to a deficiency or defect in the Von Wille brand Factor.
Types of Von-Willebrand diseases are Type I, Type II A and II B, and Type III.

Bleeding Disorders Management

Surgical procedures can be performed in patients with mild Von Willebrand disease by using DDAVP and EACA.
Patients with severe Von Willebrand disease require cryoprecipitate and Factor VIII concentrate.
Bleeding should be controlled by using local measures such as pressure packs, gel foam with thrombin, tranexamic acid, etc.
Aspirin and NSAIDs are avoided and acetaminophen can be given to patients.
In the majority of patients with Von Willebrand disease, the hemostatic defect is controlled with desmopressin via nasal spray.
Type I Von Willebrand disease is treated with desmopres sin while Type II A and B, and Type III require clotting factor replacement.

Bleeding Disorders Disseminated Intravascular Coagulation

Correction of hemodynamic instability by fluid therapy, transfusion of packed cells, or whole blood.
Factor replacement: This is the specific therapy, in this fresh frozen plasma, cryoprecipitate, and platelet concentrate transfusions are essential.
Fresh-frozen plasma is given at the dose of 15 mL/Kg. The platelet is transfused at the dosage of 0.1 unit/Kg.