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Table of Contents
Year : 2019  |  Volume : 7  |  Issue : 1  |  Page : 18-20

Hemophilic patients and orthodontics

1 Department of Pediatric Dentistry and Orthodontics Sciences, College of Dentistry, King Khalid University, Abha, Kingdom of Saudi Arabia
2 Department of Restorative Dental Sciences, College of Dentistry, King Khalid University, Abha, Kingdom of Saudi Arabia
3 Department of Dental Technology, College of Applied Medical Science, Abha, Kingdom of Saudi Arabia
4 Department of Periodontics and Community Dentistry, College of Dentistry, King Khalid University, Abha, Kingdom of Saudi Arabia

Date of Web Publication26-Jun-2019

Correspondence Address:
Dr. Shamsuddin V Shaheen
Division of Orthodontics, Department of Pediatric Dentistry and Orthodontics Sciences, College of Dentistry, King Khalid University, Abha
Kingdom of Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/INJO.INJO_19_19

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The most common congenital bleeding disorder is hemophilia. It is a pathology inherited and caused by a defective or an absence of the coagulation factors so that hemophiliacs cannot form an efficient clot. These patients have been treated with fear in the dental profession without having achieved the goals and ideals most appropriate according to the requirements of each case, which is demonstrated in the little existing literature available. However, they are currently treated as healthy orthodontic patients thanks to the advances in orthodontics. The review emphasis is on the importance of interconsultation with the hematologist, prevention, and oral hygiene and that of the beneficial effect of orthodontic.

Keywords: Hemophilia, Oral hygiene, Orthodontic patients, Orthodontics

How to cite this article:
Shaheen SV, Mohammed KM, Vinod MB, Ahmed MB, Mohamed KS, Jimly KJ. Hemophilic patients and orthodontics. Int J Oral Care Res 2019;7:18-20

How to cite this URL:
Shaheen SV, Mohammed KM, Vinod MB, Ahmed MB, Mohamed KS, Jimly KJ. Hemophilic patients and orthodontics. Int J Oral Care Res [serial online] 2019 [cited 2022 Aug 12];7:18-20. Available from: https://www.ijocr.org/text.asp?2019/7/1/18/261325

  Introduction Top

Orthodontic treatment leads to improvement in the appearance, increased confidence and satisfaction. Orthodontic treatment is fully consistent with the model of health that aims to improve life expectancy and quality of life.[1] Advances in medicine, more expectations from life’s quality have increased life expectancy, which results in increasing demand for optional dental and medical treatments.[2] Orthodontists need to be aware of some clinical requirements for a number of diseases. Further, orthodontists see their patients every 6–8 weeks. They can detect problems at its initial stage.[3] Hereditary deficiency of coagulation factor has caused 90% of hereditary diseases: hemophilia A, hemophilia B, and von Willebrand’s hemophilia A is a sex-linked disease and mostly seen in men. These patients are faced with coagulation factor VIII deficiency. The severity of bleeding problems depends on the level of factor VII in blood. Hemophilia B is due to the coagulation factor IX deficiency.[4] Dentists are sometimes in doubt about “Can people with hemophilia have orthodontic treatment?” and “what special considerations for orthodontic treatment will be provided to these patients?” and “Can people with hemophilia have orthognathic surgery?” Hemophilia is the most common congenital bleeding disorder.[4] It is a congenital hematological condition inherited and caused by a deficient activity or absence of clotting factors that are essential for normal hemostasis. Thus, people with hemophilia cannot efficiently form a clot.[5] The severity of hemophilia depends on the amount of this coagulation factor. The normal concentrations of clotting factor are between 50% and 150% of average value, and the minimum level of a factor for adequate hemostasis is 25%.[2] Hemophilia is classified as mild, when the decrease of factor VIII is 5%–25% of normal; moderate, when the decrease is 1%–5%; and severe, when the decrease is less than 1%. When levels of clotting factor is found below 1%, it can cause spontaneous bleeding.[2] There are different types of hemophilia[2],[4]: Hemophilia A or classic or deficit of factor VIII: this kind of hemophilia is the most frequent and presents in one in every 10,000 people. Approximately 80% of the affected have this type. Hemophilia B or Christmas disease or factor IX deficiency is present in 15 persons per million,[5] and represents 15% of those affected. The patient with hemophilia usually presents a normal analytical bleeding time (BT), platelet recount, and aggregation. This process is sufficient to produce hemostasis in small puncture wounds and lacerations. However, there appears an increased prothrombin time (PT) and/or thromboplastin; therefore, the clotting mechanism does not fulfill its normal function in these patients.[6]

  Case History of Patients with Hemophilia Top

A thorough medical and dental history and a complete review are essential components in the management of patients with hemophilia. It is of vital importance to know the type and severity of the disease, the presence or absence of inhibitor agent, the current medications, family history of the condition, previous hospitalizations, transfusions, other diseases (including hepatitis and human immunodeficiency virus [HIV] by replacement therapy), and previous dental treatments.[7] It is necessary to know, which are the invasive dental procedures that require appropriate preoperative coverage of the patient with hemophilia because there may be an increased risk of significant bleeding. Therefore, the dental management of these patients requires collaboration and integration of the hematologist and the professional in the oral cavity.[4],[8]

  Clinical Features of Hemophilia Top

Severe cases may manifest with massive intrauterine hemorrhage leading to still birth and neonatal intracranial hemorrhage. Tendency toward easy bruising and massive hemorrhage after trauma or minor surgical procedures are commonly encountered. Also, spontaneous hemorrhage from the middle ear, epistaxis, bleeding into the joints causing hemarthrosis, and bleeding into soft tissues may occur. Weight-bearing joints become hot, tender, and painful, leading to synovial hypertrophy, destruction of cartilage, and secondary osteoarthritis. Untreated muscle hematomas in calf muscle can cause a rise in pressure with eventual ischemia, necrosis, fibrosis, and subsequent contraction and shortening of Achilles tendon. Tissue hemorrhage forms tumorlike masses termed as “pseudotumors of hemophilia.”[9],[10] Complications in hemophiliacs include musculoskeletal complications such as chronic hemophilic arthropathy, synovitis, contractures, pseudotumor formation, development of inhibitors against factor VIII, and most importantly transfusion-related infections such as HIV, hepatitis B virus, hepatitis C virus, and hepatitis A viruses.[11],[12],[13]

  Oral Manifestations of Hemophilia Top

Hemophilia is characterized by bleeding from multiple sites, frequently manifested in the mouth as gingival and post-extraction hemorrhages. Patients may also have multiple oral bleeding events over their lifetime, depending on the severity of hemophilia. Bleeding episodes are more frequently encountered in severe hemophilia, followed by moderate hemophilia, and are minimal in mild hemophilia. Poor oral hygiene and iatrogenic factors can also induce the oral bleeding. In toddlers, oral ulcerations and ecchymosis involving lips and tongue are common.[14]

  Investigations of Hemophilia Top

Positive family history or a clinical history of exaggerated bleeding response to minor trauma gives a clue regarding the presence of hemophilia A. Laboratory diagnosis shows normal platelet count, normal BT, prolonged activated partial thromboplastin time (APTT), and normal prothrombin time (PT). Prolonged APTT in hemophilia A evaluates the intrinsic and common pathways of blood coagulation. APTT is considered normal if the control APTT and the test APTT are within 10s of each other. Control APTT is usually 25 ± 10s. It is determined by adding an activator to plasma (e.g., kaolin) along with an extract of phospholipid (to mimic plasma membrane). Normal BT assesses the vascular and platelet phases of blood clotting but is of limited sensitivity. PT test evaluates the extrinsic system and measures the presence or absence of clotting factors I, II, VII, and X. To standardize PT, in 1983, the World Health Organization introduced the international normalized ratio (INR). It is the ratio between the PT of a patient in seconds and a control PT standardized by means of International Sensitivity Index (ISI), which indicates the sensitivity of the thromboplastin used as reagent. In this context, human brain thromboplastin is assigned the reference standard of 1. An ISI greater than 1 indicates a less sensitive thromboplastin, whereas a value less than 1 indicates a more sensitive thromboplastin. For a PT value within the normal range, INR = 1. Definitive diagnosis is established by quantification of the procoagulant activity of factor VIII, which is found to be reduced in hemophilia A.[1],[2],[3],[4],[5],[6],[7],[8],[9]

  Orthodontic Treatment Management among Patients of Hemophilia Top

Identification of the patients based on clinical and family history, disease antecedents, and laboratory testing is an important phase. Consultation with a specialist to determine the type of disorder involved is essential. Counseling can help to increase the awareness of importance of good oral hygiene to avoid invasive dental care and reduce the number of visits to the dentist. The most important objective is to provide oral care and oral care instructions, which help orthodontic patients to prevent most prevalent dental diseases such as dental caries and gingival diseases. Teeth should be brushed at least twice daily for plaque control with toothpaste containing fluoride and mouthwashes of triclosan or chlorhexidine, which also help reduce plaque.[1] Minimizing the intake of any food containing sticky carbohydrates between meals reduces the possibility of pH drop below 5.5 (normal 7.0) and demineralization of enamel and dentine. Another approach is to use pit and fissure sealants to reduce the incidence of dental caries.[15],[16] Patients with hemophilia are susceptible to periodontal diseases more than ordinary people because of their inability to perform oral hygiene procedures.[17],[18],[19] Moreover, gingival sulcus hosts various aerobic and anaerobic organisms leading to periodontal destruction. Regular oral hygiene instructions and practice prevent these organisms from causing gingival inflammation and also develop in the patient an awareness of the need to return regularly for professional prophylaxis, examination, and treatment.[20] Orthodontic treatment or removable prosthesis is not contraindicated in the cases of hemophilia A.[21] However, these appliances may encourage plaque accumulation, which necessitates vigorous oral hygiene programs. Nevertheless, care must be taken to avoid damage to gingiva.[22],[23],[24],[25] Orthodontic treatment pain can usually be controlled with a minor analgesic such as acetaminophen. Aspirin is avoided as it has effect on platelet aggregation. The use of any nonsteroidal anti-inflammatory drug must be discussed with the patient’s hematologist because of its effect on platelet aggregation. Teeth should be brushed twice a day with a medium texture brush and a toothpaste containing fluoride to remove plaque deposits.

  Conclusion Top

The close cooperation between hematologists, general physicians, oral physicians and surgeons, and general dentists will help to provide utmost care and appropriate treatment for patients with hemophilia A, avoiding all unfavorable consequences. Genetic counseling is an important part of hemophilia care to help people with hemophilia, carriers, and their families to make more informed choices about having children where there is a possibility of having a child with hemophilia or risk of having another affected child and the options available. Orthodontist can not only provide complete oral care for patients with hemophilia A but also contribute in the genetic counseling through wide range of tests for diagnostic and carrier detection, as well as individual counseling.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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Gilchrist GS, Hammond D, Melnyk J. Hemophilia A in a phenotypically normal female with XX-XO mosaicism. N Engl J Med 1965;273:1402-6.  Back to cited text no. 3
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