10
Bleeding Disorders
10.1 Haemophilia
Section I: Clinical Scenario and Dental Considerations
Clinical Scenario
A 36‐year‐old male attends for an emergency dental appointment at the end of the day. He complains of persistent dull pain of 2 weeks’ duration from the lower right second molar (#47). There is associated buccal swelling. Painkillers have been required daily and he requests dental extraction of this painful tooth.
Medical History
- Haemophilia A with inhibitors to factor VIII (FVIII)
- Prior to inhibitor development, required recombinant FVIII (rFVIII) on demand only (mild–moderate haemophilia)
- Inhibitors developed 7 years ago following high‐dose FVIII required after an ankle injury, converting patient’s phenotype to severe
- Initially required prophylactic recombinant factor VIIa (rFVIIa) when inhibitor titre was high
- As inhibitor titre has reduced, rFVIIa now only required on demand
- Left ankle arthrodesis (fusion) 1 year ago due to haemarthrosis
- Chronic hepatitis C infection acquired from blood transfusions as a child
Medications
- Recombinant FVIIa (on demand)
- Paracetamol as required
Dental History
- Irregular dental attender; only presents when he has dental issues
- Dental anxiety due to previous poor experiences with dental extractions
- Three wisdom teeth extracted under general anaesthesia 4 years ago
- Brushes teeth once daily at night only; no interdental cleaning
Social History
- Single, lives alone, no children
- Mixed heritage: Hispanic, Japanese and Anglo‐Saxon ethnic origin
- Mother carrier for haemophilia gene (lives overseas)
- Escort: friend as required but needs advance notice due to his job
- Occupation: support worker for adults living with disability; feels haemophilia has limited his employment opportunities (e.g. labour jobs)
- Walks with a limp; unable to walk long distances or run/jog due to previous left ankle injury
- Tobacco consumption nil
- Alcohol: consumes 20 cans of premixed alcohol‐containing drinks per week (~30 units)
Oral Examination
- Extensive caries on #36 and #47
- Multiple other smaller dental caries on #15, #21 and #22
- Moderate supra‐ and subgingival calculus
Radiological Examination
- Orthopantomogram undertaken (Figure 10.1.1)
- Extensive distal caries on #47 with pulpal involvement and periapical radiolucency
- Mesial caries on #36 with likely pulpal involvement
- Missing teeth #18, #28, #38 and #46
- Mild horizontal bone loss ~10%
Figure 10.1.1 Orthopantomogram showing extensive caries.
Structured Learning
- This patient has developed inhibitors to factor replacement therapy for his haemophilia. Why is this?
- Inhibitors (antibodies) are produced because the body sees the factor concentrates used to treat patients with haemophilia as foreign; this activates an immune response in the patient to destroy the foreign substances
- Additional risk factors in this patient for the development of inhibitors include:
- Patients with haemophilia A are more likely to develop inhibitors, i.e. approximately 20–30% of people with haemophilia A develop inhibitors, compared to <6% for patients with haemophilia B
- This patient received a high dose of factor replacement in relation to the ankle injury which may trigger a string immune response
- Patients of Hispanic (or African) heritage have an increased risk of inhibitor formation; the mechanisms that account for these racial/ethnic differences remain unclear
- The patient reports that his inhibitor level has fallen since first detected. Could he be correct?
- In approximately two‐thirds of cases, the inhibitors disappear on their own or with treatment known as immune tolerance induction
- Immune tolerance induction with the regular infusion of FVIII in small doses can induce FVIII antigen‐specific tolerance
- What is the significance of the patient’s inhibitor development when planning dental care?
- Although the patient reports that he now has low titres of factor inhibitor, advice of the haematologist must be sought to clarify this
- Clotting factor replacement may not be effective at preventing excessive bleeding if surgical dental procedures are planned
- Alternative agents which bypass the need for factor replacement may be required (e.g. recombinant FVIIa or FVIII inhibitor bypassing agent)
- However, bypassing agents are not as effective in controlling bleeding
- Furthermore, the cost of treatment is twice that for patients without inhibitors, partly due to the cost of the products required but also related to the need for admission in relation to increased bleeding risk
- This patient should be managed in a specialised dental unit with appropriate clinical expertise and laboratory support
- What factors do you need to consider in your risk assessment when planning dental treatment for this patient?
- Social
- Lives alone
- Needs advance notice to arrange an escort if required
- Reduced mobility
- High alcohol intake – may impact on his attendance, capacity and also his bleeding risk
- Medical
- Haemophilia complicated by development of inhibitors
- Potential for residual hepatic cirrhosis due to chronic hepatitis C; impact on bleeding, drug metabolism, development of hepatocellular carcinoma
- Dental
- Dental anxiety
- High sugar/acid consumption
- Poor oral hygiene habits
- Irregular dental attendance
- Social
- You advise dental extraction of #36 and #47 – the patient wishes to have #47 extracted the same day as his assessment appointment as the tooth is painful. Would you proceed?
- Do not extract the tooth on the same day – multiple factors need to be considered and appropriate treatment modifications put in place
- Focus on pain and infection control: review painkillers taken and consider draining any infection through the tooth/antibiotic prescription
- This patient is at particularly high risk of bleeding due to underlying haemophilia, presence of inhibitors, potential liver cirrhosis secondary to hepatitis C, leading to depletion of clotting factors, and the haematological effects of alcohol excess (see Chapter 15.5)
- Obtaining further information on liver function status is prudent to identify further potential issues with coagulation and drug metabolism
- Spontaneous bleeding in patients with haemophilia has also been reported under conditions of emotional stress – this patient has reported dental anxiety in relation to dental extractions
- Dental anxiety and risk of a failed procedure are high
- An escort is preferable and is not available on the day
- The patient has presented at the end of the working day – if he has bleeding issues, he will not be able to return to you/access routine support from the haematology team
- Close liaison with the haematologist is required; in this case the haematologist advised pre‐ and postoperative oral tranexamic acid in addition to rFVIIa and DDAVP; observation on the ward for 24 hour postoperatively was also recommended
- Following completion of dental extractions, the patient returns to you 4 months later and has developed further dental caries. What precautions would you undertake when providing the restorations?
- Avoid soft tissue trauma
- Care with matrix bands, wedges and rubber dam clamp positioning
- Care with high‐volume vacuum aspirators and saliva ejectors in the floor of mouth to avoid gingival bleeding/production of haematomas
- Care with taking mandibular intraoral radiographs
- Moisten cotton rolls to avoid sticking to mucosa
- Subgingival restorations performed using retraction cord with haemostatic solution, or local anaesthesia with epinephrine
- Tranexamic mouthwash may be useful to control bleeding
Figure 10.1.2 Spontaneous gingival bleeding.
Figure 10.1.3 Persistent bleeding from hyperplastic pulpitis (pulp polyp).
General Dental Considerations
Oral Findings
- Petechiae
- Ecchymoses
- Spontaneous gingival bleeding (Figure 10.1.2)
- Prolonged bleeding after invasive procedures (Figure 10.1.3)
Dental Management
- It is important to determine the severity of the haemophilia and the degree of invasiveness of the proposed dental treatment (Table 10.1.1; Figure 10.1.4)
- This will allow formulation of an appropriate plan in conjunction with the haemophilia team
- The use of topical haemostatic agents should be considered for invasive dental procedures (see Appendix I)
Table 10.1.1 General dental management considerations.
Risk assessment - Bleeding from surgery and trauma
- Mild haemophilia may go undiagnosed until prolonged bleeding results from dental extraction
- Impaired mobility and careful positioning (haemarthroses)
- Blood‐borne viruses in older patients with haemophilia due to unscreened blood transfusions
- Hepatitis (increased bleeding, impaired drug metabolism, cross‐infection risk)
- HIV (infection risks, cross‐infection risk)
- Analgesic drug tolerance due to chronic pain medications
Criteria for referral - Most patients with mild haemophilia can receive the majority of their routine dental care in the primary care setting, following the principles below:
- Initial liaison with patient’s haemophilia centre to confirm disease severity and management
- Focus on prevention of dental disease, with regular reviews tailored to the individual’s need
- Judicious planning of procedures likely to cause bleeding, with close liaison maintained between dentist and haemophilia centre. Prophylactic cover to be arranged by haemophilia unit. Postoperative management with tranexamic acid and local measures
- Invasive procedures performed in a specialised/hospital‐based dental unit, ideally linked to the haemophilia unit
- Dental treatment in patients with moderate/severe haemophilia should be performed in a specialist dental unit where available. The exception is if prior arrangements have been made between the haemophilia centre and primary care dentist
- Patients with inhibitors should be managed in specialised dental units with appropriate clinical expertise and laboratory support
Access/position - Many patients with haemophilia have experienced refusal of treatment by primary care dentists, so may avoid the dentist until in need of extensive treatment
- Ensure appropriate setting for invasive dental care
- Consider the need for an escort
- Timing of treatment: optimally 1 hour after factor replacement therapy, and preferably in the morning and early in the week
- Ideally perform treatment in 1 visit or on consecutive days to minimise the number of factor replacement sessions (thus likelihood of antibody development and costs); however, must be weighed up against the need to stage extractions to minimise bleeding risk
- Care with patient positioning due to haemarthroses and chronic pain/impaired mobility; consider ground floor surgery
Communication - Close liaison with the haemophilia team to confirm disease severity and management
Consent/capacity - Warn patient of the increased risk of intraoral/postoperative bleeding and intra/extraoral bruising
- Inform of the measures which will be used to reduce bleeding risk, so as to not discourage patient from attending appointments
- Capacity is not impaired unless there is brain haemorrhage/impaired cognition
Anaesthesia/sedation - Local anaesthesia
- No factor replacement is required for buccal infiltrations
- Regional block injections, lingual infiltrations or injections into the floor of mouth require factor replacement, due to risk of haemorrhage or life‐threatening airway blockage (80% risk of haematomas)
- Consider alternative anaesthesia methods: buccal, intraligamentary, intraosseous, papillary or electronic dental infiltrations
- Delivered using an aspirating syringe and should include a vasoconstrictor, unless contraindicated
- Anaesthetic solution should be delivered slowly to avoid rapid expansion of the soft tissues
- Sedation
- Oral sedation and nitrous oxide preferred
- Intravenous sedation cannulation poses a risk of haematoma formation, though this is rare under factor cover. Dorsum of the hand is preferred to the antecubital fossa
- General anaesthesia
- Hazards of anaesthesia, especially nasal intubation and intramuscular injections
- This must be considered when planning factor replacement
- Assessment by the anaesthetist prior to the day of planned surgery; must work in close consultation with haematologist
- Ensure haematology protocol followed, and that tranexamic acid, DDAVP and factor replacement are available as required
- Careful planning prior to surgery to ensure all necessary surgical dental treatment is performed in 1 session. This should include a detailed radiographic survey to identify any other teeth that may also require exodontia
- Overnight stay and postoperative follow‐up should be considered, especially if the patient has developed inhibitors
Dental treatment
Stay updated, free dental videos. Join our Telegram channel
VIDEdental - Online dental courses
