Child Dental Patient with Hereditary clotting disorders - HAEMOPHILIA

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   the Child Dental Patient with


 

 

Hereditary clotting disorders

- Haemophilia -

 

 

Haemophilia

Definition: Hereditary clotting disorders due to factor VIII, IX or XI deficit  (*).


(* Merck Manual, 1999)

 

Genetic transmission of haemophilia

 

 

Females can also have haemophilia, but much rarer - when both X chromosomes are affected/one is affected and the other is missing or non-functioning. Bleeding symptoms can be similar to males with haemophilia.

 

Haemophilia

severe
  •  < 1% clotting factor
  •  profound spontaneous bleeding  in muscles and joints, cerebral bleeding
moderate
  •  2-5% clotting factor
  •  milder bleedings, usually after minor trauma/ bleeding therapeutic procedures
mild
  •  5-40% clotting factor
  •  longer bleeding after important trauma/ extractions

 

Dentistry      Hereditary clotting disorders


The first sign in very mild clotting disorders is heavy bleeding from a dental procedure, an accident, or surgery.

(Merck Manual, 1999)

The dentist can be the first medical professional in the position to suspect the disorder

 

Factors to consider in the management of a hemophiliac dental patient (*)

  • Dental neglect → need for frequent extractions
  • Trauma and surgery
  • Factor VIII inhibitors
  • Hazards of anesthesia and injections
  • Risk of hepatitis B,C and liver disease and HIV infection
  • Aggravation of bleeding by drugs
  • Anxiety
  • Drug dependence

    (*)Scully C. Medical problems in dentistry. 6th Ed. Elsevier, London 2010. 

 

Dental treatments in haemophiliac children


⇒ Endodontic therapy - non vital techniques are preffered – do not require protection
⇒ Infiltration anesthesia  - only under protection
⇒ Extractions

  • under haemathological control
  • require protection  
  • hospitalization

 

Restorative dental treatment

  • avoid brusque maneuvers during dental treatment
  • prevent accidental damage to the oral mucosa (saliva ejectors, placement of X-ray films/ sensors)
  • atention when using matrix bands, rubber dam, wooden edges→ risk of bleeding
  • tests and precautionary measures -  HIV, hepatitis B, C.

 

Preventive treatment

  •  Toothbrushing twice/day using medium texture brush
  •  Fluoride containing toothpaste:
    •  1000 ppm for children under 6 years old
    •  1400+ ppm for children over 6 years old.
  •  Dental floss or interdental brushes when possible
  •  Regular dental visits - starting with the first tooth eruption, every 6 months
  •  Early interception of orthodontic problems – avoid need for fixed appliances if possible (risk of bleeding), minimize crowding (can favour plaque retention and gingivitis, with increased risk of bleeding)

 

Dental anesthesia

  • local infiltration and periodontal ligament injection are preferred
  • nerve-block injections (inferior alveolar and posterior superior alveolar) can cause airway obstruction
  • anesthetic block or intramuscular injections must be preceded by replacement therapy in all cases
  • if FVIII levels<50% of the normal reference value: anesthetic block or intramuscular injections are contraindicated.

 

Extractions and dentoalveolar surgery

  • All necessary surgery -  in one operation (if possible)
  • Factor VIII level of 50–75% is required
  • Maintenance of normal Factor VIII levels for approx one week – in major surgery procedures and for moderate and severe patients
  • Non-traumatic needle, minimum number of sutures and  resorbable sutures are recommended
  • Post-operatively, a diet of cold liquid and minced solids - for  to 5–10 days

 

Dental care under GA (*)

  • Intubation  can cause submucosal hemorrhages (can be life threatening).
  • Avoid nasal intubation  - can be traumatic; bleeding from the site can lead to aspiration.
  • Carefully positioning of the extremities and pressure points  padding - to prevent intramuscular hematomas or hemarthrosis.

* Rayen R et al. (2011). Dental management of hemophiliac child under general anesthesia. Journal of the Indian Society of Pedodontics and Preventive Dentistry 2011; 29 74-9.

 

Pain control

  • Aspirin and it derivatives must be avoided
  • Acetaminophen and paracetamol = safe alternatives

 

For haemophilic children are mandatory:
 

  •   very good oral hygiene – to avoid gingivitis and gum bleeding
  •   regular and frequent dental check-ups
    •     - to avoid complicated caries lesions
    •     - interceptive therapy
  •   strict measures of asepsis and antisepsis - avoiding the spread of infectious diseases
  •  parents’ awareness about the consequences of the disease

 

? Possible questions from parents

  •  tooth eruption - normal

(!) traumatizing the overlaying gingiva during eruption may cause bleeding
 

  •  deciduous teeth shedding - normal

(!) extended mobility may impose extraction under protection
 

  •  orthodontic treatment -  yes, under haematological control. Interception if possible