PRACTICA MEDICALĂ Ref: Ro J Med Pract. 2018;13(4) CERCETARE ŞTIINȚIFICĂ DOI: 10.37897/RJMP.2018.4.6 Minimal invasive oro-dental procedures – recommendations for optimal care in children with haemophilia CS II Andrei KOZMA1,4, PhD, MMD, MDHC, CS Emilia URSU2, MD, Conf. Roxana OANCEA3, MD, PhD, Assist. Prof. Ana Maria Alexandra STANESCU5, MD, PhD, Prof. Margit SERBAN2,4*, MD, PhD, Assoc. Prof. Smaranda ARGHIRESCU3, MD, PhD 1“Alessandrescu-Rusescu” National Institute for Mother and Child Health, Bucharest 2“Louis Turcanu” Children`s Emergency Hospital, Timisoara, Romania 3“Victor Babes” University of Medicine and Pharmacy, Timişoara, Romania 4Romanian Academy of Medical Sciences 5"Carol Davila" University of Medicine and Pharmacy, Bucharest ABSTRACT Oral and dental treatments are an essential condition for a good quality of life for hemifiliacs. Hemophilia is a hereditary disease that is manifested in male subjects and consists in lifelong alteration of antihemorrhagic mechanisms. Clinical investigations show that a significant percentage of haemophiliacs are diagnosed with bleeding of the oral and periodontal mucosa during both prosthetic and dental morphology restoration maneuvers. Small dental surgery procedures such as anesthesia, extirpation of dental pulp and other surgical procedures require pre- and postoperative treatment with Factor VIII or factor IX. The authors present steps that are indicated to be performed before, during and after dental treatments that can cause bleedings in the oral cavity. The area and mode of use of alternative or complementary hemostatic therapy is also presented, as well as the precautions required during local anesthesia. In conclusion, it can be said that people with haemophilia or congenital bleeding tendencies are a priority group for dental and oral health care, since bleeding after dental treatment may cause severe or even fatal complications. Maintenance of a healthy mouth and prevention of dental problems is of great importance, not only to quality of life and nutrition, but also to avoid the dangers of surgery. Keywords: haemophilia, dental treatment, antihemorrhagic factors, local anesthetics Dental and oral health represent an impor- 1/50,000 males in haemophilia B. They are con- tant condition for a good quality of life. To reach sidered severe when plasma activity - for F VIII it can be a challenging process in persons with or IX is <1 IU/dl, moderate if it ranges between haemophilia (1-3). 1-5 IU/dl and mild if it is between 5-40IU/dl. Haemophilia and related disorders are a Clinical investigators, estimating the nature of group of inherited bleeding disorders character- symptoms which are leading to the diagnosis, ized by a life-long defect in the clotting mecha- reported that 14% of haemophiliacs and 30% of nism. Haemophilia A and B are defined by their cases with mild haemophilia were diagnosed deficiency in Factor VIII and IX , respectively. based on a significant oral or dental bleeding They are rare diseases with an estimated fre- (3,4). quency of 1/10,000 males in haemophilia A and Corresponding author: Prof. Dr. Margit Serban, „Louis Turcanu” Children`s Emergency Hospital, Timişoara, Romania E-mail: [email protected] PRACTICA MEDICALÅ – VOL. 13, NR. 4(61), AN 2018 267 PRACTICA MEDICALÅ – VOL. 13, NR. 4(61), AN 2018 TABLE 1. Average age for tooth eruption TABLE 2. The need of hemostatic replacement therapy Deciduous (Primary) Teeth LOCAL ANESTHETIC TECHNIQUES Age (in months) NO HEMOSTATIC COVER HEMOSTATIC COVER Upper Lower REQUIRED REQUIRED 1.Central incisors 8-13 6-10 Bucal infiltration Inferior dental (ID) 2.Lateral incisors 8-13 10-16 Intra-papillary injections block Intra-ligamentary injections Lingual infiltration 3.Canines (cuspids) 16-23 16-23 4.First molars 13-19 13-19 In the following we will present some recom- 5.Second molars 25-33 23-31 mendations to be respected in our medical ac- Permanent Teeth tivity (6-9). Age (in years) Upper Lower First Steps – before stomatological treatment 1.Central incisors 7-8 6-7 2.Lateral incisors 8-9 7-8 • informing the dentist that the patient has 3.Canines (cuspids) 11-12 9-10 haemophilia before beginning any procedu- 4.First premolars (bicuspids) 10-11 10-12 re 5.Second premolars (bicuspids) 10-12 11-12 • people with bleeding disorders need close 6.First molars 6-7 6-7 cooperation between their physician and 7.Second molars 12-13 11-13 their dentist to receive safe, comprehensive 8.Third molars 17-21 17-21 dental care • in order to help the dentist for planing a cor- Physiological events (decidual or permanent rect course of treatment, it is important to tooth eruption, mobile tooth roots and natural- provide the following information ly falling out of decidual tooth) (Tab.1), but – the type and severity of hemophilia moreover medical interventions (treatment of – the medications used dental caries or of periodontal damage, dental – whether pre-treatment with factor con- plaque correction) or invasive procedures (tooth centrate, nasal desmopressin or an antifi- extraction, suturing of wounds or incisions, lo- brinolytic agent (tranexamic acid or epsi- cal anaestesia, fixed or removable orthodontic lon amino caproic acid) is required appliances, endodontic pulpectomy, anesthesia – contact information about the Hae- with inferior alveolar dental block or lingual in- mophilia Treatment Center filtration) can cause bleedings, that persist for – whether there are: days or weeks, generating life - threatening - inhibitors anti-FVIII or FIX complications, which can be stopped only with - infectious diseases, such as hepatitis, specific, disease - adapted measures. They can HIV infection occur even in asymptomatic haemophiliacs with - previous joint replacement moderate or mild disease (4,5). - venous access device (port) Taking all these into consideration, it is obvi- • dental appointments of children with blee- ous that successful protocols can only be the ding disorders, as well as education in pre- result of cooperation between dentist and he- ventive dentistry for children and caregivers, matologist , in frame of a comprehensive care should be started when the baby teeth begin team and center. The purpose of such guide- to erupt lines is the safety of the group of patients, vul- • comprehensive dental assessment is needed nerable from the point of view of their hemo- at the age of about 12 or 13, to plan for the stasis, presenting the risky burden of very rare future and to decide how best to forestall di- disorders, with unmet and not generally known fficulties resulting from overcrowding or mi- needs (Table 2 and Table 3). splaced third molars or other teeth TABLE 3. Suggested plasma factor peak level and duration of administration of coagulation factor concentrates in minor invasive interventions Haemophilia A Haemophilia B Type of bleeding Desired Factor Durati on Desired Factor Durati on level UI/kg dosage UI/kg days level UI/dl dosage UI/kg days Pre-operati ve 50-100 25-50 1 50-100 50-100 Post-operati ve 30-80 15-40 1-5 30-80 30-80 1-5 268 PRACTICA MEDICALÅ – VOL. 13, NR. 4(61), AN 2018 • for those with severe haemophilia, factor re- thrombocytopenia, which can worsen the placement is necessary before scaling, sur- bleeding tendency gery or regional block injections. For exam- • hepatitis C is extremely common in people ple, a dose of 50 international units per with haemophilia, can be associated with a kilogram of body weight (IU/ kg) of factor VIII prolonged prothrombin time or INR (Interna- is desirable before a tooth extraction in a tional Normalized Ratio) and thrombocyto- person with haemophilia A; in haemophilia penia; in such cases, bleeding cannot be pre- B, a dose of 100 IU/ kg of factor IX is required vented with factor VIII (or IX); fresh frozen (Table 3) plasma may be required. General tips during the dental treatment of TABLE 4. Alternative or adjunctive hemostatic therapy haemophilic pacients Medicati on Dosage Route • fillings, scalling, root canal and all cosmetic Desmopressin (DDAVP) 0,3 μg/ kg IV, SC and preventative care can be carried out ro- Tranexamic acid 25mg/ kg x 3/ d IV, oral, topic utinely on patients with mild bleeding disor- Epsilon-amino-caproic 50mg/ kg x 4/ d IV, oral, topic acid (EACA) ders • however, care should be taken with the soft Areas of concerns tissues, especially the use of impression trays, aspirators and x-ray films in the floor of • extractions and surgery the mouth • gingival surgery/ deep root planning and • for people with mild or moderate haemophi- • implant placement , all pose a bleeding risk, lia, non-surgical dental treatment can be car- even in patients with mild haemophilia; so, it ried out under antifibrinolytic cover, but a is essential that these procedures are carried hematologist must be consulted before out with the necessary pre-operative special other procedures are done (Table 4) measures such as factor replacement, DDAVP • for people with mild haemophilia A (with or Cyclokapron tablets factor VIII > 10%) and most people with VWD • deep injections, surgical procedures particu- (type 1), scaling and some minor surgery larly those involving bone (extractions, den- may be possible under desmopressin tal implants) and regional local anesthetic (DDAVP) cover; however, DDAVP is not effec- blocks should be avoided, where possible, as tive in haemophilia B (even mild cases) as it they may start a bleeding crisis does not boost factor IX levels • local use of fibrin glue and swish-and- • tranexamic acid used tropically significantly swallow rinses