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38 International Journal of Contemporary Surgery. January-June., 2013, Vol.1, No.

Management of Hemophilia Patients in Dentistry


Sachin Mittal1, Neha Koshal2, Vidhi Vinayak1, Mamta Malik3, Kaustubh Bhad4
Senior Lecturer, Dept. of Oral Medicine & Radiology, Shree Bankey Bihari Dental College & Research Centre,
Ghaziabad, Uttar Pradesh, 2Senior Lecturer, Dept. of Oral Medicine & Radiology, Indraprastha Dental College,
Ghaziabad, Uttar Pradesh, 3Senior Lecturer, Dept. of Oral Medicine & Radiology, Swamy Devi Dayal Hospital &
Dental College, Panchkula, Haryana, 4Senior Lecturer, Dept. of Oral & Maxillofacial Surgery, Karnavati School of
Dentistry, Ghandhi Nagar, Gujarat
1

ABSTRACT
Medically compromised patients are occasionally dealt with in dentistry. Sometimes patients with
bleeding and clotting disorders may also be encountered in our day to day practice. Hemophilia
being one of those clotting disorder which is commonly seen in males whereas females are carriers of
the disease. The management of such patients in various specialties of dentistry is discussed in this
article.
Keywords: Hemophilia, Tranexamic Acid, Factor VIII

INTRODUCTION
Hemophilia was seen in European royalty and thus
is sometimes known as the royal disease. Queen
Victoria passed the mutation to her son Leopold. The
first written account of hemophilia occurred in the 2nd
century in the Babylonian Talmud. In 1803, Dr. John
Conrad Otto recognized that the disorder was
hereditary and that it affected mostly males and was
passed down by healthy females. 1 The term
hemophilia is derived from the term
hemorrhaphilia which was used in a description of
the condition written by Friedrich Hopff in 1828.
Hemophilia A is the most common and best-known
clotting defect, with a prevalence of about 5 per 100
000 of the population. It is about 10 times as common
as hemophilia B except in some Asians, where
frequencies are almost equal. Inherited as a sex-linked
reces-sive trait, hemophilia affects males.2
Close cooperation is needed between patients
dentist and physician for such cases. So as to manage
these patients education of parents and the patients

Corresponding author:
Sachin Mittal
Senior Lecturer
Department of Oral Medicine & Radiology, Shree
Bankey Bihari Dental College & Research Centre,
Ghaziabad, Uttar Pradesh - 201302,
E-mail: sachin2627@yahoo.co.in

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38

should be started as early as possible, use of fluorides,


fissure sealants, decreased sugar rich diet is advised,
block anesthesia, lingual infiltrations should be
avoided; intraligamentary injections are safer so they
should be preferred. Intramuscular injections should
be avoided and care must be taken while placing dental
films, high speed vacuum and saliva ejectors.3 Other
precautions to be taken are mentioned below according
to respective speciality in dentistry.
Surgery and postoperative hemorrhage
Dental extractions and surgery are dangerous for
hemophiliacs. Surgery should therefore be carefully
planned to avoid complications. All necessary surgery
(and other dental treatment) should of course be
performed at one operation. Hemophiliacs require the
care of specialists of many disciplines and should
therefore be treated in Hemophilia Reference Centers,
or associated units. Hemophilia cards are issued to
confirmed hemophiliacs and give details of the
diagnosis and the Centre from which advice can be
obtained. Radiographs should be taken for any
unsuspected disease and to assess whether further
extractions might prevent future trouble.2
Injections
Local anesthesia should be avoided in the absence
of Factor VIII replacement. Regional (inferior dental
or posterior superior alveolar) blocks or injections in
the floor of the mouth must not be used since they can

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International Journal of Contemporary Surgery. January-June., 2013, Vol.1, No. 1 39

cause haemorrhage which, by allowing blood to track


down to cause airway obstruction, can be lifethreatening. Rarely, even submucosal infiltrations have
caused widespread hematoma formation, but
Intraligamentary injections may be safe. Infiltration
anesthesia may be used with caution and is adequate
for conservative work in children, but lingual
infiltration must be avoided. If factor replacement
therapy has been given, regional anaesthesia can be
used, provided the Factor VIII level is maintained
above 30 per cent, but infiltration is still preferable.
Intravenous midazolam or relative analgesia can be
used. Intramuscular injections should be avoided
unless replacement therapy is being given, as they can
cause large hematoma. Oral alternatives are in any case
satisfactory in most instances.4
Conservative dentistry
Conservative treatment of the primary dentition
and sometimes of the permanent dentition may be
carried out without anesthesia. If conservative
treatment is not tolerated without anesthesia, papillary
or intraligamentary infiltration may achieve sufficient
analgesia and is unlikely to cause serious bleeding. Soft
tissue trauma must be avoided and a matrix band may
help prevent gingival laceration. However, care must
be taken not to let the matrix band cut the periodontal
tissues and start gingival bleeding. A rubber dam is
also useful to protect the mucosa from trauma but the
clamp must be carefully applied. High speed vacuum
aspirators and saliva ejectors must be used with
caution in order to avoid production of hematomas.
Trauma from the saliva ejector can be minimized by
resting it on a gauze swab placed in the floor of the
mouth.5
Endodontics
Root canal treatment may obviate the need for
extractions and can usually be carried out without
special precautions other than care to avoid reaming
through the apex. Topical application of 10 per cent
cocaine to the exposed pulp is the choice for vital pulp
extirpation. However, in severe hemophilia, bleeding
from the pulp and periapical tissues can be persistent
and troublesome.4
Periodontal treatment
In all but severe hemophiliacs scaling can be
carried out under antifibrinolytic cover. Periodontal
surgery necessitates factor replacement.3

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39

Orthodontics
There is no contraindication to the movement of
teeth in hemophilia. However, there must be no sharp
edges to appliances, wires etc., which might traumatize
the mucosa.3
Minor surgery
Endotracheal intubation for general anesthe-sia
may cause bleeding from nasal trauma and is
dangerous in unprepared patients, but since
replacement therapy has to be given for the surgical
procedure, intubation can be carried out. An oral latex
cuffed endotracheal tube is recommended to minimize
trauma to the nasal and trachea lining.6
A Factor VIII level of between 50 and 75 per cent is
required for dental extractions. AHF may also need to
be given postoperatively but many patients can be
managed with antifibrinolytic agents given during the
subsequent 10 days. If oral bleeding recurs
postoperatively, Factor VIII must be given. Some
advise the administration of a further single dose of
Factor VIII as a routine on the fourth or fifth
postoperative day. However, this should be
unnecessary if adequate Factor VIII has been given
preoperatively. Antifibrinolytics significantly reduce
Factor VIII requirements.5
Tranexamic acid (Cyklokapron) is used in a dose
of 1 g (30 mg/kg) orally, four times daily starting 24
hours preoperatively. Tranexamic acid used topically
significantly reduces bleeding. 10 ml of a 5 per cent
solution used as a mouth rinse for 2 minutes, four times
daily for 7 days, is recom-mended.6
Desmopressin (deamino-8-D arginine vasopressin:
DDAVP) is a synthetic analogue of vasopressin which
induces the release of Factor VIIIC, von Willebrands
factor (vWF) and tissue plasminogen activator (tPA)
from storage sites in endothelium. Given as an
intra-venous infusion (0.3-0.5 g/kg just before
surgery, and repeated 12 hourly if necessary for up to
4 days), desmopressin can temporarily correct the
haemostatic defect in mild hemophilia.7
It is now avail-able for subcutaneous or intranasal
use when doses of 300 mg appear as effective as 0.2
mg/kg i.v. As desmopressin also causes release of
plasminogen activator, tranexamic acid should also be
given.7

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40 International Journal of Contemporary Surgery. January-June., 2013, Vol.1, No. 1

Local measures are also important to protect the


operation area and minimize the risk of postoperative
bleeding. Thus surgery should be carried out with
minimal trauma to both bone and soft tissues, and
careful mouth toilet postoperatively is also essential.

Suturing (though theoretically unnecessary) is


desir-able to stabilize gum flaps and to prevent
postoperative disturbance of wounds by eating.
Non-resorbable sutures are preferred and should
be removed at 4-7 days.

In the case of difficult extractions, when


mucoperiosteal flaps must be raised, the lingual
tissues in the lower molar regions should
preferably be left undisturbed since trauma may
open up planes into which haemorrhage can track
and endanger the airway.

The buccal approach to lower third molars is


therefore safer. Minimal bone should be removed
and the teeth should be sectioned for removal
where possible.

The packing of extraction sockets is unnecessary


if replacement therapy has been adequate but some
advice the packing of a small amount of oxidized
cellulose soaked in tranexamic acid into the depths
of the sockets.

Acrylic protective splints are rarely used now, in


view of their liability to cause mucosal trauma and
to promote sepsis, but they may be needed in
certain sites such as the palate. Local hemostasis
can be aided by collagen, Gelfoam or Surgicel
inserted into extraction sockets, and by
cyanoacrylate or fibrin glues.3

Prevention of infection
Antimicrobials such as oral penicillin V 250 mg four
times daily should be given postoperatively for a full
course of 7 days to reduce the risk of secondary
haemorrhage. Infection also appears to induce
fibrinolysis. Postoperatively, a diet of cold liquid and
minced solids should be taken for up to 10 days. Care
should be taken to detect hematoma formation which
may manifest itself by swelling, dysphagia or
hoarseness. The patency of the airway must always
be ensured.7
Major surgery
Before major surgery the patient is assessed by
haemostatic screening (AFTT, PT, and platelet count),

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40

Factor VIII assay, specific antibody test, fibrinogen


estimation, hepatitis B, C and HIV tests and liver
function tests. The patient should be admitted to
hospital and hemoglobin estimation carried, out. Blood
is also grouped and cross-matched for use in
emergency. Surgery is best carried out on Thursdays
and Fridays, since bleeding is most likely on the day
of operation or from 4 to 10 days postoperatively. All
surgical proce-dures must be covered with AHF which
is given 1 hour preoperatively. The dose of AHF given
before operation depends both on the severity of
hemophilia and the amount of trauma expected. Factor
VIII is effective only for about 12 hours and therefore
must be given regularly at least twice-daily
postoperatively for major surgery.3
Trauma to the head and neck
Hemophiliacs with head and neck injuries are at
risk from bleeding into the cranial cavity or into the
fascial spaces of the neck. They should, therefore, be
given factor replacement to a level of 100 per cent
prophylactically after a head or facial trauma. If there
are lacerations that need suturing, a minimum level
of Factor VIII of 50 per cent is required at the time,
with further cover for 3 days.5
CONCLUSION
Patients with hemostatic disorders are the regular
visitors to the dental clinics, so the knowledge about
these disorders is must for a dental physician. These
patients should be managed carefully in the dental
clinics to prevent undue complications for these
patients.
ACKNOWLEDGEMENT
We acknowledge Dr. Babita Singh, Lecturer, Shree
Bankey Bihari Dental College & Research Centre,
Ghaziabad, Uttar Pradesh.
Conflicts of Interest: None
Sources of Funding: None
Ethical Clearance: Not Applicable
REFERENCES
1.

Colman RW et al: Overview of hemostasis, in


Hemostasis and Thrombosis, 5th ed, RW Colman
et al (eds). Philadelphia, Lippincott Williams &
Wilkins, 2006, pp 3-16

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Little JW, Fallace DA, Miller CS, Rhodus NJ :


Dental management of medically compromised
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Crispian Sclly CBE, Roderick A Cawson : Medical
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Stajcic Z, Baklaja R, Elezovic I, et al. Primary
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Kaneda T, Shikimori I, Watanabe F, et al. The


importance of local hemostatic procedures in
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