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Case Report
Dental management of hemophiliac child under
general anesthesia
Abstract
Hemophilia is the most common inherited bleeding disorder.
Hemophilic patients should be cosidered as special patients.
There is no contraindication to general dental treatment for
hemophiliacs, as they generally do not involve bleeding. But
caution must be used with any surgical procedures that involve
the local and general anesthesia. Such patients should always
be managed in the setting of specialized units with appropriate
clinical expertise and laboratory support. Recent advances in the
management of hemophilia have enabled many hemophiliac
patients to receive surgical dental procedures in an outpatient
dental care on a routine basis. The purpose of this case report
is to provide a few management strategies when providing
full mouth rehabilitation under anesthesia and replacement
therapies that are available. In addition, overviews of possible
complication that may be encountered when providing such
treatment are discussed here.
Key words
Full mouth rehabilitation, general anesthesia, hemophilia,
kidznteenz pediatric pentagon
Introduction
Among the congenital coagulation defects, hemophilia A,
hemophilia B (Christmas disease), and von Willebrands
disease are the most common.[1] Hemophilia is an
X-linked disorder with a frequency of about one in
10000 births.[2] Hemophilia is caused by a deficiency
of coagulation factor VIII (hemophilia A) or factor IX
(hemophilia B) related to mutations of the clotting factor
gene.[3] Although a case of female hemophilia has been
reported by Gilchrist in 1961, it manifest only in males.
Hemophilia A is more common than hemophilia B,
representing 80 to 85% of the total population. [2] Many
countries have started to establish care for hemophilia,
even though they do not have standard protocols to
ensure the proper management of hemophilia.
74 JOURNAL OF INDIAN SOCIETY OF PEDODONTICS AND PREVENTIVE DENTISTRY | Jan - Mar 2011 | Issue 1 | Vol 29 |
Rayen R, Hariharan VS1, Elavazhagan N2,
Kamalendran N3, Varadarajan R4
Reader, Department of Pedodontics and Preventive Dentistry,
Thai Moogambigai Dental College, Mogappair, 1Senior
Lecturer, Department of Pedodontics and Preventive Dentistry,
Sree Balaji Dental College and Hospital, 2Department of
anesthesia, Meenakshi Ammal medical College and Hospital,
Chennai, Tamilnadu, 3KidznTeenz Health Care Centre, 4Senior
Consultant Haematologist,Centre for Blood Disorders,T Nagar,
Chennai, India
Correspondence:
Dr. Roshan Rayen, Kidznteenz Healthcare Pvt Ltd, Exclusive
Pediatric Dental Centre, AH-48/175, Shanthi Colony 4th main
Road, Anna Nagar, Chennai. 600 040, India.
E-mail: kidznteenz@yahoo.in
Access this article online
Quick Response Code: Website:
www.jisppd.com
DOI:
10.4103/0970-4388.79954
PMID:
21521925
Oral healthcare providers must be aware of the impact
of bleeding disorders. In hemophiliacs, even the
routine dental treatment like extraction can produce
a life-threatening situation; hence, they must be given
special importance. Hence, these patients are best
accomplished both efficiently and effectively only by
the practitioners who are knowledgeable about the
pathology, complications, prophylactic, restorative,
and surgical dental treatment options associated with
these conditions.
Case Report
A 4-year-old boy came along with the parents to
the exclusive child specialty dental center with the
complaints of pain in the right upper back tooth
region. His parents gave the history of swelling in
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Rayen, et al.: Dental management of hemophiliac child
right upper jaw region for past two days. Medical
history revealed that at the age of 3 years, he had
prolonged and uncontrolled bleeding after a fall,
following which the patient was diagnosed with
hemophilia A. There was no history of factor
transfusion at that time. Family history yielded no
relevant details. There was no other significant history
of any medical disorder or exposure to anesthesia or
surgical intervention.
In clinical examination, following findings were noted:
51 - root stump, 54 - dentoalveolar abscess, 74 - deep
dental caries, 61,62,64,75,84,85 - moderate dental
caries, 73,72 - initial dental caries. Based on the clinical
findings, the treatment plan was designed. Considering
the age of the patient, complexity of the treatment,
and complication in the coagulation, it was decided to
carry on the treatment under general anesthesia. The
consent from medical, hematological, and anesthetical
care centers was obtained before the treatment. The
patient was scheduled for full mouth rehabilitation
and put under chlorhexidine mouthwash for a week, to
enhance the oral hygiene prior to the procedure.
Preanesthetic investigation values were as follows:
hemoglobin - 12.5gm%, prothrombin time -13 seconds,
and activated partial thromboplastin time (aPTT) -
39.88 seconds. Factor VIII concentration was found
to be 25% of normal activity. For the procedures, an
18G intravenous cannula was inserted. Based on the
discussion with the hematologist, it was decided to go
for the factor VIII transfusion, in which 200 Units as
a preoperative loading dose by slow infusion, half an
hour prior to treatment and 200 Units as a maintenance
dose 12 hours postoperative was done. Infiltration was
done by slow intravenous infusion.
The following treatments were done for the patient by
quadrant dentistry. In the Second Quadrant, 61, 62, 64,
65 were restored with composite after complete caries
removal and pulp protection.
In the third quadrant, composite restoration was
done in 75. In 74, pulpectomy followed by stainless
steel crown was given. During the pulpectomy, care
was taken to avoid over instrumentation in periapical
region. And stainless steel crown was placed with
smooth margin without impinging the gingival sulcus
[Figures 1 and 2]. The interdental carious lesions
in the gingival margins of 73 and 72 were left out
from extensive cavity preparation, as it may induce a
gingival bleeding. Spoon excavator was used to remove
JOURNAL OF INDIAN SOCIETY OF PEDODONTICS AND PREVENTIVE DENTISTRY | Jan - Mar 2011 | Issue 1 | Vol 29 |
the caries and fluoride varnish (FlourprotectorR) was
given to avoid further demineralization. In the fourth
quadrant 84 and 85, composite restorations were done
[Figures 3 and 4].
In the first quadrant, 51 was extracted atraumatically
[Figure 5]. The presence of mobility and intraoral
sinus made us [Figure 6] to opt for extraction, rather
than pulpectomy in 54. Care was taken to remove the
granulation tissue underneath the socket which may
prolong the bleeding. After extraction, the sockets
were irrigated with metronidazole thoroughly and local
pressure was applied. As the bleeding was continuous,
the sockets were packed with Surgicel* (Oxidized
Cellulose, Johnson and Johnson Co, India.), as suggested
by Gupta et al. [4] [Figure 7]. Then, resorbable sutures
were placed using vicryl 4-0 and nontraumatic needles.
During the entire procedure, complete isolation was
made by the saliva ejector by resting it on a moist
condensed sterile cotton roll in the floor of the mouth.
The vital parameters were well maintained throughout
the procedure. Postoperatively, factor VIII was infused
to the patient, twelve hours after the procedure, as per
the guidelines of hematologist. Patient was admitted
in the intensive care unit and vital signs were closely
monitored. Twelve hours postoperatively, slim bleeding
was noticed around the stainless steel crown margin.
It was controlled by the local placement of Surgicel.
Twenty-four hours postoperatively, slight extrusion of
Surgicel was found from the extracted socket, which
was removed carefully. After the consent from the
pediatrician, the patient was discharged. After a week
during the review, band and loop space maintainer was
given for 54 [Figure 8].
Discussion
Hemophilia A can be classified as severe (less than 1%
of normal factor VIII activity), moderate (1-5% of
normal activity), or mild (5-25% of normal activity).[4]
As the age increases, the childs physical activity
naturally increases, which results in more exposure to
trauma, especially in hemophiliacs, as stated by Scully
and Cawson.[5]
Hemophilia generally affects males on the maternal
side. In hemophiliacs, family history of bleeding is
commonly obtained. However, both FVIII and FIX
genes are prone to new mutation and as many as one-
third of all patients may not have a family history of
these disorders.[6]
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Rayen, et al.: Dental management of hemophiliac child

Figure 1: During pulpectomy procedure Figure 2: After stainless steel crown placement

Figure 3: After caries removal Figure 4: After composite restoration

Figure 5: After extraction and Surgicel packing in 51 Figure 6: Sinus in relation to 55

A prolonged aPTT will be seen in severe and Hemophilia B is much less common than hemophilia A,
moderate cases but may not show prolongation in mild and affects only 1/300000 males born alive. The clinical
hemophilia. A definite diagnosis can be made based on features will be very similar to that of hemophilia
FVIII or FIX factor assay. A, with a prolongation of aPTT. Few hemophilia B

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Rayen, et al.: Dental management of hemophiliac child
Figure 7: Surgicel packing for extracted socket
Pediatric dentist
Pediatrician Parents
Special child with
bleeding disorder
Anesthetist Hematologist
Figure 9: Kidznteenz pediatric pentagon
patients present an abnormal factor IX that slightly
prolongs the PT.[5]
In the evaluation and treatment of hemophiliacs, the
psychosocial aspect should never be underestimated. As
there are some reports that the emotional disturbance
is a contributing cause of spontaneous hemorrhage
in hemophiliacs,[7] the dentist must be alert to the
emotional problems of the young hemophiliac as well
as to the over-concern of the mother.
Preanesthetic management
If bleeding starts or is expected to start, Factor
VIII must be replaced to a level adequate to ensure
hemostasis. Though the heat and chemical treatment
of blood products began in 1986,[8] still plasma (fresh
or frozen), cryoprecipitate, or fractionated human
factor concentrates obtained from pooled blood sources
carry the risk of transmitting blood-borne pathogens
like hepatitis virus or HIV.[9] At present, replacement
of missing factor is commonly achieved with porcine
Factor VIII or recombinant Factor VIII.
One unit of Factor VIII concentrate per kilogram of
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Figure 8: After band and loop space maintainer placement during
follow-up
body weight elevates the Factor VIII level by 2%. The
amount of factor VIII to be infused was discussed and
decided with the hematologist.
Anesthetic procedures
After induction of anesthesia, extra care should
be taken while intubation of the airway as it can
cause submucosal hemorrhages, which can prove life
threatening. Nasal intubation was avoided, as it can
prove traumatic and bleeding from the site can lead to
aspiration. Care was taken during positioning of the
extremities and pressure points were padded to prevent
intramuscular hematomas or hemarthrosis.
Operative procedures
Surgery should be carried out with minimal trauma
to soft tissues and bone; careful postoperative mouth
toilet is essential. Especially, extractions should be
accomplished as atraumatically as possible.[10]
It is important to consider the local measures to
minimize the risk of postoperative bleeding. According
to authors,[3] suturing is desirable to stabilize gum flaps
and to prevent postoperative disturbance of wounds
by tongue movements. But, Brewer [1] reported in a
small series where sutures were not used routinely
and there was no significant increase in postextraction
hemorrhage. It must be decided, based on the age of
the patient, number of teeth and severity of the wound.
Resorbable and nonresorbable sutures may be used
at the operators discretion. In this case, resorbable
sutures were used, as it avoids need for the postoperative
removal [11] and the possibility of bleeding when the
suture is removed.
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Rayen, et al.: Dental management of hemophiliac child
A nontraumatic needle was used and the number of
sutures minimized as suggested by Shoa.[12] According
to Kumar et al.[3] vicryl sutures are preferred and
catgut is best avoided. If a nonresorbable suture such
as black silk is used, it should be removed within four
to seven days.
The local hemostatic measures recommended are
Surgicel, Oxidized cellulose, and fibrin glue. Tranexamic
acid, collagen, cyanoacrylate, and fibrin glues can also be
helpful. Local use of fibrin glue and swish and swallow
rinse of tranexamic acid before and after the procedure is
also suggested as a cost-effective alternative. [2] Based on
the previous discussions with hematologist, we opted out
for SurgicelR.Some authors recommended soft vacuum-
formed splints to provide local protection following a
dental extraction or prolonged postextraction bleed.[2]
But, Kumar et al. stated that packing of surgical site is
usually unnecessary if replacements have been sufficient.
[3]
Acrylic protective splints can be more troublesome
than helpful by accumulating debris within them which
results in sepsis.[3] As our patient was too young to
maintain the splint sterile, it was decided to avoid the
splint.
Preventive and restorative dentistry are of particular
importance to the hemophiliac since early dental
treatment minimizes the need for later oral surgery.
Every restoration in a hemophiliac eliminates a
potential extraction. The dentist need not compromise
the standard of dental service because the patient
is a hemophiliac. Poor dentistry does not prevent
complications, it merely aggravates them.
Powel et al.[13] stated that certain precautions taken
during restorative treatment greatly reduced the
hazard of excessive bleeding. Isolation with rubber dam
provides gingival retraction, improved visibility, and
minimizes the laceration of the buccal mucosa and lips.
Soft tissue trauma must be avoided as much as possible
and a matrix band may help to avoid gingival laceration.
In this case, as the treatment was done under oral
intubation, rubber dam was not used. But, extra care
was taken to protect the tongue, buccal mucosa, and
lips. Conservative extensions of gingival margins
were prepared, as it will ease the concern of soft tissue
injury. Wet cotton rolls were placed to reduce mucosal
bleeding.
During the entire restorative procedure, the saliva
ejector was placed carefully and judiciously. Evans and
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Aledort suggested that high-speed vacuum aspirators
and saliva ejectors can cause hematomas.[14] Trauma
from the saliva ejector was minimized by resting it on
a gauze swab in the floor of the mouth as suggested
by the author.[14]
If extraction is indicated, the possibility of conventional
endodontic treatment must also be considered. As per
the literatures, endodontic treatment generally is of
low risk to hemophiliacs. But the instrument reach
beyond the periapical foramen should be avoided. It is
important that the procedure be carried out carefully
with the working length of the root canal calculated,
to ensure that the instruments do not pass through the
apex of the root canal, which was followed in our case.
Saline and metronidazole (IV) were used as an
irrigant. Interestingly, some authors suggested sodium
hypochlorite, followed by calcium hydroxide paste to
control the bleeding. Formaldehyde-derived substances
may also be used in cases where there is persistent
bleeding or even before the pulpectomy.[6]
As suggested by many authors, the pulpal treatment
was followed with the stainless steel crown. Osamu
Chiono [7] quoted that the possible dental complications
in hemophiliacs include postsurgical hemorrhage,
periodontal bleeding secondary to placement of
stainless steel crowns, and hemorrhage due to trauma
to the soft tissues. Even though the care was taken to
maintain the smooth margins of SSC, slight periodontal
bleeding secondary to placement of stainless steel
crowns was noticed. It was controlled by local
hemostatic agents Surgicel.
Authors suggested that indirect pulp capping of vital
primary and permanent teeth, which permits removal
of carious dentin before pulpal exposure, was well
tolerated by hemophiliacs. According to MC Nicol et al,
any large, exophytic clot should be removed down to
the level of the socket as they may provide a pathway
for continued bleeding and prevent application of
adequate pressure to the site. During the postoperative
period, slight extrusion of Surgicel was found and it
was removed till the socket level carefully. [4]
For the band and loop space maintainer, band was
placed supragingivaly and was ensured that it was not
impinging the gingiva.
Postoperatively, a diet of cold liquid or semisolid food
should be advised to take for five to ten days.[3] Care
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Rayen, et al.: Dental management of hemophiliac child
was taken to watch for hematoma formation manifesting
as dysphagia, swelling, or hoarseness. Postoperative
antibiotics were advised as this will reduce the late
bleeding which is due to the infection. As Non-Steroidal
Anti-inflammatory Drug (NSAID) can aggravate
the bleeding tendency,[3] the safer alternatives like
acetaminophen was preferred.
Postoperative care also involves the use of chlorhexidine
gluconate, an antibacterial mouthwash. Usually,
mouthwash is advised for 30 to 60 seconds twice a day;
as the patient was preschool child, his parents were
advised to wipe oral cavity with gauze soaked with
chlorhexidine.
Conclusion
Dental care under general anesthesia for the special
children with bleeding disorders like hemophiliacs needs
a complete coordination of the parents, Pediatrician,
Hematologist, Anesthetist, and the Pediatric dentist,
with the ultimate focus on the child. So, keeping the
special child in the center, the Kidznteenz Pediatric
Pentagon [Figure 9] can be drawn to represent the
above conclusion. This will ensure the effective and
efficient management of the special child with the
bleeding disorder.
Hematologist is involved in this pediatric pentagon
for a special child with bleeding disorder. In similiar
instances a special child with other systemic problems
will involve the co-ordination of the concerned
speciality person to provide the necessary scientific
advice to handle that particular situation pertaining to
pediatric dentistry. Simultaneously, the pediatric dentist
and physician should choose the least traumatic course
of management affording the minimum amount of risk.
Finally, the circumstances must be carefully controlled
and the procedure should be carried out in the least
traumatic way possible.
Acknowledgement
The authors acknowledge Dr.Vijailakshmi Acharya M.D.S,
JOURNAL OF INDIAN SOCIETY OF PEDODONTICS AND PREVENTIVE DENTISTRY | Jan - Mar 2011 | Issue 1 | Vol 29 |
for providing the needed technical and scientific support to
carry out the treatment successfully.
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2. Israels S,Schwetz N,Boyar R,McNicol A. Bleeding disorders:
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10. Brewer A, Correa ME .Guidelines for dental treatment of
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12. Shoa DN.Apicoectomy on a hemophiliac performed in the
dental office with home care to prevent bleeding.J Mich Dent
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Source of Support: Nil, Conflict of Interest: Nil
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