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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 10  |  Issue : 6  |  Page : 314-316

An unusual oral manifestation of hemophilia in a child


1 Department of Pediatric Dentistry, Institute of Dental Sciences, SOA (Deemed to be University), Bhubaneswar, Odisha, India
2 Department of Community Dentistry, Institute of Dental Sciences, SOA (Deemed to be University), Bhubaneswar, Odisha, India

Date of Web Publication24-Dec-2018

Correspondence Address:
Dr. Sonu Acharya
Department of Pediatric Dentistry, Institute of Dental Sciences, SOA (Deemed to be University), Bhubaneswar, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jioh.jioh_183_18

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  Abstract 

Dentists must know the impact of blood dyscrasias in the management of their patients. A good history taking in these patients is required to prevent any untoward incident in the operatory, and more so if any invasive treatment is planned. Most bleeding episodes are not worrisome and do not cause any emergency. However, there are cases where emergency management is required and must be provided immediately. Here, we discuss an extraordinary case of an 8-year-old male child who started to bleed after a minor trauma to lips and the blood clot restricted his mouth opening, to the point that there was complete mouth closure.

Keywords: Bleeding, child, clot, disorder, hemophilia, mouth closure


How to cite this article:
Acharya S, Rathore K, Mahapatra U, Sethi S, Sahu N. An unusual oral manifestation of hemophilia in a child. J Int Oral Health 2018;10:314-6

How to cite this URL:
Acharya S, Rathore K, Mahapatra U, Sethi S, Sahu N. An unusual oral manifestation of hemophilia in a child. J Int Oral Health [serial online] 2018 [cited 2019 Jun 24];10:314-6. Available from: http://www.jioh.org/text.asp?2018/10/6/314/248429


  Introduction Top


Pediatric dentists play a major role in the maintaining the general health of all pediatric patients and particularly those with bleeding disorders such as hemophilia, von Willebrand disease, or platelet function disorders.[1] Bleeding disorders can be classified as coagulation factor disorders, platelet deficiencies, vascular disorders, and fibrinolytic defects.[2] Among these, hemophilia A, hemophilia B (Christmas disease), and von Willebrand disease are categorized under congenital coagulation defect disorders. Hemophilia encapsulates a group of disorders which are hereditary and caused by the deficiency of one or more of the clotting factors leading to prolonged coagulation time and excessive bleeding that can be serious. It is broadly divided into hemophilia A (deficiency of factor VIII), hemophilia B or Christmas disease (deficiency of factor IX), and hemophilia C or Rosenthal syndrome (deficiency of factor XI).[3] Hemophilia A is more common of the two types, causing almost 80%–85% of the total hemophilia. Hemophilia may be a challenge to the dental specialists due to the induction of bleeding during treatment, which may be fatal in certain cases. The oral manifestations are characterized by frequent bleeding from many places, frequently seen as gingival and postextraction hemorrhages.[4] Patients can also complain of multiple oral bleeding episodes during their life, which depends on the severity of hemophilia. Bleeding episodes are more frequently encountered in severe hemophilia. Hemophilia patients are considered to be a special group of patients as routinely done procedures may be fatal in them. It was seen that almost 14% of all hemophilia patients and 30% of cases with mild type of hemophilia have been diagnosed early following an episode of severe oral bleeding.[5] The most common area of oral bleeding was the labial frenum and the tongue.[6] Here, we discuss the case of an 8-year-old male child who had a minor trauma to the upper lip, but the consequences were almost life-threatening.


  Case Report Top


An 8-year-old male with a history of hemophilia A was referred to the pediatric department with a complaint of inability to open his mouth for 2 days due to blood clot inside mouth [Figure 1]. The child was not able to eat anything per oral. History revealed trauma to the upper lip while playing. The child had a slight bleeding episode wherein the bleeding could be controlled at home. The bleeding started again after some time for which the parents applied tablet tranexa (tranexamic acid) locally after making a powder of the tablets. The oozing continued, and the parents were advised to give clot factor VIII by the hematologist. The bleeding was controlled. However, at night, there was bleeding again and a clot was formed intraorally because of which the child was unable to open his mouth the next morning. The parents took him to a nearby hospital when the child was unable to eat anything till evening. The doctors referred the case to us as they suspected the bleeding to be from a tooth. The child was alright when visited the outpatient department. The only complaint was the inability to open the mouth. The hematologist was informed to be present in the dental operatory for any untoward incident which may occur. Factor VIII was given by the parents after consultation with the hematologist as the child was a known case of Hemophilia A. Informed consent was taken from parents. We parted the lips slowly and could see a big clot inside the mouth which was sticking with the lips [Figure 2]. A gauze piece wetted with povidone-iodine was used to separate the clot from lips and tongue [Figure 3]. The clot could be completely removed intact, and the child was able to open his mouth [Figure 4]. The mouth was checked thoroughly to see any other bleeding point. There was no further bleeding, and so the child was left with instructions to be careful next time while playing. The patient visited the department after 5 days for review, and no further bleeding was detected.
Figure 1: Initial presentation

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Figure 2: Clot formed

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Figure 3: Clot removal

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Figure 4: Clot removed

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  Discussion Top


The high vascularity of oral cavity is the reason for frequent hemorrhagic episodes in children afflicted with hemophilia.[6] The common areas of bleeding are the frenum of the lip and the tongue.[5] Spontaneous gingival bleeding can occur frequently, even when triggered by minor stimuli such as tooth brushing, abrasion by food, or infection. In these oral regions, there are a large number of engorged capillaries present near the surface.[7] Therefore, the tendency of bleeding increases, as the child's physical activity increases. Hemophilia A can also be classified according to its severity as severe, moderate, and mild.[8] In severe cases, there is <1% of factor VIII normal plasma level, patients tend to bleed spontaneously in cases of mouth abrasions, during eruption or shedding of deciduous teeth and even without much trauma. In situation of oral and dental needs, children should be managed with a multidisciplinary approach in connivance with a medical team, preferably in a hospital setup which has a hemophilia treatment center.[9] The patient was referred to us because we had a tertiary setup with hemophilia center. When planning dental treatment, it is important that practitioners know about the half-life of the each of the deficient clotting factor which might be needed. Operative sessions should be scheduled on consecutive days, if replacement therapies are planned to be given. Pediatric dentists play an important role because they might be the first to suspect a case of hemophilia in a child.[9] However, routine screening for blood disorders is hardly looked for in the clinical setting, because most children needing dental care do not provide any prior history of excessive bleeding. Many hemophiliacs' children, from tender age, might have attended dental operatory during their hematological control visit and received a more rigorous and effective caries prevention schedule than the general population. Furthermore, patients and their parents are better motivated for a better oral hygiene, by virtue of their medical condition. In addition, needed oral treatment is usually provided by an appropriate dental staff, who are very familiar with hemophilia in children, as an integral part of the medical review.[10] Ours was a unique case as the child was unable to open his mouth due to the blood clot and could not eat anything for 2 days which caused much concern to the parents. There are many complications seen with hemophilia, but this was one of the rare manifestations of hemophilia.


  Conclusion Top


Pediatric dentists must provide special attention and know the potential risks of bleeding disorders; hemophilia is a very common clotting disorder worldwide and it represents a serious challenge during the clinical practice, since routine dental treatment can produce life-threatening conditions. The clinician should be aware of all the different manifestations of hemophilia and be prepared to tackle such situations without much panic.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Shastry SP, Kaul R, Baroudi K, Umar D. Hemophilia A: Dental considerations and management. J Int Soc Prev Community Dent 2014;4:S147-52.  Back to cited text no. 1
    
2.
Patton LL. Bleeding and clotting disorders. In: Greenberg MS, Glick M, Decker BC, editors. Burket's Oral Medicine: Diagnosis and Treatment. 10th ed. Hamilton, ON: B.C. Decker; 2003. p. 45-77.  Back to cited text no. 2
    
3.
Rogaev EI, Grigorenko AP, Faskhutdinova G, Kittler EL, Moliaka YK. Genotype analysis identifies the cause of the “royal disease”. Science 2009;326:817.  Back to cited text no. 3
    
4.
Martínez-Rider R, Garrocho-Rangel A, Márquez-Preciado R, Bolaños-Carmona MV, Islas-Ruiz S, Pozos-Guillén A, et al. Dental management of a child with incidentally detected hemophilia: Report of a clinical case. Case Rep Dent 2017;2017:7429738.  Back to cited text no. 4
    
5.
Sonis AL, Musselman RJ. Oral bleeding in classic hemophilia. Oral Surg Oral Med Oral Pathol 1982;53:363-6.  Back to cited text no. 5
    
6.
Smith JA. Hemophilia: What the oral and maxillofacial surgeon needs to know. Oral Maxillofac Surg Clin North Am 2016;28:481-9.  Back to cited text no. 6
    
7.
Abed H, Burke M, Shaheen F. The integrated care pathway of nephrology and dental teams to manage complex renal and postkidney transplant patients in dentistry: A holistic approach. Saudi J Kidney Dis Transpl 2018;29:766-74.  Back to cited text no. 7
[PUBMED]  [Full text]  
8.
Brewer A, Correa ME. Guidelines for Dental of Patients with Inherited Bleeding Disorders. Vol. 40. Montreal: World Federation of Hemophilia Dental Committee; 2016.  Back to cited text no. 8
    
9.
Silva CA, Santana CL, Andrade RD, Fraga TD, Prado FO. Evaluation of dentistry students' knowledge on hematologic disorders. Rev Gaúch Odontol Camp 2016;64:287-92.  Back to cited text no. 9
    
10.
Freedman M, Dougall A, White B. An audit of a protocol for the management of patients with hereditary bleeding disorders undergoing dental treatment. J Disabil Oral Health 2009;10:151-5.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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Abstract
Introduction
Case Report
Discussion
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