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Table of Contents   
CASE REPORT  
Year : 2022  |  Volume : 33  |  Issue : 2  |  Page : 219-222
Glanzmann thrombasthenia: Use of hemocoagulase (BotroClot) for arrest of bleeding during a primary tooth endodontic procedure


1 Department of Pediatric and Preventive Dentistry, Faculty of Dental Sciences, Ramaiah University of Applied Sciences, Bengaluru, Karnataka, India
2 Department of Preventive Dentistry, College of Dentistry, Riyadh Elm University, Riyadh, Saudi Arabia; Department of Pediatric and Preventive Dentistry, Sharad Pawar Dental College and Hospital, Datta Megha Institute of Medical Sciences (Deemed to be University), Wardha, Maharashtra, India

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Date of Submission23-Dec-2021
Date of Decision15-Jun-2022
Date of Acceptance17-Jun-2022
Date of Web Publication13-Oct-2022
 

   Abstract 


The Rationale: Glanzmann thrombasthenia is a rare platelet disorder affecting 0.0001% of the population. Dentists may often be unaware of this condition, and manipulation of soft tissue can lead to grave consequences, which may even result in fatality. Patient Concerns: In this case report, a 4-year-old patient with Glanzmann thrombasthenia reported to the department with a chief complaint of a discoloured tooth. Clinical Findings: On examination, 51 was nonvital, and pulpectomy was the treatment planned. The non-vital anterior tooth was treated with a pulpectomy procedure. There was uncontrolled bleeding during the procedure. Treatment: A topical solution of BotroClot was used to arrest the bleeding, and obturation was completed following that. The post-operative period was uneventful. Take-away Lessons: Case report explored the use of a topical hemostatic agent to arrest bleeding from the canal. This case report warrants eliciting a thorough medical history before any dental procedure.

Keywords: BotroClot, Glanzmann Thrombasthenia, haemocoagulase, primary teeth

How to cite this article:
Jairam LS, Kotha SB. Glanzmann thrombasthenia: Use of hemocoagulase (BotroClot) for arrest of bleeding during a primary tooth endodontic procedure. Indian J Dent Res 2022;33:219-22

How to cite this URL:
Jairam LS, Kotha SB. Glanzmann thrombasthenia: Use of hemocoagulase (BotroClot) for arrest of bleeding during a primary tooth endodontic procedure. Indian J Dent Res [serial online] 2022 [cited 2022 Nov 29];33:219-22. Available from: https://www.ijdr.in/text.asp?2022/33/2/219/358436



   Introduction Top


Glanzmann thrombasthenia (GT) is a qualitative platelet disorder characterized by a deficiency in the platelet membrane glycoproteins (GP) IIb/IIIa.[1] It is one of the rarest congenitally inherited conditions. Glanzmann thrombasthenia (GT) was first described in 1918 by Dr. Edward Glanzmann who described a functional abnormality of platelets with a defective clot retraction. Glanzmann's thrombasthenia is a well-defined inherited disorder of blood platelets characterized by normal platelet counts; normal prothrombin (PT) and partial thromboplastin times (PTT); a prolonged bleeding time; abnormal clot retraction; and the absence of platelet aggregation in vitro in the presence of adenosine diphosphate (ADP), epinephrine, collagen, or thrombin but normal aggregation in response to ristocetin.[2]


   Case Report Top


A 4-year-old male patient reported to the Department of Pedodontics and Preventive Dentistry with a chief complaint of a discoloured anterior tooth. The mother gave a history of a fall that the child had 3 months ago while climbing the stairs. The episode of fall was associated with bleeding from the maxillary anterior region, which did not stop for 10 minutes. The parents applied constant finger pressure with a piece of cotton for 8–10 minutes to arrest the bleeding. On eliciting medical history, the parents reported episodes of prolonged bleeding following minor trauma and hematemesis since the age of 1. The mother reported recurrent episodes of epistaxis when the child was 1 year old, which had prompted them to visit a hospital.

Family history revealed that he was born out of a consanguineous marriage. There was no significant family history of bleeding disorder. Past blood investigations revealed a normal platelet count and morphology, prolonged bleeding time, decreased clot retraction and abnormal platelet aggregation. Immunisation history was uneventful. Milestones were normal. Currently, the patient was under the care of a physician and was not on any medication. The parents were advised to be cautious when the child played to prevent the child from getting bruised or having a fall that could provoke bleeding.

On examination, the child was conscious and cooperative (Frankl rating 3) and oriented. He had a mesomorphic built, normal gait and was well oriented. Intra-oral examination showed a full complement of primary teeth as seen in [Figure 1]. Petechial spots were seen in the floor of the mouth and buccal mucosa depicted in [Figure 2] and [Figure 3]. Tooth 51 was discoloured suggestive of a non-vital tooth. An intra-oral periapical radiograph was performed with respect to tooth 51 to confirm adequate root length.
Figure 1: Intra-oral photograph showing non-vital 51

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Figure 2: The floor of the mouth showed a petechial spot

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Figure 3: The left buccal mucosa showed petechial spots

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With consent from the mother, a pulpectomy procedure was planned as a treatment for the non-vital 51 tooth. On performing an access opening [Figure 5], bleeding was encountered in the canal, which was persistent even after thorough irrigation with saline and chlorhexidine [Figure 6]. A cotton pellet soaked with BotroClot solution [Figure 4] was inserted into the canal [Figure 7]. Bleeding was arrested after 5 min [Figure 8]. A clot was formed inside the canal. Careful irrigation was done by keeping the syringe 2 mm short of the working length until the entire clot was dislodged. The canal space was dried using paper points 2 mm short of working length, and the root canal space was obturated using zinc oxide eugenol paste as an obturating material [Figure 9]. Resin-modified glass ionomer cement was used to seal the access cavity. The use of suction was avoided throughout the procedure to protect the tissues from bruising. Isolation was obtained using cotton rolls.
Figure 4: BotroClot topical solution

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Figure 5: Access opening done with respect to 51

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Figure 6: Uncontrolled bleeding encountered

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Figure 7: Use of cotton soaked in BotroClot solution

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Figure 8: Arrest of bleeding after the use of BotroClot solution

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Figure 9: Obturation of tooth 51

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When a case of uncontrolled bleeding is encountered in patients with bleeding disorders, it is invaluable to have a topical hemostatic agent, which could help in the timely management of the patient.


   Discussion Top


The pediatric dentist plays a pivotal role in the diagnosis of this condition. As they see children early in life, the oral cavity can reveal some clues about this condition. Spontaneous mucosal bleeding, petechiae, purpura or ecchymoses in the mucosa, excessive bleeding following a tooth extraction, gingival bleeding during teething or shedding of teeth are suggestive of an underlying bleeding disorder. The differential diagnosis includes Bernard–Soulier Syndrome, Von Willebrand disease and platelet secretory defects.[2] In the present case, the child has had epistaxis since the age of 1 and was diagnosed with GT at the age of 2. The investigations revealed 10% to 20% of normal GPIIb-IIIa levels, which suggested a less severe variant of GT.

The most common clinical features that are seen are petechiae. These petechiae develop because of local pressure. Generally, they spontaneously disappear. Bruises may also be seen. These bruises are termed 'ecchymoses'. Ecchymoses tend to invite attention as they cause diffuse swelling and give a bluish discolouration under the skin. The patient can also present with bleeding from the mucosa. Mucosal bleeds can involve the mucous membranes of the mouth, nose, rectum, intestine, vagina and uterus. Mucosal bleeding can be a result of trauma in the form of irritation such as dental cleaning, gastroenteritis, rhinitis, etc. Certain childhood habits such as nose-picking combined with a superficial plexus of arterioles in the nasal mucosa increase the vulnerability of epistaxis.[3],[4] During the COVID-19 pandemic, a nasal swab was the mode of diagnostic testing that increased the vulnerability of epistaxis in patients with bleeding disorders.[5] Gingiva is another common site for spontaneous bleeding and it's more in children who present with poor oral hygiene.[6] In females, the onset of menarche predisposes the child to menorrhagia and is a cause of concern that it may require a blood transfusion.[4] Other sites of bleeding include gastro-intestinal haemorrhage, haematuria and haemarthroses, which if present will be severe.[7]

BotroClot is a haemocoagulant that is obtained from the venom of the Brazilian snake Bothrops jararaca or atrox which is approved by the WHO. It contains hemocoagulase derived from Bothrops atrox or Bothrops jararaca, chlorhexidine as a preservative and water for injection IP q.s. The mechanism of action is by accelerating the formation of fibrin at the site of haemorrhage. Through its action on factor XIIIa, it stabilizes the fibrin. The topical haemocoagulase properties are well documented; therefore,[8] we decided to use this to achieve arrest of bleeding from the canal.

To date, there is no known cure for this condition. Management strategies are directed toward the control of hemorrhage, which is the main clinical concern. In mild to moderate episodes, local measures and/or anti-fibrinolytic agents may be employed. Anti-fibrinolytic agents are effective for arresting mucocutaneous bleeding and reducing bleeding during dental procedures. The local measures include topical thrombin, compression, gelatin sponges and fibrin sealants. In event of a heavy bleeding episode or prior to an invasive procedure, platelet transfusion may be necessary.[9] Apart from transfusion, the treatment is primarily focused on preventive care. Prescription of drugs that affect platelet functions like aspirin or non-steroidal anti-inflammatory drugs (NSAIDs) should be avoided. In cases of menorrhagia, oral contraceptives may be prescribed. Desmopressin (dDAVP) has been tried, but not much success has been reported because it does not have a direct effect on platelets.[10] In some rare cases, bone marrow transplant has been successfully tried, but this is a complex treatment modality. The rarity of the condition makes it difficult to assess the morbidity and mortality associated with it. However, the general prognosis is good in most patients.[10] The responsibility of dentists lies in identifying any underlying bleeding disorder, which will alter the treatment protocol in such patients.


   Conclusion Top


The role of the pediatric dentist is vital as they are often the first to see the patient during childhood. Although Glanzmann thrombasthenia is a rare bleeding disorder, one should be aware of this condition while evaluating patients with spontaneous gingival bleeding or the presence of mucosal petechiae in the setting of normal platelet count. Early diagnosis and prompt treatment carry a good prognosis. It is imperative to begin with preventive therapy by educating the patient to maintain good oral hygiene. The patient should be educated regarding measures to avoid bleeding episodes which include avoidance of anti-platelet drugs and trauma and maintaining good oral hygiene. Periodic oral assessment is important to prevent dental health problems, thereby improving the quality of life.

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.
Sebastiano C, Bromberg M, Breen K, Hurford MT. Glanzmann's Thrombasthenia report of case and review of the literature. Int J Clin Exp Pathol 2010;3:443-7.  Back to cited text no. 1
    
2.
Bhavyaa R, Vignesh KC, Muthu MS, Haridoss S, Abirami S. Glanzmann Thrombasthenia: Use of the soft splint with tranexamic acid paste to reduce spontaneous oral bleeding. Int J Clin Pediatr Dent 2021;14:580.  Back to cited text no. 2
    
3.
Recht M, Chitlur M, Lam D, Sarnaik S, Rajpurkar M, Cooper DL, et al. Epistaxis as a common presenting symptom of Glanzmann's thrombasthenia, a rare qualitative platelet disorder: Illustrative case examples. Case Rep Emerg Med 2017;2017:8796425.  Back to cited text no. 3
    
4.
Botero JP, Lee K, Branchford BR, Bray PF, Freson K, Lambert MP, et al. Glanzmann thrombasthenia: Genetic basis and clinical correlates. Haematologica 2020;105:888-94.  Back to cited text no. 4
    
5.
Pagella F, Lizzio R, Ugolini S, Spinozzi G, Maiorano E, Suppressa P, Sabbà C, Matti E. Diagnostic testing for SARS-CoV-2 infection in HHT patients: nasopharyngeal versus oropharyngeal swab. Orphanet Journal of Rare Diseases. 2020;15:1-3.  Back to cited text no. 5
    
6.
Venkat V, Kalluri S, Hanumantha SR. Glanzmann's Thrombasthenia–A rare case report of spontaneous gingival bleeding. J Indian Acad Oral Med Radiol 2018;30:88-91.  Back to cited text no. 6
  [Full text]  
7.
Poon MC, Di Minno G, d'Oiron R, Zotz R. New insights into the treatment of Glanzmann Thrombasthenia. Transfus Med Rev 2016;30:92-9.  Back to cited text no. 7
    
8.
Shenoy V, Baliga M, Mahajan S, Ramesh KV. The effects of topical hemocoagulase solution on the healing process of post-extraction wounds: A split mouth design. J Max Oral Surg 2015;14:586-93.  Back to cited text no. 8
    
9.
Franco R, Miranda M, Di Renzo L, De Lorenzo A, Barlattani A, Bollero P. Glanzmann's Thrombastenia: The role of tranexamic acid in oral surgery. Case Rep Dent 2018;2018:9370212.  Back to cited text no. 9
    
10.
Sebastiano C, Bromberg M, Breen K, Hurford MT. Glanzmann's thrombasthenia: Report of a case and review of the literature. Int J Clin Exp Pathol 2010;3:443-7.  Back to cited text no. 10
    

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Correspondence Address:
Dr. Lalitha S Jairam
Department of Pediatric and Preventive Dentistry, Faculty of Dental Sciences, Ramaiah University of Applied Sciences, Bengaluru - 560 096, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdr.ijdr_1191_21

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]



 

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    Abstract
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