Idiopathic thrombocytopenic purpura (ITP) of childhood is characterized by increased autoimmune platelet destruction and inhibition of platelet production. With platelet counts <10 × 109 L−1, the risk of serious bleeding is still considered very low.1 Although guidelines in the management of ITP recommend a platelet count not less than 30 × 109 L−1 for dental extractions,2 this is based on consensus expert opinion only. Previous case reports describing management of children with chronic ITP have adhered to these guidelines commonly using either corticosteroids or platelet transfusions to obtain an “acceptable” threshold before dental extractions.3,4
The following case report describes the perioperative management of a child with chronic ITP undergoing dental treatment without using therapies to increase platelet counts above 30 × 109 L−1. With very few reported cases, the pediatric anesthesiologist and dentist should be made aware of strategies describing how to safely manage childhood ITP without prophylactic platelet transfusions, corticosteroids, or IV immunoglobulin (IVIg), which based on current evidence are not supported. Written consent for publication was obtained from the patient’s legal guardian.
A 13-year-old premenarche female presented for perioperative planning before planned dental extraction for an over-retained deciduous molar causing mucosal bleeding during brushing. She had recently been diagnosed with chronic ITP, and additional symptoms included spontaneous epistaxis. None were severe enough to require hospitalization. She did, however, have iron deficiency anemia and was receiving iron supplements. During her workup for ITP, she had received IVIg and corticosteroids as trial therapy with minimal effect on increasing her platelet count.
Examination revealed a dense petechial rash across her face, arms, body, and legs. Her platelet count using both EDTA and citrate samples were 4 × 109 L−1 (normal: 150–400 × 109 L−1). Her hemoglobin was 107 g·L−1 with a mean corpuscular volume of 79 fL (normal: 80–100 fL). Coagulation studies were normal with an international normalized ratio of 1.0 and an activated partial thromboplastin time of 28.5 seconds. Fibrinogen was 3.4 g·L−1 (normal: 1.5–4.0 g·L−1).
A multidisciplinary meeting including specialists from pediatrics, hematology, anesthesiology, and dentistry agreed to adopt a “watchful waiting” approach without using therapies to prophylactically increase her platelet count before surgery. Tranexamic acid however was commenced orally 2 hours before surgery and continued for 5 days (500 g 3 times daily) in total. Local control would be assisted by the use of gelatine dental sponges soaked in tranexamic acid. Control of her arterial blood pressure would be facilitated with general anesthesia. If hemostasis could not be achieved by these measures, platelets, IVIg, corticosteroids, and desmopressin were available for access during the perioperative period.
On the day of surgery, the patient received general anesthesia with the airway secured using a flexible laryngeal mask airway to minimize airway trauma. Her deciduous molar tooth was extracted along with a generalized scaling and cleaning. Hemostasis was obtained by local control. Recovery in the postanesthesia care unit was uneventful, and the patient was admitted overnight for an additional 24 hours of observation. She was discharged the next day, and there were no complications at the 5-day follow-up.
ITP is the most common cause of thrombocytopenia in children.1 If surgery is required, expert opinion advocates thresholds for platelet counts before proceeding with extractions and regional dental blocks ≥30 × 109 L−1; minor surgery ≥50 × 109 L−1; major surgery ≥80 × 109 L−1.2 These “critical” thresholds are not evidence based. Interestingly, the classification of severity of childhood ITP is based clinically and not just on platelet counts. These classifications confirm that the majority of children do not have serious bleeding despite severe thrombocytopenia.5 The result of this evidence is that these same experts recommend an expectant watch and wait policy for the treatment of ITP.2 Our patient clinically presented with self-limiting bleeding episodes indicating that her blood was still able to clot despite a very low platelet count. We therefore adopted this watch and wait strategy for her surgery.
Additional justification for this strategy was that trying to achieve platelet thresholds with prophylactic therapies based on consensus opinions are not without risk. Platelet transfusions are associated with an increased risk of multiorgan failure, acute lung injury, and bacterial sepsis,6 and IVIg can be associated with neutropenia.7 More recently, it has been documented that laparoscopic splenectomies were performed safely in patients with platelet counts <10 × 109 L−1.8 Therefore, when the type of surgery permits a period of time for hemostasis to be achieved without the risk of exsanguination (watchful waiting approach), the balance of risk versus benefit may favor withholding these therapies.
For this case, while we did not use such therapies to increase the platelet count, we did use antifibrinolytics prophylactically to increase preexisting platelet function. The rationale behind this decision are as follows: (1) antifibrinolytic agents such as aminocaproic acid and tranexamic acid have been used in ITP patients to control chronic bleeding;9 (2) its application to prevent perioperative bleeding in cardiac and orthopedic surgery;10 and (3) meta-analysis on the use of tranexamic acid prophylactically has not been associated with an increased risk of thrombosis.10
We used oral instead of IV tranexamic acid because of its simplicity and similar blood-sparing efficacy.11 Although this efficacy has yet to be evaluated in ITP patients, the risk versus benefit profile may favor its use in assisting surgical hemostasis without causing significant harm. As there are currently no dose-finding studies on the optimal use of tranexamic acid in ITP patients, we based our dosage regime on its use in menorrhagia. It is also available as an oral solution and has been used as mouthwash in our institution to prevent bleeding in patients receiving oral anticoagulant therapy undergoing dental treatment. We used a variant of this technique by applying tranexamic acid-soaked gelatine dental sponges directly onto the disrupted periodontal tissue.
A single intraoperative dose of desmopressin (0.3 μg·kg−1) is another therapy that has been used previously in ITP patients to reduce mucosal bleeding.12 It increases plasma levels of von Willebrand factor and factor VIII. We considered desmopressin as our first-line rescue therapy in the event that bleeding became difficult to control during our watching waiting period. Our “plan B” would then be platelet transfusions with IVIg to minimize donor destruction by the recipient.
In conclusion, this case highlights the management of dental extraction in a child with chronic ITP and platelet counts below conventional thresholds. To achieve this, we recommend: (1) assessing the patient’s clinical severity and not just platelet count; (2) multidisciplinary approach with a pediatric anesthesiologist, dentist, hematologist, and pediatrician; (3) perioperative tranexamic acid to maximize platelet function; and (4) platelet transfusion, IVIg, steroids, and desmopressin only if hemostasis cannot be achieved by local measures during the watchful waiting period. There is currently no evidence for these therapies prophylactically before surgery. E
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