Editorial Type: CASE REPORTS
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Online Publication Date: 04 Oct 2021

Kawasaki Disease and General Anesthesia for Dental Treatment: A Case Report

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DMD, and
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Article Category: Case Report
Page Range: 146 – 153
DOI: 10.2344/anpr-68-01-06
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Kawasaki disease (KD) is an acute vasculitis of childhood and is the leading cause of acquired heart disease in children in developed countries. Failure to quickly diagnose and treat patients with KD can result in severe cardiac sequelae, especially coronary artery aneurysms (CAAs). Patients with a prior diagnosis of KD who require general anesthesia (GA) may present unique challenges depending on the severity of any cardiovascular sequelae. This case report describes the perioperative management of a 5-year-old male patient previously diagnosed with incomplete KD approximately 1 year before presenting to Stony Brook University Hospital for full mouth dental rehabilitation under GA. Most uniquely, the patient was at high risk for coronary artery thrombosis due to a giant CAA of his right coronary artery and a small CAA of his left anterior descending artery. The discussion also includes the implications of dental treatment under GA for patients with a history of KD.

Keywords: Vasculitis; Coronary artery aneurysms; Dental rehabilitation; Nasal intubation; Anticoagulation; Antiplatelet; Thromboprophylaxis; Thrombosis

Kawasaki disease (KD) is one of the most common vasculitides in children and results in systemic inflammation of medium-sized arteries, particularly the coronary arteries.1,2 KD is more prevalent in boys than in girls (1.5:1) and 85 to 90% of cases are diagnosed in children younger than 5 years old.1,2

The diagnosis of classic KD is made in the setting of an unexplained high fever (often 40°C [104°F] or higher) lasting at least 5 days and the presence of at least 4 out of 5 major signs of mucocutaneous inflammation1–3:

  1. Oral mucous membrane changes, especially red, fissured lips and/or strawberry tongue

  2. Polymorphous rash

  3. Cervical lymphadenopathy

  4. Bilateral, nonexudative bulbar conjunctivitis

  5. Periungual desquamation, erythema of the palms or soles, edema of the hands or feet

Although the acute illness is self-limited, KD can lead to lasting damage to the coronary arteries.2 Coronary artery aneurysms (CAAs) develop in up to 25% of untreated patients.1–3 However, prompt diagnosis and treatment with intravenous immunoglobulin (IVIG) 2 g/kg within the first 10 days of illness reduces the incidence of CAA to 4%.1,3 Patients who develop CAAs may experience long-term cardiovascular sequelae, including persistent CAA, myocardial infarction, congestive heart failure, and death.3,4 Patients with incomplete (also known as atypical) KD present with less than 4 of the major signs of mucocutaneous inflammation of classic KD but share the same risk for developing CAA.1,3

This case report describes the management of a child with incomplete KD and persistent giant and small CAAs who required general anesthesia (GA) for dental treatment.

CASE PRESENTATION

A 5-year-old boy presented to the Dental Care Center at Stony Brook School of Dental Medicine for evaluation of a tooth causing pain in the upper right quadrant. He weighed 35 kg (99th percentile) and stood 126 cm tall, with a body mass index of 25.9 kg/m2. He was diagnosed with incomplete KD approximately 9 months earlier. His daily home medications included aspirin 81 mg and warfarin 2 mg (titrated to a prothrombin time/international normalized ratio of 2.5). Prior to the diagnosis of incomplete KD, he had no other medical conditions, no known drug allergies, and no relevant family or social history.

On visual inspection, gross caries was noted on the right maxillary second molar (tooth #A) with purulent drainage from the associated interradicular gingiva. It was not possible to assess the patient for additional dental treatment needs as the patient was combative throughout the examination and uncooperative for dental radiographs. Due to his behavioral challenges and medical conditions, the patient was scheduled for dental rehabilitation with GA in the operating room (OR).

To better understand the complexity of this patient's cardiac status, we consulted with the patient's cardiologist and hematologist and reviewed the medical reports and images of all cardiac tests conducted since his incomplete KD diagnosis, which was made in October 2018. At that time, the patient presented with a febrile illness including abdominal pain, vomiting, and a positive strep test. Treatment with antibiotics did not resolve his symptoms or fever. Two days later he developed a rash and conjunctivitis, along with continued emesis and lower extremity pain. Due to high suspicion for KD, he was referred for further evaluation, but he had no other signs of mucocutaneous inflammation and no cardiac abnormalities upon ultrasound examination. Therefore, a diagnosis of KD was not made. Eight days later, he developed peeling skin of his distal extremities, red eyes, elevated platelets, and his cardiac ultrasound showed giant CAAs. The diagnosis of incomplete KD was made, and he was admitted for treatment with IVIG, aspirin, and enoxaparin. His acute illness resolved without further complications. Repeated transthoracic echocardiogram over the next several months demonstrated a giant tubular aneurysm of the right coronary artery (RCA) measuring 10 mm in maximal diameter (Z score 18.7) and a smaller aneurysm of the left anterior descending artery (LAD) measuring 4 mm maximal diameter (Z score 4.3) (Figures 1 and 2, respectively; see discussion for explanation of the Z score). Due to the high risk of thrombosis, the patient was treated with antiplatelet (aspirin 162 mg) and anticoagulant (warfarin titrated to a prothrombin time/international normalized ratio [PT-INR] of 2.5) medications.

Figure 1.Figure 1.Figure 1.
Figure 1. Transthoracic echocardiogram image demonstrating patient's giant tubular aneurysm of the right coronary artery (RCA) measuring 10.04 mm maximal diameter; Z score 18.7 (red arrow).

Citation: Anesthesia Progress 68, 3; 10.2344/anpr-68-01-06

Figure 2.Figure 2.Figure 2.
Figure 2. Transthoracic echocardiogram image demonstrating patient's aneurysm of the left anterior descending artery (LAD) measuring 4.29 mm maximal diameter; Z score 4.3 (red arrow).

Citation: Anesthesia Progress 68, 3; 10.2344/anpr-68-01-06

Despite his persistent CAAs, his follow-up transthoracic echocardiogram prior to GA showed a structurally and functionally normal heart (normal ejection fraction, no wall motion abnormalities, no valvular dysfunction, and no evidence of any structural defects). Cardiac magnetic resonance imaging was undertaken due to concern that the aneurysms showed no signs of regression. With the higher resolution image, the RCA aneurysm was noted to be a chain of 3 saccular aneurysms. The largest and most proximal aneurysm measured 11 mm at maximal diameter (Z score 21) and the others tapered with distal progression. The LAD aneurysm measured 3.25 mm at maximal diameter (Z score 2.6). The cardiologist allowed participation in physical education and physical activity but disallowed all contact sports due to the risk of bleeding. The patient was required to have a minder following him at school in case of an accident.

The patient saw his hematologist regularly for management of his aspirin and warfarin. Given the uncertainty of dental treatment needs, the hematologist stopped both aspirin and warfarin and prescribed subcutaneous injections of enoxaparin for bridging anticoagulation. The patient received his final dose of enoxaparin the night before the procedure as planned.

On the day of surgery, the patient arrived at the preoperative holding area accompanied by his parents and 2 older siblings. His physical examination was normal. His baseline vitals by fingertip pulse oximetry included a heart rate of 85 beats per minute (bpm) and oxygen saturation of 99% on room air. All routes of induction, including a traditional intravenous (IV) induction, inhalational induction with sevoflurane, intramuscular (IM) sedation, and oral sedation were considered. Due to frequent injections of enoxaparin, the patient was not averse to subcutaneous/IM injections. In conjunction with the patient's parents, we elected to begin with IM ketamine and dexmedetomidine. With minimal restraint, an admixture of ketamine 105 mg (3 mg/kg) and dexmedetomidine 35 mcg (1 mcg/kg) was administered in the right deltoid. Pulse oximetry was continuously monitored until the patient arrived in the OR. His heart rate peaked immediately after injection at 110 bpm and then stabilized at 90 bpm within 90 seconds of the injection. Four minutes after the IM injection, the patient was separated from his parents with ease and wheeled on a hospital stretcher to the OR. His oxygen saturation was never lower than 99%.

Upon transfer to the operating table, standard American Society of Anesthesiologists monitors were placed in the usual fashion consisting of a 5-lead electrocardiogram, noninvasive blood pressure cuff, pulse oximeter, temperature probe, and capnography. A Bispectral index monitor (BIS Medtronic-Covidien, Dublin, Ireland) was applied to his forehead.

Due to the patient's agitation on transfer from the stretcher to the OR table, 8% sevoflurane in 100% oxygen at 8 L/min was administered via facemask with rapid effect. A 22-gauge IV catheter was placed in the patient's left saphenous vein, and the following agents were administered via IV bolus to optimize conditions for intubation: remifentanil 35 mcg (1 mcg/kg), fentanyl 35 mcg (1 mcg/kg), propofol 80 mg (2.3 mg/kg), and rocuronium 20 mg (0.57 mg/kg), plus dexamethasone 4 mg. Continuous infusions of remifentanil (0.1 mcg/kg/min) and propofol (180 mcg/kg/min) were also started.

A grade 1 Cormack-Lehane view using a GlideScope video laryngoscope (Verathon, Bothell, WA) allowed topicalization of the vocal cords and trachea with 2 mL of 4% lidocaine with a MADgic Laryngo-Tracheal Mucosal Atomizer (Teleflex Medical, Morrisville, NC). The patient was then intubated with a 5.5-mm Rusch Safety Clear Murphy Eye Cuffed oral endotracheal tube (Teleflex, Athlone, Ireland) with the aid of an intubating stylet on the first attempt. Proper position of the endotracheal tube was confirmed by auscultation, equal and bilateral chest rise, and capnography.

After induction, GA was maintained by the following:

  1. The propofol infusion continued at a reduced rate of 80 mcg/kg/min after intubation until discontinuation 4 minutes before extubation.

  2. The remifentanil infusion was titrated down to 0.03 mcg/kg/min after intubation. Approximately 30 minutes prior to extubation, the remifentanil was stopped until the patient resumed spontaneous ventilation, after which it was maintained at 0.05 mcg/kg/min.

  3. After intubation, the patient was maintained on sevoflurane 1.2%. The sevoflurane was discontinued 30 minutes prior to extubation upon resuming spontaneous ventilation.

  4. Nitrous oxide was administered at 67% when the patient resumed spontaneous ventilation.

The procedure consisted of a full mouth series of radiographs, examination under anesthesia, prophylaxis, sealants, fluoride application, and the extraction of tooth #A. The patient received IV clindamycin 350 mg after intubation to prevent the spread of infection after extraction of the abscessed tooth. Immediately prior to extraction, the operating dentist injected 1.0 mL 3% mepivacaine in the adjacent gingival tissues, and the socket was closed with 3-0 plain gut sutures, and hemostasis was achieved with direct gauze pressure after the extraction. Prior to emergence, fentanyl 15 mcg, ondansetron 3.5 mg (0.1 mg/kg), and acetaminophen 540 mg (15 mg/kg) were administered via IV.

During the operation, the patient's vital signs remained stable (Figure 3). The total operative time was 56 minutes. After a brief recovery in the OR, the patient was handed off to the postanesthesia care unit for monitoring. The patient required no additional medications in the postanesthesia care unit and was discharged home after an uneventful recovery with no complications.

Figure 3.Figure 3.Figure 3.
Figure 3. Patient's vitals recorded throughout the procedure in 3-minute increments. See legend on top right corner.

Citation: Anesthesia Progress 68, 3; 10.2344/anpr-68-01-06

DISCUSSION

For patients with KD who are no longer in the acute phase of the illness, the considerations for GA depend almost entirely on the presence and severity of any cardiovascular sequelae.4,5 Preoperative patient evaluation should focus on signs and symptoms of heart failure, dysrhythmias, valvulopathy, myocardial ischemia, extent of CAA, or any other functional impairment of the heart.5

Cardiovascular Sequelae of KD

The architecture of CAA evolves over time and can further dilate, regress, progress to stenotic lesions, or thrombose.6,7 Approximately half of all CAA in KD regress to a normal lumen diameter within the first 2 years of disease onset.6,8 However, the term “regression” does not imply that the coronary vasculature returns to its predisease state.8 Instead, it most often indicates remodeling.8 Myointimal thickening results in a vessel lumen with a normal internal diameter but thickened walls that do not possess typical vasoreactivity.7,8 Although the risk of aneurysm rupture decreases as the vessel walls change, this process commonly leads to stenosis at either the entrance or exit to the aneurysm.7–9 Thus, patients with regressed aneurysms remain at moderate risk for atherosclerotic development.8

CAA scoring utilizes a Z score to quantify the extent of coronary dilation and follow treatment recommendations accordingly (Figure 4).6,8 The Z score refers to the standard deviation from the mean in coronary artery dimensions, adjusted for body surface area.6 The maximum diameter of coronary enlargement as well as the number of coronary arteries involved early in the disease course are correlated with the natural progression of CAA architecture.7 In addition, the maximal diameter of a CAA is inversely proportional to the likelihood that it regresses; that is, giant aneurysms (defined as Z score >10) are least likely to regress.8 If the aneurysm has not regressed after the 2 years of initial onset of KD, it is unlikely to do so.4,6

Figure 4.Figure 4.Figure 4.
Figure 4. §“High-risk” coronary artery aneurysms (CAAs): Other experts may consider additional high-risk features, such as long length and distal location of aneurysms, large total number of CAAs, multiple branches affected, luminal irregularities, vessel wall abnormalities (calcification, luminal thrombosis, previous myocardial infarction, and ventricular dysfunction).10 £Low-dose aspirin: 3–5 mg/kg once daily).

Citation: Anesthesia Progress 68, 3; 10.2344/anpr-68-01-06

Considerations for Thromboembolic Events

Due to blood flow disturbances in the lumen of the aneurysm, patients with persistent CAA are at high risk for thromboembolic events.3,9 Patients with higher risk features may require single or dual antiplatelet therapy in combination with systemic anticoagulation (Figure 4).7,10 The use of newer direct oral anticoagulants in children is considered experimental due to a lack of available data about efficacy and side effects in this population.3,10 Based on his Z score of 21, our patient was at high risk for thrombosis and was prescribed aspirin and warfarin.

Considerations for Anticoagulation Therapy and GA

For patients on warfarin and aspirin undergoing dental rehabilitation with GA, there are 2 important considerations. First, the risk of bleeding from dental procedures must be weighed against the risk of thromboembolic events when these agents are withheld in the perioperative period.11 Second, the anesthesiologist must consider the most appropriate route of intubation.

In children who present for dental rehabilitation with GA, it is often not feasible to obtain radiographs preoperatively. Uncertainty in treatment clouds the perioperative management of patients on anticoagulants and antiplatelet agents. We consulted with the patient's hematologist regarding management of his aspirin and warfarin. Out of an abundance of caution, the hematologist recommended that the patient stop both aspirin and warfarin and use bridging anticoagulation with enoxaparin prior to surgery. As anticipated, the blood loss during this single tooth extraction was minimal and well-controlled with local measures.

Anticoagulation and Extraction

In general, there are 2 groups of medications: antiplatelet agents (eg, aspirin, clopidogrel, ticagrelor) and anticoagulants (eg, warfarin, rivaroxaban, apixaban).11 Each of these medications must be managed individually. Studies show that continuation of antiplatelet drugs in patients undergoing low bleeding risk dental procedures (eg, simple dental extractions) does not lead to an unacceptable bleeding risk.12 A joint science advisory from the American Dental Association, the American Heart Association, the American College of Cardiology, the Society for Cardiovascular Angiography and Interventions, and the American College of Surgeons with representation from the American College of Physicians notes that there is no evidence to recommend the interruption of single or dual antiplatelet agents for dental procedures.13 Similarly, the American Dental Association recommends that it is safe to perform tooth extraction in patients anticoagulated with warfarin with an INR <3.0.14

Data are emerging regarding the newer direct-acting oral anticoagulants including factor Xa inhibitors (eg, rivaroxaban, apixaban, edoxaban) and thrombin inhibitors (eg, dabigatran and argatroban).12 Insufficient evidence exists to definitely declare the safety of continuing these agents during all dental treatment.12 However, several early studies suggest these agents may be safe during low bleeding risk procedures such as simple dental extractions.11,12

The safety of exodontia in patients on antiplatelet or anticoagulant drugs depends in part on the nature of the bleeding. Extraction sockets are readily accessible to the surgeon and respond to local hemostatic measures such as mechanical pressure, hemostatic agents (eg, Gelfoam or Surgicel), suturing, or topical tranexamic acid.14 Therefore, the bleeding that does occur remains controllable and does not threaten patient safety.

Nasal Versus Oral Intubation

Traditionally, patients undergoing dental rehabilitation in the OR setting are intubated nasally to optimize the operator's surgical field. However, the delicate mucosa of the nasal passage and nasopharynx can bleed from the trauma of nasal intubation, even in the hands of experienced clinicians utilizing meticulous technique.15 This bleeding may occur during intubation,15 extubation,15 or in recovery.16 A bleed from the anterior nasal passage may be compressible, whereas a bleed from the posterior nasal cavity, choana, or nasopharynx is difficult to access and control.15 Blood in the airway reduces visibility during laryngoscopy and increases the risks of airway obstruction and aspiration.15,17 Uncontrolled bleeding in the airway can become an airway emergency or cause respiratory compromise.15 In addition, brisk bleeding can result in hemodynamic deterioration, especially in a patient with pre-existing cardiovascular pathology.

Currently, there are minimal data addressing the implications of nasal intubations for patients on anticoagulation therapy. Many consider therapeutic anticoagulation to be a relative contraindication to nasal intubation.18 Due to the recognized bleeding risk with nasal intubation in patients with platelet or coagulation impairments, it is our practice to avoid the nasal route of intubation when alternatives exist.

There are a number of studies demonstrating that bacteremia can result from nasotracheal intubations.19 Bacteremia occurs when normal nasopharyngeal flora enters the bloodstream as a result of mucosal trauma.19 Bacteremia can develop even in an “atraumatic” nasotracheal intubation.19 Patients with acquired or congenital heart disease, such as some patients with a history of KD, may be predisposed to complications like bacterial endocarditis and brain abscess following bacteremia.19,20 This has important implications for the timing of antibiotic administration.

Official American Heart Association guidelines suggest that both oral and IV antibiotics should be administered 30 to 60 minutes prior to the procedure, or, failing that, up to 2 hours afterwards.21 When a patient requires antibiotic prophylaxis and a nasal intubation, it may not be feasible to administer oral antibiotics or to wait 30 minutes between IV antibiotic administration and intubation. In these cases, the anesthesiologist must decide on the best timing and route of administration of prophylactic antibiotics prior to nasal intubation.

Hemodynamic Control

Given the patient's giant CAA, our anesthetic goals centered around hemodynamic control. We especially wanted to avoid tachycardia (increased myocardial oxygen demand and reduced oxygen supply) and hypertension (increased aneurysm wall tension) to reduce the risks of myocardial ischemia, aneurysm rupture, or other complications. We made the following decisions as a direct result of this patient's status:

  1. During a pretreatment meeting, the entire surgical and OR staff was made aware of the patient's medical condition, including the potential risks of the procedure.

  2. We achieved preoperative sedation in the presurgical area with an IM admixture of ketamine and dexmedetomidine. In our experience, dexmedetomidine effectively attenuates the hypertension and tachycardia that commonly results from IM ketamine. We believed an IM injection would give us greater hemodynamic control in comparison with a traditional mask induction. In addition, given the patient's routine experience with enoxaparin injections, the patient's parents felt he would be most accepting of an IM injection.

  3. We utilized several techniques to minimize tachycardia and hypertension throughout the procedure, especially during intubation, maintenance, extraction, and extubation.

    1. Intubation: The patient was managed with remifentanil, fentanyl, and propofol to blunt the autonomic response to intubation and airway reflexes. In addition, the trachea was anesthetized with atomized lidocaine.

    2. Maintenance: We achieved adequate “depth of anesthesia” using the guidance of a BIS monitor titrated to a value of 40.

    3. Extraction: Effective local anesthesia was achieved with 3% mepivacaine without epinephrine.

    4. Extubation: We conducted a narcotized wake up utilizing remifentanil.

  4. We administered multiple agents to reduce postoperative pain, including dexmedetomidine (IM), ketamine (IM), dexamethasone (IV), acetaminophen (IV), fentanyl (IV), and mepivacaine (infiltration).

As can be seen in Figure 3, the patient exhibited minimal changes in his hemodynamic status during intubation, extubation, and throughout the procedure.

KD and COVID-19

The exact etiology of KD remains unknown.2 Epidemiologic data suggest that it may be a reactive immunological process triggered by infectious agents in genetically predisposed individuals.1,22 There are numerous news stories of children showing symptoms similar to KD who have either tested positive or shown to be previously infected with SARS-CoV-2, the novel coronavirus that causes COVID-19.23–25 On May 14, 2020, the Centers for Disease Control and Prevention issued a national health advisory reporting cases of previously healthy children meeting the criteria for multisystem inflammatory syndrome in children (MIS-C).25 MIS-C presents as a severe inflammatory syndrome with KD-like-features in children who tested positive for an active or recent infection of SARS-CoV-2.25 MIS-C has a range of clinical presentations, but in published case series, many of the pediatric patients have had fever and mucocutaneous involvement like KD.26 In comparison with KD, MIS-C has been suspected to affect adolescents and children older than 5 years of age and has more frequent cardiovascular involvement.26 The evidence of a possible connection between these diseases warrants further monitoring.25

Finally, patients with KD are often treated with IVIG. IVIG may impair the immune response to live virus vaccines.27 The immune response to the measles vaccine, for example, may be ineffective when administered up to 8 months after IVIG administration.27 The impact of IVIG on all vaccinations is not known.27 Therefore, depending on the timing of dental treatment in relation to IVIG administration, patients with KD may not have full immunity to all vaccinations according to the recommended schedule, including SARS-CoV-2 when a vaccine becomes available. Anesthesia providers should be aware of the potentially unique immunological status of patients with KD.

CONCLUSION

This case report outlines the successful anesthetic care of a 5-year-old male with incomplete KD and a giant CAA of the RCA and a small CAA of the LAD. Because KD is the leading cause of acquired heart disease among children younger than 5 years old in developed countries, anesthesiologists are likely to encounter children with a history of KD who require GA for dental rehabilitation. Every provider should understand the pathophysiology of KD and the unique considerations for GA. In addition, the anesthesia plan should be formulated after consultation with the patient's cardiologist, hematologist, and other specialists.

Copyright: © 2021 by the American Dental Society of Anesthesiology 2021
Figure 1.
Figure 1.

Transthoracic echocardiogram image demonstrating patient's giant tubular aneurysm of the right coronary artery (RCA) measuring 10.04 mm maximal diameter; Z score 18.7 (red arrow).

Figure 2.
Figure 2.

Transthoracic echocardiogram image demonstrating patient's aneurysm of the left anterior descending artery (LAD) measuring 4.29 mm maximal diameter; Z score 4.3 (red arrow).

Figure 3.
Figure 3.

Patient's vitals recorded throughout the procedure in 3-minute increments. See legend on top right corner.

Figure 4.
Figure 4.

§“High-risk” coronary artery aneurysms (CAAs): Other experts may consider additional high-risk features, such as long length and distal location of aneurysms, large total number of CAAs, multiple branches affected, luminal irregularities, vessel wall abnormalities (calcification, luminal thrombosis, previous myocardial infarction, and ventricular dysfunction).10 £Low-dose aspirin: 3–5 mg/kg once daily).

Contributor Notes

Address correspondence to Dr Ralph H. Epstein, Department of Oral and Maxillofacial Surgery, Division of Dental Anesthesiology, 148B Rockland Hall, Stony Brook, NY 11794-8711; ralph.epstein@stonybrookmedicine.edu.
Received: 14 Jul 2020
Accepted: 15 Nov 2020
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