Kawasaki Disease

Self-limited vasculitic syndrome of unknown etiology characterized by fever, cervical adenopathies, and cardiac involvement.

(Figure)

(Figure)

Mucocutaneous Lymph Node Syndrome.

Asian people are at greater risk: 5000-6000 new cases per year occur in Japan versus 3000 in the United States. Males are affected 1.5 times more often than females. Kawasaki disease (KD) is currently the leading cause of acquired heart disease in the United States among children younger than 5 years. Mortality from coronary artery abnormalities, including coronary aneurysms, is reported in 20 to 25% of untreated individuals.

Etiology of KD is unknown. Several possibilities are considered, including an infectious etiology (retrovirus?), an immunologic abnormality, and even a toxic cause (carpet shampoo). Clinical and epidemiologic data support an infectious etiology, but many authors think that an autoimmune component or genetic predisposition also exists.

Necrotizing vasculitis of medium-size muscular arteries, including coronary and cerebral vessels. Arteries show focal segmental destruction, with subsequent ectasia and formation of aneurysms in 15 to 25% of children. Several infectious causes of KD have been theorized and include Ebstein-Barr virus, retroviruses, Streptococcus pyogenes and viridans, Staphylococcus, Chlamydia, Propionibacterium, and Pseudomonas species. However, conventional bacterial and viral cultures and serologic studies have not confirmed an infectious cause. Other postulated etiologic agents are immunizations, medications, and environmental agents, such as exposure to rug shampooing agents or house dust mites. The finding of a skewed T-cell receptor response in the myocardium and the coronary arteries led to a search for superantigens, such as toxic shock syndrome toxin-1 produced by Staphylococcus aureus. Immunohistochemical findings lend support to the hypothesis of a proinflammatory cell-mediated immune reaction possibly triggered by a superantigen or a conventional antigen.

Clinical diagnosis. No diagnostic laboratory tests are available. The Centers for Disease Control and Prevention in Atlanta (USA) defines a case of KD as illness in a patient with fever of 5 or more days' duration (or fever until the of administration of intravenous immunoglobulin, if this is given before the fifth day of fever), and the presence of at least 4 of the following 5 clinical signs:

• Rash
• Cervical lymphadenopathy (at least one lymph node ≥ 1.5 cm in diameter)
• Bilateral conjunctival injection
• Oral mucosal changes (injected or fissured lips, injected pharynx, “strawberry” tongue)
• Peripheral extremity changes (erythema of palms or soles, edema of the hands or feet, or generalized or periungual desquamation).

Patients whose illness does not meet the above KD case definition but who have fever and coronary artery abnormalities are classified as having atypical or incomplete KD.

Occurs primarily in children within the first years of life. Main clinical signs are fever, conjunctivitis, inflammation of the mucous membranes and tongue (strawberry tongue), swollen erythematous hands and feet, truncal rashes, and diffuse lymphadenopathy. Cardiac complications include pericarditis, myocarditis, myocardial infarction, or ischemia as a result of coronary artery thrombosis or stenosis, and valvular dysfunction. Pneumonitis and pleural effusions can occur. Cerebral hemorrhage caused by aneurysm rupture has been reported. Other CNS complications are cranial nerve palsies, aseptic meningitis, and subdural effusions. Aneurysmal rupture of any affected vessel present with hemorrhage. Stenosis of vessels can cause hypoperfusion of distal extremities or other organs. Hepatic involvement occurs in less than 10% of cases, and renal involvement occurs in less than 1%. Anemia may be present. Management consists of γ-globulin and aspirin. Corticosteroids, pentoxifylline (a methyl-xanthine compound that inhibits TNF-α messenger RNA-transcription), ulinastatin (a human trypsin inhibitor that inhibits neutrophil elastase), abciximab (a platelet glycoprotein IIb/IIIa receptor antagonist), monoclonal antibodies, and cytotoxic agents (cyclophosphamide) have all been used with variable success in these patients.

No elective procedures during the course of the disease. Rule out cardiac involvement prior to anesthesia: history and physical examination, electrocardiogram (dysrhythmias), echocardiogram to detect myocardial or valvular dysfunction (usually mitral or tricuspid regurgitation), and even coronary angiogram to confirm myocardial ischemia caused by coronary artery aneurysms or stenosis. Cardiac medications should be continued throughout the perioperative period. Radiography or CT scan of the chest to rule out pulmonary involvement. Any neurologic deficits should be documented prior to anesthesia. If cerebral hemorrhage has occurred, check for signs of raised intracranial pressure. Complete blood count to check for anemia; electrolyte levels to rule out abnormalities that may occur with some cardiac medications (diuretics, angiotensin-converting enzyme inhibitors) and renal dysfunction.

Maintain optimum balance of myocardial oxygen supply to demand ratio if myocardial perfusion is compromised. Five-lead ECG monitoring (leads II and V5) for signs of myocardial ischemia is mandatory. Avoid myocardial depressants if cardiac function is depressed. Have vasoactive and antidysrhythmic medications readily available to manage complications. Avoid hemodynamic instability at any stage of anesthesia to minimize the risk of aneurysm rupture. Maintenance of normal cerebral perfusion pressure in the presence of cerebral hemorrhage with raised intracranial pressure. Use of invasive arterial and central venous monitoring may be useful. Platelet dysfunction as a consequence of treatment with aspirin may predispose to increased blood loss. Careful laryngoscopy should be performed to avoid injury to inflamed lips, tongue, and oropharynx. Patients should be monitored for myocardial ischemia postoperatively.

Avoid cardiodepressive drugs. Steroid supplementation if necessary. Some patients may be on anti-platelet drugs, heparin, or oral anticoagulation therapy. Check before (regional) anesthesia.

Other vasculitic diseases of children, particularly the following:

Systemic Lupus Erythematosus (SLE): Characterized by unpredictable exacerbations and remissions of inflammatory multisystemic disorder of connective tissue. The circulation of immune complexes and activation of complement leads to involvement mainly of the skin, joints, kidneys, serosal membranes, lungs, gastrointestinal tract, and heart.

Chauffard Syndrome: Chronic inflammatory disease of variable severity that involves the joints and potentially also the connective tissues and viscera.

Polyarteritis Nodosa (PAN): Severe condition very similar to Kawasaki disease with unexplained fever, major alteration of physical status, and histologic lesions of arteritis nodosa (often discovered on postmortem examinations only).