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Learning Modules ❯ Gastrointestinal

Medium Vessel Disease:Kawasaki Disease

Kawasaki disease is a medium vessel vasculitis associated with mucocutaneous lymph node syndrome, and it frequently occurs in infants and children younger than 5 years. Although exact pathogenesis is unknown, it seems likely that infection or autoimmunity play a critical role.
Coronary artery involvement is common in patients with KD, therefore myocardial ischemia or infarction frequently occur. CT findings include coronary artery aneurysm, ectasia, premature atherosclerosis and stenosis. A typical braid-like appearance of coronary arteries is one of the characteristics of KD

Kawasaki Disease: Facts

Occurs in children younger than 5 yrs with peak under age 2 yrs
Male > female by 1.5x
More common in Asians especially Japanese
Acute febrile systemic illness in children
AKA mucocutaneous lymph node syndrome

Kawasaki Disease: Facts

Fever for 5 days or more without a cause and four of the following

Bilateral conjunctival injection
Mucous membrane changes (strawberry tongue)
Extremity abnormalities including erythema of palms or soles, edema of hands or feet
Rash
Cervical lymphadenopathy

Kawasaki Disease: Facts

Cardiac abnormalities

Pericardial effusion (30%)
Myocarditis
Mitral regurgitation (30%)
Aortitis and aortic regurgitation
Congestive heart failure
Coronary artery aneurysms

Kawasaki Disease: Facts

Coronary artery aneurysms occur in up to 25% of patients treated with aspirin alone or no treatment
Aneurysms develop within 2 weeks (1-4 weeks) with a 2% mortality rate
Aneurysms are more common in the proximal than distal coronary artery
Aneurysms may thrombosis which results in sudden death
Vascular complication rate decreased with proper therapy (aspirin and intravenous immune globulin) to around 5% for aneurysms

8 mm Aneurysm of the Left Main Coronary Artery

11 year old male with a history of Kawasaki’s disease at 8 months of age. The patient complained of episodic mid sternal chest pain occasionally.
8 mm Aneurysm of the Left Main Coronary Artery

Kawasaki Disease with Giant Coronary Artery Aneurysms

12 year old male with a hx of Kawasaki disease being treated with coumadin and aspirin. CTA was requested to define the status of the coronary arteries. CTA demonstrated multiple RCA aneurysms measuring up to 9mm in size. Portions of the RCA wall have begun to calcify
Kawasaki Disease with Giant Coronary Artery Aneurysms

Coronary Artery Aneurysms: Etiology

Kawasaki’s disease
Atherosclerosis
Takayasu’s vasculitis
Infection
Congenital
Trauma (including post-surgical)
Polyarteritis nodosa
Loeys Dietz Syndrome
May also be associated with cocaine use, SLE, syphilis and rheumatic fever

Coronary Artery Aneurysm in Kawasaki’s Disease

Kawasaki’s with Multiple Coronary Artery Aneurysms

Medium Vessel Vasculitis: Polyarteritis Nodosa

Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium and small arteries without glomerulonephritis, and is unassociated with the anti-neutrophil cytoplasmic antibody (ANCA)
Polyarteritis nodosa affects men twice as often as women in the 5–7th decades. Etiology is unclear, but the hepatitis B virus may play an important role in the development of the disease. Renal involvement is common with an incidence of 70–80%, resulting in proteinuria, hematuria, and hypertension, but not glomerulonephritis.

Polyarteritis Nodosa: Facts

Systemic illness characterized by necrotizing inflammation of small and medium-sized arteries
Most frequently occurs in middle aged men
Most common clinical symptoms are persistent fever, weight loss & polyarthralgia's
Hallmark: aneurysm formation of small and medium sized arteries
Renal involvement is characteristic
- infarcts, microaneurysms, indistinct corticomedullary differentiation

PAN with Multiple Aneurysms in Kidney, Mesenteric Vessels