1. A case of Kawasaki
disease with severe CNS
complications
Professor Dr. Ali A. Hadi Al-Saady
2. What is Kawasaki Disease?
• Idiopathic multisystem disease
characterized by vasculitis of small &
medium blood vessels, including
coronary arteries.
• The most common acquired pediatric
heart disease all over the world.
3. KD epidemiology
• World wide distribution
• Male preponderance
• Commoner in oriental children
• Some seasonality and occasional epidemics
• The incidence of KD is rising world-wide,
including the UK.
• Incidence in the UK is 8.1 /100 000 children
aged less than 5 years old
4. Cause of Kawasaki disease
• Multifactorial ( genetic + Environmental )
• Genetic is polygenetic one ( many genes )
• Environmental ( may be viral infection , but
not known yet )
5. Diagnostic Criteria
Fever for at least 5 days
At least 4 of the following 5 features:
1. Changes in the extremities
Edema, erythema, desquamation
2. Polymorphous exanthem, usually truncal
3. Conjunctival injection
4. Erythema&/or fissuring of lips and oral cavity
5. Cervical lymphadenopathy
Illness not explained by other known disease
process
6.
7.
8. Phases of Disease
• Acute (1-2 weeks from onset)
• Febrile, irritable, toxic appearing
• Oral changes, rash, edema/erythema of
feet
• Subacute (2-8 weeks from onset)
• Desquamation, may have persistent
arthritis or arthralgias
• Gradual improvement even without
treatment
• Coronary aneurysm occurs here.
• Convalescent (Months to years later)
9.
10. Case report
• Gian Amjad , a 3 years old male.
• Consulting cardiac clinic on 28 /5/2022.
• Main complain was with fever for 4 days prior to
admission then he developed recurrent fit at hospital,
associated with DLO.
• after 5 days admission he start to has skin rash at the
napkin area with swelling of hands and feet and his lips
cracked with strawberry tongue and cervical LAP with
conjunctivitis his fever continued.
• He was suspected as a case of kawasaki disease based on
the diagnostic criteria.
13. Treatment
•He received 2 doses of IVIG , 2 days
apart
•Aspirin as a high dose , which was
reduced to 5 mg/kg after 48 hrs from
fever cessation.
•Luminal for seizure.
14. Progress
•Seizure cessation , with
improvement in level of
consciousness.
•Improvement in coronary artery
aneurysms / dilatation.
15.
16. After 2 weeks of treatment
Coronary arteries
size return back
to mean SD for
B.S.A.
17. CNS complications of Kawasaki disease
• Very diverse.
• Incidence is 1-30 %
• Includes : irritability , headache , seizures ,
meningeal irritations signs, hemiplegia , facial
nerve palsy.
• Subdural hematoma is a very rare complication,
may be not even reported in the literature.
• They need prompt recognition & early start of
IVIG.