Case Presentation By Eman El Sharkawy Ass. Professor of cardiology Alexandria University 6m old baby girl Past history : - At the age of 2m attack of fever, diarrhea, mouth ulcers, difficult breast feeding - This attack lasted for 2ws and was diagnosed as severe gastroenteritis, - hospitalized due to dehydration - Received antibiotic and IV fluids. 1
The mother said that her girl was severely distressed with rapid breathing rate No cyanosis, no convulsions After this attack she didn t have normal breast feeding ( distressed after 10 min), disturbed sleep pattern, always crying Her mother noticed visible pulsations in her neck No recurrence of fever. she asked medical advice 2
Examination: HR=110 BPM Weight =5kg, average height. Increased cardiac size, systolic murmer allover the pericordium. Chest : scattered crepitation. Abdomen, CNS : normal Echocardiography report : MILD LV Global hypokinesia EF=50 % Mild-Moderate mitral regurgitation No pericardial effusion No congenital cardiac anomalies X-ray chest: cardiomegaly -wide mediastinum 3
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She was sent for MDCT of the chest 5
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After 4 month She had an attack of convulsions, with no residual neurological deficits. 128-MDCT brain was done and revealed: ( cortical and subcortical hypodensity in right fronto-parital area with effacement) = non-haemorrhagic infarction in right middle cerebral artery territory She was followed up in pediatric cardiology department till the age of 3ys then they lost contact with her family Summery History of fever-diarrhea-mouth ulcers LV wall motion abnormalities (MI) Multiple vascular aneurysms: iliacs, subclavian, Small renal arteries Ectatic coronary arteries (aneurysms ) LAD huge thrombosed aneurysm. Middle cerebral artery infarction WHAT IS THE POSSIBLE DIAGNOSIS???? 18
New General Classification Of Childhood Vasculitis 1 Predominantly large vessel vasculitis Takayasu arteritis 2 Predominantly medium-sized vessel vasculitis Childhood poly arteritis nodosa Cutaneous poly arteritis Kawasaki disease NEW GENERAL CLASSIFICATION OF CHILDHOOD VASCULITIS 3. Predominantly small vessel vasculitis (A) Granulomatous Wegener granulomatosis Churg-Strauss syndrome (B) Non Granulomatous Microscopic polyangiitis HSP Isolated cutaneous leukocytoclastic vasculitis Hypocomplementemic urticarial vasculitis 19
Kawasaki disease Kawasaki Disease: Mucocutaneous Lymph Node Syndrome A self-limited vasculitis of unknown etiology that predominantly affects children younger than 5 years. It is now the most common cause of acquired heart disease in children in the United States and Japan. Jane Burns, MD* Burns, J. Adv. Pediatr. 48:157. 2001. 20
What is Kawasaki Disease? Idiopathic multisystem disease characterized by vasculitis of small & medium blood vessels, including coronary arteries Etiology Infectious agent most likely Age-restricted susceptible population Seasonal variation Well-defined epidemics Acute self-limited illness similar to known infections No causative agent identified Bacterial, retroviral, superantigenic bacterial toxin Immunologic response triggered by one of several microbial agents 21
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 Modified from Centers for Disease Control. Kawasaki Disease. MMWR 29:61-63, 1980 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 Convalescent (Months to years later) 22
Kawasaki disease - AHA diagnostic criteria Fever of 5 days duration + four of five criteria 1. 2. Oropharyngeal changes (90%+ of cases) Changes in peripheral extremities (90%+ of cases) 3. 4. Bilateral non-purulent conjunctival injection (90%+ of cases) Polymorphous rash (95%+ of cases) 5. Cervical lymphadenopathy (~75% of cases) Trager, J. D. N Engl J Med 333(21): 1391. 1995. 23
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Trager, J. D. N Engl J Med 333(21): 1391. 1995. Coronary Arterial Changes 15% to 25 % of untreated patients develop coronary artery changes 3-7% if treated in first 10 days of fever with IVIG Most commonly proximal, can be distal Left main > LAD > RCA 25
Coronary Aneurysms Size Small = <5 mm diameter Medium = 5-8 mm Giant = 8 mm Highest risk for sequelae Shape Saccular Fusiform Coronary Aneurysm History 50 % regress to normal by echo 25 % become smaller 25 % do not regress 7-20 % develop stenosis or myocardial infarction attributed to their aneurysms 26
Coronary Aneurysm History Approximately 50% of aneurysms resolve Smaller size Fusiform morphology Female gender Age less than 1 year Giant aneurysms (>8mm) worst prognosis Unfortunately Our patient had Giant LAD aneurysm anterior MI Multiple peripheral aneurysms Cerebral infarction?? Other undiagnosed complications Bad Prognosis 27
Thank you Dr. Eman El Sharkawy 28