(MIS-C)

acronym for Pediatric Multisystem Inflammatory Syndrome

Rare: 2 to 6 % of children infected with COVID 19. The African ethnicity or African ancestry could be a risk factor. This multisystemic inflammatory syndrome similar to Kawasaki syndrome occurring in children infected or recently infected with SARS-CoV-2 (COVID 19) has been described in 2020. Sometimes considered as a form of TAMOF (Thrombocytopenia-Associated Multiple Organ Failure).

WHO definition: children under 19 years of age with fever for 3 days or more AND at least two of the following clinical criteria:

-        skin rash,

-        hypotension or shock due to acute myocardial involvement or vasodilation in case of hyperinflammatory syndrome

-        signs of myocardial dysfunction, pericarditis, valve dysfunction or coronary involvement (echocardiography, troponine or proBNP); rhythm disorders: ST segment abnormalities, prolonged QTc, atrial or ventricular extrasystoles

-        signs of coagulopathy: high D-dimers, thrombopenia

-        acute gastrointestinal disorders: diarrhea, vomiting, abdominal pain

AND

-        elevated blood markers of inflammation: CRP, procalcitonin, VS

AND

-        no microbial cause of sepsis or inflammation

AND

-        evidence of COVID 19 infection: positive PCR test, or positive IgG serology or probable contact with a COVID 19 positive patient.

The time interval between COVID 19 infection and the onset of the disease varies (a few days to a few weeks), suggesting a post-infectious immune origin.

A dilation of the coronary arteries with or without aneurysms, is observed in 6 to 24 % of cases. Intensive management with inotropes, corticosteroids, immunoglobulins IV, aspirin or other anticoagulation, ECMO is sometimes necessary. The prognosis is generally good with few sequelae.

Differential diagnosis: Kawasaki disease (vascularitis of the middle-size arteries more common in children under 5 years of age) (see this term) especially its form associated to shock.

Anesthetic implications:

echocardiography: myocardial dysfunction ? coronary aneurysms ?; corticosteroid therapy; careful fluid management, avoid systemic hypotension.

References :  

-        Riphagen S, Gomez J, Gonzalez-Martinez  C, Wilkinson N, Theocharis P.
Hyperinflammatory shock in children during COVID-19 pandemic.
The Lancet 2020; 395:1607-8

-        Verdoni L, Mazza A, Gervasoni A, Martelli L, Ruggeri M, Ciuffreda M, Bonanomi E, D’Antiga L.
An outbreak of severe Kawasaki-like disease at the Italian epicentre of the SARS-CoV-2 epidemic: an observational cohort study.
The Lancet doi.org/10.1016/S0140-6736(20)31103-X (May 13, 2020)

-        Sperotto F, Friedman KG, Son MBF, VanderPluym CJ, Newburger JN , Dionne A.
Cardiac manifestations in SARS-CoV-2-associated multisystem inflammatory syndrome in children : a comprehensive review and proposed clinical approach.
Eur J Pediatr 2021; 180: 307-22.

-        Puri S, Bandyopadhyay A, Ashok V.
Multisystem inflammatory syndrome in children : what is in it for the pediatric anesthesiologist ?
Pediatr Anesth 2021 ; 31 :736-7

Updated: June 2021