Possible life-threatening multifactorial syndrome
resulting from a previous viral infection treated by aspirin and a genetic
sensibility. Dehydration, intracranial hypertension, and liver insufficiency
must be considered. Presentation includes seizures, vomiting, lethargy,
irrational behavior, and delirium. Tachypnea, jaundice, fever, and coma
suggest end-stage of disease. Reye Syndrome appears usually 3 to 5 days
after the onset of chickenpox rash.
Fatty Liver with Encephalopathy; Reye-Johnson Syndrome.
Rare (1:1,000,000 in the general population); incidence
has decreased since 1993.
There is no known genetic association with
Reye Syndrome is a two-phase illness because it is
almost always associated with a previous viral infection, such as influenza,
cold, or chickenpox. It tends to appear with greatest frequency during
January, February, and March. It has been proposed that Reye syndrome
develops from the interaction of a viral illness, genetic susceptibility to
the disease, and exposure to chemicals, such as salicylates, pesticides, and
aflatoxin. Abnormal accumulations of fat begin to develop in the liver and
other organs of the body, which can cause a severe increase of intracranial pressure.
Unless diagnosed and treated successfully, death is common, often
within a few days.
Syndrome occurs in children and teens (96%).
Diagnosis is made by clinical signs (persistent or recurrent vomiting,
listlessness, personality changes present during or soon after a viral
illness) and by elimination of other diagnoses such as metabolic illness,
meningitis, encephalitis, diabetes, drug overdose, sudden infant death,
toxic ingestion, head trauma, renal or hepatic failure poisoning, that could
imitate Reye Syndrome.
Persistent or continuous vomiting; signs of brain
dysfunction; listlessness, loss of pep and energy; drowsiness can be
observed first and then irritability, aggressive behavior, disorientation,
confusion, irrational behavior delirium, convulsions, coma. Beyond these
neurological signs, it is possible to observe respiratory disturbances such
as hyperventilation. Anemic episodes and hypoglycemia are common;
Evaluate liver function (echography,
CT, laboratory tests: coagulation, serum glutamic-oxaloacetic transferase
[SGOT] and serum glutamic-pyruvic transaminase [SGPT] [increase generally],
albumin); respiratory function (clinical, chest radiograph, arterial blood
gas analysis); hydration status of the patient (clinical, electrolytes
values, urea, creatinine); neurological function (clinical, CT).
Indication of anesthesia during the
evolution of a Reye Syndrome has to be exceptional. Steady state should be
raised before starting. Careful monitoring of respiratory function should be
done (SpO2 [arterial oxyhemoglobin saturation], etCO2 [end-tidal
carbon dioxide concentration], arterial blood gas analysis) in case of
respiratory disturbance, since normoor mild hypocapnia are important,
considering the risk of high intracranial pressure. Fluid regimen also needs
to be adapted to the existence of dehydration and of high intracranial
pressure. It is necessary to measure regularly blood levels for hypoglycemia and
Avoid succinylcholine because of the possibility of
severe raise in intracranial pressure. Avoid drugs with known hepatotoxic effect and
metabolism because of common cytolytic hepatitis. Choice of anesthetic drugs
should consider their action on intracranial pressure. Regional ...