Waterhouse-Friderichsen Syndrome

At a glance

It is a medical condition characterized by fulminant clinical manifestation of bilateral adrenal hemorrhages as a result of pansystemic coagulopathy. The clinical features include: overwhelming organ failure, cardiovascular shock, disseminated intravascular coagulopathy, generalized purpura, adrenocortical insufficiency, and death if not treated immediately. The associated cyanosis, as a result of shock, is often extreme and often misleading of death condition. Major electrolyte disturbances, including severe hyperkaliemia and hypoglycemia require immediate attention. Leukopenia is considered a very poor prognostic sign. The association of extreme thrombocytopenia, prolonged prothrombin time (PT), and partial thromboplastin time (PTT) are suggestive of disseminated intravascular coagulopathy (DIC). It is caused by severe sepsis, classically meningococcemia (Neisseria meningitidis is the most common), but occasionally with other infections (influenza or colon bacillus). Prevention of the disease relates on routine vaccination against meningococcus, which is recommended by the Centers for Disease Control. It is highly recommended for all 11- to 18-year-olds and individuals with splenectomy, sickle-cell disease, and immunodeficiency.

Synonyms

Friderichsen Syndrome; Friderichsen-Waterhouse Syndrome; Friderichsen-Waterhouse-Bamatter Syndrome; Marchand-Waterhouse-Friderichsen Syndrome.

History

It is an acquired postinfectious disease that was first observed by Arthur Francis Voelcker in 1894. It was subsequently described by Rupert Waterhouse (1873-1958), an English physician, and Carl Friderichsen (1886-1979), a Danish pediatrician, in 1918.

Incidence

Infection with meningococcal is highest in children between 6 months and 1 year of age. It has also been reported during adolescence. Around 10 to 20% of patients with generalized meningococcal infection develop the fulminant meningococcemia that may lead to Waterhouse-Friderichsen Syndrome (WFS). Staphylococus aureus generalized infection has recently been implicated in pediatric WFS.

Pathophysiology

Dissemination of meningococci via the blood stream from the primary focus of infection in the nasopharynx, resulting in extensive and acute inflammatory reaction in various organs, particularly the meninges. It is postulated that endotoxin released from the diplococci induces a severe reaction including significant vasomotor disturbance, myocardial failure, DIC, bleeding, and focal necrosis in the adrenal glands and skin.

Diagnosis

It is a medical condition presenting with an abrupt and rapid deterioration of clinical condition. The hematological profile confirms the presence of DIC (high polymorphonuclear leukocyte counts, thrombocytopenia, prolonged PT and PTT). Biochemical analysis shows raised serum urea and creatinine levels, electrolyte disturbances, especially severe hypoglycemia and hyperkaliemia. The clinical presentation includes severe cyanotic pallor and peripheral coldness due to profound vasoconstriction (often considered premortal), and generalized petechial or purpuric rash. Fever is initially moderate but subsequently becomes high.

Clinical aspects

Circulatory collapse, which is characterized by clammy skin, high fever, a rapid, thready pulse, labored respiration, and coma. Other clinical features may include dehydration, vomiting, diarrhea, oliguria, and neck stiffness, and occasionally anuria. Usually occurs in infants or children, ...