Fulminant disease associated with bilateral adrenal
hemorrhage as a result of coagulopathy associated with severe sepsis,
classically meningococcemia (Neisseria meningitidis most common), but occasionally with other
infections (influenza or colon bacillus). Fatal if not treated immediately.
Friderichsen Syndrome; Friderichsen-Waterhouse Syndrome;
Friderichsen-Waterhouse-Bamatter Syndrome; Marchand-Waterhouse-Friderichsen
Acquired postinfectious disease that was first described
by Arthur Francis Voelcker in 1894, and subsequently explained by Carl
Friderichsen, Danish pediatrician in 1918.
Attack rate of meningococcal disease is highest for
children between 6 months and 1 year of age. Another, but lower,
incidence has been reported during adolescence; 10 to 20% of patients with generalized
meningococcal infection develop the fulminant meningococcemia.
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, disseminated intravascular coagulopathy (DIC), bleeding, and focal
necrosis in the adrenal glands and skin.
Abrupt and rapid deterioration of clinical symptoms;
hematological (high polymorphonuclear leukocyte counts, thrombocytopenia,
evidence of DIC); biochemical (raised serum urea and creatinine levels,
electrolyte disturbances), and clinical (cyanotic pallor, a petechial or
purpuric rash, pale with coldness and cyanosis of the extremities as a
result of generalized vasoconstriction). Fever is initially moderate but subsequently becomes
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, occasionally in adults. Death usually occurs after a few hours if not treated.
Adrenal insufficiency being the immediate cause. Patients who recover may
suffer from extensive sloughing of the skin and loss of digits as a result
Evaluate cardiac function (clinical,
echography, Swan-Ganz catheter), adrenal function (clinical, electrolytes),
coagulation and hemostasis (complete laboratory test including coagulation
factors, soluble complexes), and renal and liver functions (clinical,
Surgical indications are strictly
limited in such patients. Anesthesia requires aggressive resuscitation using
plasma expanders and inotropes, with the aid of invasive hemodynamic
monitoring through arterial lines, central venous catheter, and pulmonary
arterial catheter, if indicated. Specific antibiotic therapy, preferably
high-dose penicillin, should be started as soon as possible because of the natural
frequency of meningococcus infectious spread. Corticosteroids may also be indicated if
patients remain unresponsive to fluid resuscitation and inotropic therapy. Secure airway
immediately in comatose patients; use positive pressure ventilatory support to optimize oxygen
delivery. Onset of pulmonary edema or acute respiratory distress syndrome
requires the use of positive end-expiratory pressure (PEEP). Avoid nasal intubation
with its increased risk of bleeding from the adenoids in patients with DIC.
Treatment of elevated intracranial pressure may also be necessary, as well
as insertion of devices to monitor intracranial pressure. Patients ...