Waterhouse-Friderichsen Syndrome

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 Syndrome.

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 high.

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 of gangrene.

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, laboratory).

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 who survive the first few days of infection remain at risk for arrhythmias, myocarditis, and pericarditis. Regional anesthesia should be avoided, but controversies exist about providing vasodilatation that could help treat peripheral ischemic lesions. Central blockade is a high risk procedure in this indication because of the bleeding risk and because of its potential action on blood pressure; plexus blockade presents a lower risk.

Avoid anesthetic drugs that could affect contractility and cardiac output. Ketamine is a better choice if duration of shock is not prolonged (because of its action via catecholamine release). However, its use must be limited to patients who do not already present with cardiovascular collapse since most endogenous catecholamine reserves may be exhausted and worsening of the collapse may happen. Hypnomidate is best avoided because of its action on the adrenal glands. If regional anesthesia is provided, avoid bupivacaine because of its cardiac toxicity. Neuraxial blockade is not recommended. Peripheral block however may be used to increase peripheral circulation.

Schönlein-Henoch Purpura Syndrome: Characterized by purplish or brownish red discolorations on the skin and often associated with internal bleeding in various areas of the body. It affects the joints, gastrointestinal (GI), system, and kidneys. The central nervous system (CNS) can be, very rarely, affected.

Rheumatic Fever: The result of streptococcal infection. This inflammatory syndrome presents initially with moderate fever, sore throat, fatigue and a red rash. Major complications can include cardiovascular complications, severe joint pain and arthritis, dyskinetic movements with characteristic grimaces.

Rocky Mountain Spotted Fever: A tick-borne disease presenting after an incubation period of 2 to 12 days. The initial symptoms include the presence of gradual or abrupt rise in body temperature, followed by a characteristic purplish skin rash on the wrists and ankles. The fever and the rash usually become more severe after 7 to 14 days. The diagnosis is confirmed by a blood test.

Toxic Shock Syndrome: Symptoms appear very suddenly and manifest mostly by severe hyperthermia. The clinical features include headache, sore throat, and conjunctivitis. Other early symptoms include profound lethargy, periods of disorientation, vomiting, severe diarrhea, and a diffuse sunburn-like rash leading to sloughing of skin after several days. In severe cases, the syndrome may progress to cardiovascular shock and circulatory collapse within 48 hours.

Infective Endocarditis: Usually very sudden. Low back pain, arthralgia, and/or myalgia are common. Fever, night sweats, chills, headache and loss of appetite may also occur. Hematuria, petechiae, and oval spots on the retina can be observed.