Waterhouse-Friderichsen syndrome

This code represents Waterhouse-Friderichsen syndrome, a severe and often fatal complication of meningococcal septicemia characterized by massive bilateral adrenal hemorrhage. It results in acute adrenocortical insufficiency, leading to profound shock, purpura, and disseminated intravascular coagulation (DIC).

Use this code when documentation clearly indicates the presence of Waterhouse-Friderichsen syndrome, typically in the context of overwhelming sepsis, most commonly due to Neisseria meningitidis. Supporting documentation will describe adrenal hemorrhage, acute adrenal crisis, and the clinical signs of severe septic shock.

• Always code the underlying cause of the sepsis (e.g., meningococcal infection) first, followed by this code.
• Look for documentation of adrenal hemorrhage, purpura fulminans, and acute adrenal insufficiency.
• Do not confuse with other forms of adrenal insufficiency not caused by acute hemorrhage secondary to sepsis.