Pathological Features of Waterhouse-Friderichsen Syndrome in Infancy and Childhood
Between 1997 and 2002, five cases of fatal Waterhouse—Friderichsen syndrome (WFS) that occurred in infancy or childhood were investigated in our institute. The diagnosis of WFS was based on the following clinical as well as morphological criteria: (a) fulminant sepsis, (b) patchy purpura of the skin as a result of disseminated intravascular coagulation (DIC), and (c) bilateral hemorrhagic necroses of the adrenals. All cases had a very rapid clinical course of the disease (about 1 day or less). In two cases, postmortem microbiological examinations yielded meningococci as the infective agent. In both cases, the children examined underwent autopsy very early after death (5 hours and 24 hours, respectively) and did not receive antibiotics prior to death. In two other cases, meningococci were cultured in antemortem blood samples. Macroscopically, the leptomeninges looked inconspicious in all five cases investigated. However, histology revealed meningitis to a mild or moderate degree in four cases. A previously clinically undiagnosed interstitial myocarditis was diagnosed in three cases by histological means, of which two cases showed Gram-negative diplococci in the myocardial lesions. For the pathologist investigating cases of WFS in infancy and childhood, the following points have to be emphasized: (a) when performing a medicolegal autopsy in a case of suspected WFS, the postmortem interval should be as short as possible (not longer than 24 hours) to provide the opportunity for an accurate microbiological examination of postmortem swabs; (b) in cases of WFS, meningitis does not seem to play a leading role for the clinical course of the disease or the actual cause of death; however, histologically a mild to moderate degree of meningitis is a frequent finding; (c) the presence of Gram-negative diplococci in myocardial lesions by histological means suggests that invasion of meningococci might be a causative factor for myocarditis in WFS; and (d) in accordance with the literature, myocarditis is often present in cases of WFS and therefore might be of importance for the clinical course. It is not certain yet if all children die from shock or DIC or rather, if myocarditis, leading to complete heart block, forward failure of the heart, or arrhythmia, is the actual cause of death.
Key WordsWaterhouse—Friderichsen syndrome (WFS) infancy Childhood Neisseria meningitidis Diplococci Meningitis Myocarditis disseminated intravascular coagulation (DIC) postmortem microbiology histopathology
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