Waterhouse-Friederichsen syndrome in infants and children (Forensic case series)

Abstract

Massive adrenal haemorrhage is an uncommon finding at autopsy, but patognomonic for Waterhouse-Friederichsen syndrome (WFS -described for the first time as clinical entity in 1911), ussually associated with fulminant meningococemia. Aim of our research was to analyze demographic, clinical and necroptic findings in cases of lethal WFS in infants and children (0 -2 years). We conducted a retrospective study on forensic autopsy cases performed in Mures county between the years 2011 -2015 by reviewing the necropsy protocols together with the clinical documentation and the reinterpretation of microscopic findings. During the period of five years, we found 133 cases of autopsies in the age group 0 -2 years, among them in 5 cases (3.76%) WFS was revealed. Male gender prevailed (80%) and average annual incidence of fatal WFS was 5.88/100.000 relative to the population in our County of the same age group. A poor socioeconomic status was described in 4 out of 5 cases (80%). Time from onset of symptoms to death varied from 45 minutes to 8 hours. All children had earlier a form of respiratory infectious pathology. The typical rash of purple spots on the skin was described in four cases, while internal examination revealed cerebral edema and interstitial pneumonia also in four cases each. Adrenal necrosis was noticed in two cases and contrary to other reports in the literature, we found no myocardial infalmmatory involvement in any of our cases. Positive microbiological finding was noted in only one case (Neisseria meningitidis).