Advertisement

Pathological Features of Waterhouse-Friderichsen Syndrome in Infancy and Childhood

  • Jan P. Sperhake
  • Michael Tsokos
Part of the Forensic Pathology Reviews book series (FPR, volume 1)

Summary

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 Words

Waterhouse—Friderichsen syndrome (WFS) infancy Childhood Neisseria meningitidis Diplococci Meningitis Myocarditis disseminated intravascular coagulation (DIC) postmortem microbiology histopathology 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Waterhouse R (1911) A case of suprarenal apoplexy. Lancet 1, 577.CrossRefGoogle Scholar
  2. 2.
    Friderichsen C (1918) Nebennierenapoplexie bei kleinen Kindern. Jahrb Kinderheilk 87, 109.Google Scholar
  3. 3.
    Harris P, Bennett A (2001) Waterhouse—Friderichsen syndrome. N Engl J Med 345, 841.PubMedGoogle Scholar
  4. 4.
    Mühlig K, Theile J, Dalitz E (1995) Einseitige Nebennierenblutung—ein Waterhouse Friderichsen-Syndrom? Ultraschall Med 16, 293–296.PubMedCrossRefGoogle Scholar
  5. 5.
    Külz J, Kroll 0 (1984) Zur aktuellen Problematik des sogenannten WaterhouseFriderichsen-Syndroms im Kindesalter. Kinderärztl Praxis 52, 3–15.Google Scholar
  6. 6.
    Ryan CA, Wenman W, Henningsen C, Tse S (1993) Fatal childhood pneumococcal Waterhouse-Friderichsen Syndrome. Pediatr Infect Dis J 12, 250–251.PubMedCrossRefGoogle Scholar
  7. 7.
    Tsokos M (2003) Fatal Waterhouse—Friderichsen syndrome due to Ewingella americana infection. Am J Forensic Med Pathol 24, 41–44.PubMedGoogle Scholar
  8. 8.
    Mirza I, Wolk J, Toth L, Rostenberg P, Kranwinkel R, Sieber SC (2000) WaterhouseFriderichsen syndrome secondary to Capnocytophaga canimorsus septicemia and demonstration of bacteremia by peripheral blood smear. Arch Pathol Lab Med 124, 859–863.PubMedGoogle Scholar
  9. 9.
    Jacobs RF, Hsi S, Wilson CB, Benjamin D, Smith AL, Morrow R (1983) Apparent meningococcemia: clinical features of disease due to Haemophilus influenzae and Neisseria meningitidis. Pediatrics 7, 469–472.Google Scholar
  10. 10.
    Lineaweaver W, Franzini D, Dragonetti D, McCarley D, Rumley T (1986) Haemophilus influenzae meningitis and Waterhouse-Friderichsen syndrome in an adult. South Med J 79, 1034–1036.Google Scholar
  11. 11.
    McKinney WP, Agner RC (1989) Waterhouse-Friderichsen syndrome caused by Haemophilus influenzae type b in an immunocompetent young adult. South Med J 82, 1571–1573.PubMedCrossRefGoogle Scholar
  12. 12.
    Ip M, Teo JG, Cheng AF (1995) Waterhouse-Friderichsen syndrome complicating primary biliary sepsis due to Pasteurella multocida in a patient with cirrhosis. J Clin Pathol 48, 775–777.PubMedCrossRefGoogle Scholar
  13. 13.
    Agraharkar M, Fahlen M, Siddiqui M, Rajaraman S (2000) Waterhouse-Friderichsen syndrome and bilateral renal cortical necrosis in meningococcal sepsis. Am J Kidney Dis 36, 396–400.PubMedCrossRefGoogle Scholar
  14. 14.
    Karakousis PC, Page KR, Varello MA, Howlett PT, Stieritz DD (2001) WaterhouseFriderichsen syndrome after infection with group A streptococcus. Mayo Clin Proc 76, 1167–1170.PubMedCrossRefGoogle Scholar
  15. 15.
    Busund R, Straume B, Revhaug A (1993) Fatal course in severe meningococcemia: clinical predictors and effect of transfusion therapy. Crit Care Med 21, 1699–1705.PubMedCrossRefGoogle Scholar
  16. 16.
    Cremer R, Leclerc F, Martinot A, Sadik A, Fourier C (1997) Adverse outcome in children with meningococcemia. J Pediatr 131, 649–651.PubMedGoogle Scholar
  17. 17.
    Havens PL, Garland JS, Brook MM, Dewitz BA, Stremski ES, Troshynski TJ (1989) Trends in mortality in children hospitalized with meningococcal infections, 1957 to 1987. Pediatr Infect Dis J 8, 8–11.PubMedCrossRefGoogle Scholar
  18. 18.
    Sorensen HT, Steffensen FH, Schonheyder HC, Nielsen GL, Hansen I, Madsen KM, et al. (1998) Trend in incidence and case fatality of meningococcal disease over 16 years in Northern Denmark. Eur J Clin Microbiol Infect Dis 17, 690–694.PubMedCrossRefGoogle Scholar
  19. 19.
    Gradaus F, Klein RM, von Giesen H-J, Arendt G, Heintzen MP, Leschke M, et al. (1999) Klinischer Verlauf und Komplikationen der Meningokokkensepsis. Med Klin 94, 633–637.CrossRefGoogle Scholar
  20. 20.
    Mok Q, Butt W (1996) The outcome of children admitted to intensive care with meningococcal septicaemia. Intensive Care Med 22, 259–263.PubMedCrossRefGoogle Scholar
  21. 21.
    Schroten H (2000) Meningokokkeninfektionen. In: Deutsche Gesellschaft für pädiatrische Infektiologie e.V., ed., Infektionen bei Kindern und Jugendlichen, 3rd ed. Futuramed, München, pp. 437–442.Google Scholar
  22. 22.
    Böhm N (1982) Adrenal, cutaneous and myocardial lesions in fulminating endotoxinemia (Waterhouse-Friderichsen syndrome) Pathol Res Pract 174, 92–105.PubMedGoogle Scholar
  23. 23.
    Tsokos M, Püschel K (2001) Postmortem bacteriology in forensic pathology: diagnostic value and interpretation. Legal Med 3, 15–22.PubMedCrossRefGoogle Scholar
  24. 24.
    Rosenberg H, Bortolussi R, Gatien JG (1981) Rash resembling anaphylactoid purpura as the initial manifestation of meningococcemia. Can Med Assoc J 15, 179–180.Google Scholar
  25. 25.
    Vos GD, Wiegman A, Romijn JA, Meurs AM, Bruins-Stassen MJ, Bijlmer RP, et al. (1989) Not meningitis but septic shock is the killer in acute meningococcal disease. Ned Tijdschr Geneeskd 133, 772–775.PubMedGoogle Scholar
  26. 26.
    Riordan FAI, Marzouk O, Thomson APJ, Sills JA, Hart CA (1995) The changing presentations of meningococcal disease. Eur J Pediatr 154, 472–474.PubMedCrossRefGoogle Scholar
  27. 27.
    Detsky AS, Salit IE (1983) Complete heart block in meningococcemia. Ann Emerg Med 12, 391–393.PubMedCrossRefGoogle Scholar
  28. 28.
    Böhm N (1978) Waterhouse-Friderichsen-Syndrom: Morphologische Befunde und Aspekte zur Pathogenese. Verh Dtsch Ges Path 62, 449–455.Google Scholar
  29. 29.
    Schütte B (1978) Waterhouse-Friderichsen-Syndrom—Klinischer Verlauf und Gerinnungsstatus. Verh Dtsch Ges Path 62, 442–448.Google Scholar
  30. 30.
    Dashefsky B, Teele DW, Klein JO (1 983) Unsuspected meningococcemia. J Pediatr 102, 69–72.Google Scholar
  31. 31.
    CDC (1997) Control and prevention of meningococcal disease. Recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Recomm Rep 46 (No. RR-5), 1–10.Google Scholar

Copyright information

© Springer Science+Business Media New York 2004

Authors and Affiliations

  • Jan P. Sperhake
  • Michael Tsokos

There are no affiliations available

Personalised recommendations