P2.251: Novel Case of Waterhouse-Friderichsen Syndrome in Neonate with Enterovirus Illness

Introduction: Waterhouse-Friderichsen Syndrome (WFS) is a rare but dangerous presentation of bilateral adrenal hemorrhage and
consequent adrenal crisis. It is most often caused by meningococcal sepsis, but a few viruses have also been reported. Of note, never before seen with enterovirus. WFS is more common in children than adults, though vaccinations have decreased its incidence (0.26/100,000).

Case Description: Previously healthy 1 week old term male presented to the ED for lethargy and poor feeding. Work up showed elevated inflammatory markers, hypoglycemia to 48 mg/dl, mild hyperkalemia to 5.9, and positive enterovirus result on nasopharynx and CSF PCR. Initial borderline hypotension improved with fluids. After admission to the floor for viral meningitis, the patient developed hypoxic respiratory failure and hypotension requiring PICU transfer for intubation and blood pressure management. He was found to be hyponatremic (132) and hyperkalemic (7.8) so was started on stress dose hydrocortisone with improvement. Due to myocarditis complication seen on cardiac markers and echo, ID recommended IVIG and antiviral treatment with Pocapavir. During the hospitalization we saw correction of shock and electrolyte derangements on scheduled hydrocortisone. An adrenal US was done to follow up on adrenal crisis which showed bilateral adrenal hemorrhage. An ACTH stimulation test demonstrated a poor response of cortisol production (Figure 1). This confirmed primary adrenal insufficiency consequently from adrenal hemorrhage. Aldosterone and renin were both low, requiring fludrocortisone, hydrocortisone, and salt supplementation prior to discharge. Once at home, a second adrenal crisis with hyponatremia and hyperkalemia occurred due to incomplete medication compliance. Once regimen was optimized, the patient stabilized with continued need for glucocorticoid, mineralocorticoid, and salt replacement.

Discussion: Enterovirus is known to cause disease in neonates with reports of febrile illness, meningitis, hepatitis, and myocarditis; but never reported to cause WFS. WFS classically presents with a shock-like picture in the setting of non-specific sick symptoms and a characteristic petechial rash. The mechanism proposed for adrenal hemorrhage is a coagulopathy due to the sharp transition from multi-arterial supply and minimal venous drainage. An increase in ACTH promotes blood flow and increased pressure within the adrenal system predisposing to hemorrhage.

Conclusion: We are reporting a pivotal case of enterovirus causing adrenal hemorrhage and the primary adrenal insufficiency sequela of salt wasting crisis. WFS has a high mortality rate partly due to quick deterioration and often late recognition, with late diagnosis causing 50% mortality. Due to substantial risk of fatality if missed and viable treatment options; it is important to consider WFS in a patient who presents like septic shock, especially if refractory to fluids/pressors.