Clinical Case Scenario

A 36-year-old female presented with history of 1 day of fever associated with rash, and breathlessness, rapidly progressing to severe metabolic acidosis, refractory septic shock requiring multiple vasopressors, acute kidney injury, and disseminated intravascular coagulopathy with multi-organ dysfunction. Physical examination revealed multiple petechial patches (Figure 2) all over the body. Despite aggressive management, including intubation, mechanical ventilation, broad-spectrum antibiotics, and fluid resuscitation, the patient's condition deteriorates, ultimately leading to cardiac arrest and death within 36 hours of ICU admission.

A POCUS of the abdomen of this patient during resuscitation revealed the findings which are shown in figure 1. A screening echo was normal with good left ventricular systolic function. Preliminary blood culture findings show gram-negative diplococci.

Figure 1: POCUS scan of right adrenal gland
(Case courtesy of Mohammad walid Ahmad Amin, Radiopaedia.org, rID: 192003)

Figure 2: Petechial patches on body
(Image sourced from DermNet)

Wrong Answer: A. Toxic Shock Syndrome (TSS):
The rash is typically a diffuse erythroderma (sunburn-like) followed by desquamation. Although it can cause fever, rash and shock with multi-organ dysfunction, the specific widespread ecchymotic (purpuric) rash and the direct association with adrenal hemorrhage are not as characteristic as in WFS. TSS is more commonly associated with Staphylococcus aureus or Streptococcus pyogenes.

Wrong Answer: B. Disseminated Intravascular Coagulation (DIC):
DIC is a component of WFS and is present in the patient (indicated by the ecchymotic patches). However, DIC is a coagulation disorder that can be triggered by various underlying conditions, whereas WFS specifically refers to the adrenal hemorrhage and profound shock often seen due to fulminant meningococcemia.

Right Answer: C. Waterhouse-Friderichsen Syndrome (WFS):
WFS is due to direct toxic effects of meningococcemia, which can lead to adrenal infarction with subsequent adrenal insufficiency. This syndrome is characterized by massive, bilateral non-traumatic adrenal hemorrhage in the setting of severe bacterial infection, most commonly Neisseria meningitidis (meningococcemia), leading to acute adrenal insufficiency and profound, refractory shock, along with a widespread purpuric rash. The rash appears as lesions 1 to 2 mm in diameter, mostly on the trunk and lower portions of the body. The rapid progression, severe shock, and prominent rash in the patient's presentation are highly suggestive of this syndrome, which is a severe complication of fulminant meningococcemia.

Key Mnemonic: "CRASH" for WFS
● Cortisol deficiency (Addisonian crisis)
● Rash (purpura)
● Adrenal hemorrhage
● Shock (refractory)
● Hemorrhagic DIC

Explaination
The gram-negative diplococci on preliminary blood culture is suggestive of Neisseria meningitidis (meningococcaemia).
The POCUS in Figure 1 shows a cyst-like lesion with septations and without detectable internal vascularity, measuring about 3.2 x 3.2 cm, is located in the anatomical location of the right adrenal gland, suggesting adrenal haemorrhage (white arrows). (Figure 1)
Figure 3: POCUS scan of right adrenal gland (Case courtesy of Mohammad walid Ahmad Amin, Radiopaedia.org, rID: 192003)

Figure 3: POCUS scan of right adrenal gland
(Case courtesy of Mohammad walid Ahmad Amin, Radiopaedia.org, rID: 192003)

Wrong Answer: D. Cardiogenic shock with multi-organ dysfunction: Considering that the patient had no prior cardiac disease and screening echocardiography showed good left ventricular systolic function, the possibility of cardiogenic shock is ruled out