We present a case of circulatory collapse found to be due to autoimmune adrenal insufficiency. Cardiac arrest and circulatory collapse occur uncommonly in children, with respiratory causes (e.g., pneumonia and bronchiolitis) being the most common.1 Other common etiologies include congenital heart diseases, sepsis, arrhythmias, fulminant myocarditis, blunt trauma and sudden infant death syndrome (SIDS). In considering the differential diagnosis, especially in the absence of known congenital heart disease, and the constellation of clinical and laboratory findings, other etiologies should be considered, especially reversible ones. The H’s and T’s mnemonic in Pediatric Advanced Life Support algorithm1 provides a useful quick screening method when triaging patients presenting with shock/cardiac arrest.
Primary adrenal insufficiency (PAI) results from decreased production of glucocorticoids or both gluco- and mineralocorticoid and is a life-threatening condition. This disease can be congenital or acquired. Congenital adrenal hyperplasia due to 21- Hydroxylase deficiency is the most common cause of congenital PAI.2 Addison’s disease characterized by autoimmune destruction of adrenal cortex is the most common cause of acquired PAI.3 This autoimmune process is defined by exaggerated humoral and cellular immune response in genetically predisposed patients. Usually, there is a trigger for the autoimmune process, including viral infections, drugs, or stress.4
The clinical presentation of PAI is nonspecific, often delaying diagnosis (Table 1), including a more chronic indolent course. As is illustrated in this case, the disease can also present as a life-threatening endocrine emergency, or so-called adrenal crisis. The manifestations include altered mental status, severe weakness, hypotension, hypoglycemia, and electrolyte disturbances (mostly hyponatremia and hyperkalemia).4 Hypoglycemia is more common in children compared to adults.3 This patient presented in decompensated shock which rapidly progressed to cardiopulmonary arrest requiring ECMO. This is an extremely rare presentation and has been rarely reported.5 Clinicians should be vigilant to clues that help reach prompt diagnosis. The most helpful exam and laboratory findings include skin hyperpigmentation, hyponatremia, and hypoglycemia respectively. Patients with Addison’s disease have high levels of proopiomelanocortin. The cleavage of proopiomelanocortin produces Adrenocorticotropic hormone (ACTH) and melanocyte-stimulating hormone, resulting in bronzing/hyperpigmentation of the skin.2 The presence of 21-hydroxylase autoantibodies is diagnostic of autoimmune PAI but are only positive in approximately 50% of cases.4
Table 1
Clinical features of adrenal insufficiency
| Nonspecific symptoms | Symptoms of hypoglycemia | Symptoms of mineralocorticoid deficiency | Features of elevated ACTH* concentrations | Laboratory findings |
| Fatigue, vomiting, nausea, abdominal pain, weakness, morning headaches, and failure to thrive | Pallor, sweatiness, disorientation and mood swings | Dehydration, collapse, hypotension, tachycardia, weight loss, dizziness and salt craving | Hyperpigmentation of nail beds, mucous membranes, palmar creases and scars | Hyponatremia (90%), hyperkaliemia (50%), hypochloremia, metabolic acidosis, fasting hypoglycemia with low cortisol, high ACTH and high renin |
| ACTH: adrenocorticotropic hormone |
When PAI is suspected, stress dose intravenous (IV) hydrocortisone is the treatment of choice and is lifesaving. IV hydrocortisone should be continued until clinical improvement and/or diagnosis of PAI is ruled out. When clinical and laboratory presentation do not fit the picture of adrenal insufficiency, the use of stress dose steroids is controversial, though considered not unreasonable as it is often used in refractory shock in patients undergoing cardiac surgery.6 Despite the frequent use of hydrocortisone, a systematic review and meta-analysis on the effects of steroids for relative adrenal insufficiency in pediatric shock showed no benefit.7
It is known that patients with adrenal shock have poor response to vasoactive medications.8 The unique feature of this case is the associated myocardial stunning and severe biventricular dysfunction. One might speculate that the air bubbles seen on transesophageal echocardiogram during cardiac catheterization were significant enough to cause myocardial ischemia and worsened dysfunction. This may have contributed to prolonged myocardial recovery time. Furthermore, adrenal crisis usually presents with hypovolemic shock9, rather than a cardiogenic shock. In the rare cases of decompensated shock that have been reported on patients with newly diagnosed Addison’s disease, it was speculated that congestive heart failure and resulting cardiogenic shock could be secondary to severe cachexia and malnutrition.10 However, this patient had normal growth parameters.