the case.
a 28 year old female is brought into your resuscitation bay by paramedic crews following a two day history of nausea and vomiting. As she rolls past, you notice that she is distressed with pain, looks clinically very dry and is quite drowsy.
Her vital signs are:
- Pulse: 156
- BP – unrecordable
- SaO2 – 82%
- RR – 32
- GCS – 13 (E3V4M6)
- Temp – 38.2*C
As you commence your primary survey her sister enters the room and informs you that she has had nausea and vomiting for “a few days” and that she has developed “a strange bruise around the belly button”.
On examination:
- Distressed with pain.
- Tachypnoeic without obvious accessory muscle use.
- Cold hands & feet. Warm centrally.
- Chest clear to auscultation.
- HS dual. No murmurs, rubs or gallop.
- Abdomen: generalised tenderness with periumbical swelling, tenderness and surrounding ecchymoses. No organomegaly. No prior surgical scars.
- No peripheral oedema.
- No focal neurological deficit.
You pause for a moment and think…
- Sepsis:
- Pneumonia
- Meningoencephalitis
- Intra-abdominal (biliary, appendicitis, colitis, etc…)
- + others…
- Gastrointestinal:
- Pancreatitis ?Cullen’s sign
- Mesenteric ischaemia
- Bowel obstruction
- Malignancy (+gastric outlet obstruction)
- Cardiogenic:
- Dysrhythmia
- Valvular heart disease
- Myo-pericarditis
- Metabolic/Endocrine:
- Diabetic ketoacidosis
- Adrenal insufficiency
- Ectopic pregnancy
- Mixed acid-base disturbance
- Features of metabolic acidosis:
- Lactate >8, Urea 26 – contributing to a HAGMA
- Features of respiratory acidosis:
- Elevated pCO2 (56 mmHg)
- Likely concomitant metabolic alkalosis:
- Despite above findings, pH is only 7.32.
- Hx of vomiting predicts volume-contracted metabolic alkalosis
- Features of metabolic acidosis:
- Severe hyperlactataemia
- Life threatening hyperkalaemia requiring immediate correction
- Moderate hyponatraemia
- Elevated serum urea & creatinine
- Acute kidney injury ?2* hypovolaemia/hypotension/sepsis
- Elevated haematocrit
- Supportive of volume depletion
Interpretation.
Mixed acid-base disturbance with markedly elevated lactate, acute kidney injury and life-threatening hyperkalaemia plus evidence of hypovolaemia.
This could be explained by septic shock, gut ischaemia or toxic ingestion (toxic alcohol, metformin or iron overdose, etc).
The combination of hyperkalaemia, hyponatraemia & elevated lactate raises suspicion for adrenal insufficiency.
The patients’ sister wants to give you more details that she thinks is important…
- She tells you that the patient has a history of Arthritis as well as Adult-onset Still’s disease
- She takes 25mg of Prednisone daily
- She has not opened her bowels or passed flatus for the past 2 days.
You are now convinced her umbilical swelling is an incarcerated hernia…
- Maximise oxygenation.
- High flow oxygen via non-rebreather mask
- Optimise perfusion
- IV access
- 20mL/kg 0.9% Saline bolus (~500-1000mL), repeat x2
- Aim: SBP >100 (MAP >65), cap refill <2 sec, lactate clearance (>10% per hour), urine output >0.5 mL/kg/hr.
- Correct life-threatening hyperkalaemia
- Calcium (QRS >100msec)
- 20mL of 10% gluconate solution
- Salbutamol
- 10mg via nebuliser
- Insulin + dextrose
- Calcium (QRS >100msec)
- Steroid replacement
- IV Hydrocortisone 4mg/kg (~200mg)
- Continue 50-100mg q6h IV
- Treat potential underlying sepsis
- Fever at triage, plus elevated lactate & abdominal pain.
- Empiric antibiotics (Ampicillin, Metronidazole, Gentamicin)
- Detect & correct underlying precipitating illness
- Urgent surgical bedside review
- Strict fluid balance
- Nasogastric tube
- Supportive care
- Analgesia
- Simple (paracetamol)
- Titrated intravenous opiates
- Urinary catheter
- Control temperature
- DVT prophylaxis
- Update family
- Analgesia
Adrenal Crisis
Adrenal crisis refers to acute adrenal insufficiency, which is the clinical manifestation of deficient production or action of glucocorticoids, with or without deficiency also in mineralocorticoids and adrenal androgens. It is a disorder that can result from primary adrenal failure or secondary adrenal disease due to impairment of the hypothalamic–pituitary axis.
Adrenal cortex refresher.
The adrenal cortex has three distinct zones, which secrete the various hormones under the direct control of well understood feedback mechanisms.
The Adrenal Glands. Three cortical layers with different hormone production & function. Courtesy of BC Open Textbooks
The basics.
Adrenal crisis is most common in patients with primary adrenal insufficiency, but may also occur in those with secondary or tertiary adrenal insufficiency (eg. from acute illness or stressor in patients with chronic adrenal insufficiency who are not adequately replaced). It is a life-threatening emergency that requires immediate diagnosis and management.
Depending on the underlying mechanism, adrenal insufficiency is classified as primary, secondary, or tertiary.
Primary adrenal insufficiency.
- Mineralocorticoid deficiency
- Results from disease intrinsic to the adrenal cortex.
- Causes:
- Autoimmune adrenalitis (eg. Addison’s)
- Isolated vs Polyendocrinopathy
- Infectious adrenalitis (eg. TB, HIV, syphilis)
- Bilateral adrenal haemorrhage (Waterhouse-Friderichsen syndrome)
- Bilateral adrenal metastases/infiltration
- Genetic disorders
- Autoimmune adrenalitis (eg. Addison’s)
- Major clinical features: volume depletion & hypotension
Secondary & tertiary adrenal insufficiency.
- aka. central adrenal insufficiency
- Isolated glucocorticoid deficiency
- Causes:
- Pituitary tumours
- Post-pituitary surgery/radiation
- Pituitary infiltration/infection
- Pituitary apoplexy (including Sheehan’s syndrome)
- Genetic disorders
- Drug-induced adrenal insufficiency – as in this case, is the most common cause of tertiary adrenal insufficiency.
- Major clinical features: hypotension (from decreased vascular tone)
- Volume depletion tends not to occur
Clinical features.
Primary adrenal insufficiency.
This clinical picture results from deficiency of all adrenocortical hormones (aldosterone, cortisol, androgens); they can also include signs of other concurrent autoimmune conditions. Most of the symptoms are non-specific (fatigue, anorexia, weight loss, nausea & vomiting). Hypoglycaemia can be the presenting sign in children.
A specific sign of chronic (but not acute) primary adrenal insufficiency is hyperpigmentation. This predominantly affects areas of skin subjected to pressure (elbows, knuckles, palmar creases, lips, buccal mucosa). It is caused by stimulation of the melanocortin-1 receptor in the skin by the high circulating corticotropin concentrations.
Central adrenal insufficiency.
The clinical manifestations of secondary or tertiary adrenal insufficiency result from glucocorticoid deficiency only (secretion of aldosterone and adrenal androgens is preserved). They may also herald signs of the primary underlying disorder as well.
ADRENAL CRISIS:
A life-threatening adrenal crisis can be the first presentation of adrenal insufficiency. The acute presentation can be precipitated by a physiological stress, such as surgery, trauma, or an intercurrent infection.
Clinical features include vomiting, fever, confusion, abdominal pain, myalgia, joint pains, severe hypotension and hypovolaemic shock.
Investigations typically reveal hypoglycaemia, hyponatraemia (with hypoosmolarity), hyperkalaemia & elevated urea and creatinine.
The diagnosis is made clinically, or by (1) plasma cortisol level < 80 mmol/L or (2) a short synacthen test of 250mcg (normal response = cortisol > 525mmol/L).
Management.
Treatment of acute adrenal crisis consists of immediate administration of hydrocortisone (100mg IV q6h) and volume replacement. Hypoglycaemia should be immediately reversed and electrolyte derangements should be corrected as required (especially hyperkalaemia).
NB. The use of hydrocortisone will complicate further assessment of adrenal function. An alternative, which does not interfere with measurement of cortisol and ACTH stimulation testing, is the administration of dexamethasone 4 to 6 mg every 12 h given intravenously or intramuscularly.
Mineralocorticoid replacement (oral fludrocortisone 0.1mg Q6 hrly) may be required beyond the acute resuscitation phase, however is often not required in patients with secondary adrenal insufficiency or in those with primary adrenal insufficiency receiving more than 50mg hydrocortisone daily, given its potent mineralocorticoid activity at high doses.
The underlying precipitating cause should be sought and addressed also.
Stress dose steroids in patients with chronic adrenal insufficiency.
Recommendations regarding glucocorticoid coverage during non-surgical illnesses are largely based on expert consensus. Traditionally patients have been advised to double or triple their daily dose of glucocorticoid therapy during a febrile illness until recovery.
Here is her CXR…
Clear lung fields. Visible dilated loops of small-bowel in upper abdomen.
The Surgical Registrar arrives at the patients’ bedside. He announces that he will take the patient to theatre now if you can prove that they have a bowel obstruction…
Dilated small bowel loops (>3.4cm) with oedematous wall.
The patient leaves your resus bay for the operating theatre within 90 minutes of arriving to hospital.
She undergoes a laparotomy, small bowel resection (~8cm) with an end-to-end anastomosis and umbilical hernia repair. Following this, she makes an uneventful recovery with stress-dose steroid coverage under the guidance of the Endocrinologists.
References.
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Charmandari, E., Nicolaides, N. C., & Chrousos, G. P. (2014). Adrenal insufficiency. The Lancet, 383(9935), 2152–2167.
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Jung, C., & Inder, W. J. (2008). Management of adrenal insufficiency during the stress of medical illness and surgery. The Medical Journal of Australia, 188(7), 409–413.
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Shenker, Y., & Skatrud, J. B. (2001). Adrenal insufficiency in critically ill patients. American Journal of Respiratory and Critical Care Medicine, 163(7), 1520–1523.
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Clinical manifestations of adrenal insufficiency in adults – UpToDate.com
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Treatment of adrenal insufficiency in adults – UpToDate.com
- Adrenal insufficiency – Life in the Fast Lane
Similar cases via #FOAM.
UOTW #44 via Ultrasound of the Week
Clinical Case 064: Um-bil-obstruction via Broome Docs
Author: Andi Rauch
Web editing + additional writing: Chris Partyka
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