What is the recommended emergency management for an adult with pituitary apoplexy presenting with sudden severe headache, visual loss or field defects, ophthalmoplegia, altered mental status, or acute hormonal insufficiency?

Emergency Management of Pituitary Apoplexy

Immediately initiate intravenous hydrocortisone 50–100 mg every 6–8 hours as soon as pituitary apoplexy is suspected, without waiting for laboratory confirmation, to prevent life-threatening secondary adrenal insufficiency. 1

Initial Stabilization and Hormone Replacement

Corticosteroid Therapy (First Priority)

• Start stress-dose IV hydrocortisone (50–100 mg every 6–8 hours) immediately upon clinical suspicion—do not delay for lab results 1
• Continue stress dosing until hemodynamically stable, then taper to oral maintenance over 5–7 days 1
• Transition to oral hydrocortisone 15–20 mg daily in divided doses (approximately 2/3 morning, 1/3 early afternoon) once stable 1
• Never discontinue corticosteroids abruptly—gradual taper is mandatory to avoid adrenal crisis 1

Critical Sequencing Rule

• If both adrenal insufficiency and central hypothyroidism are present, always start corticosteroids first and add thyroid hormone only after at least one week of steroid therapy to avoid precipitating an adrenal crisis 2, 1

Fluid Resuscitation

• Administer at least 2 L of isotonic saline for volume resuscitation in the acute phase 1
• Monitor fluid and electrolyte balance closely, as imbalances are common 1

Diagnostic Workup

Immediate Laboratory Assessment

• Draw morning cortisol and ACTH (or random sample if critically ill) immediately on presentation 1
• Measure TSH and free T4 to identify central hypothyroidism 1
• Obtain gonadotropins (LH, FSH) and sex steroids (testosterone in males, estradiol in premenopausal females) at baseline 1
• Check serum sodium and osmolality initially, then repeat every 4–6 hours to detect diabetes insipidus or SIADH 1

Imaging

• MRI of the sella with pituitary cuts is the optimal first-line imaging test to confirm diagnosis 1
• Look for pituitary enlargement, stalk thickening, suprasellar convexity, heterogeneous enhancement, and hemorrhagic/necrotic components 1
• Noncontrast sequences are sensitive for hemorrhage, showing T1 hyperintensity, low T2 signal, or fluid-hemorrhage levels 1

Monitoring for Posterior Pituitary Dysfunction

Fluid Balance Surveillance

• Measure urine output hourly initially 1
• Monitor for polyuria (>3 L/day or >200 mL/hour for 2+ consecutive hours) indicating diabetes insipidus 1
• Watch for oliguria with hyponatremia suggesting SIADH 1
• Serial urine specific gravity and osmolality measurements should be obtained to detect sudden changes 1

Anticipated Patterns

• Transient AVP deficiency can develop even with initially preserved function 1
• Biphasic response (initial diabetes insipidus followed by SIADH) can occur after surgery or spontaneously 1
• Triphasic pattern (diabetes insipidus → SIADH → permanent diabetes insipidus) may occur post-operatively 1

Surgical Decision-Making

Indications for Urgent Transsphenoidal Decompression

• Progressive neurological deterioration 1
• Severe mass effect threatening the optic chiasm on imaging 1
• Persistent or worsening visual field defects despite medical management 3

Conservative Management Criteria

• Mild visual deficits with improving symptoms may be managed conservatively in highly selected cases 4, 5
• Good response to medical therapy without progressive neurologic compromise 1
• However, surgical decompression by experienced pituitary surgeons remains the preferred approach for most cases 1

Surgical Approach

• Transsphenoidal surgery by experienced pituitary surgeons is preferred 1
• Endoscopic rather than microscopic technique may provide better visualization and potentially superior outcomes in preserving pituitary function 1
• Surgeon experience is more critical than the specific technique chosen 1

Symptom Management

Headache Control

• Treat severe headache initially with acetaminophen and NSAIDs 1
• Refer to neurology if pain persists despite these agents 1

Visual Assessment

• Conduct formal visual field testing in any patient with visual symptoms 1
• Early assessment of visual acuity and visual fields is mandated, as visual deterioration is a well-described presentation requiring urgent surgical decompression if sight is threatened 2
• Abnormal results should be communicated to driver-licensing authorities 1

Post-Acute Management

Endocrinology Consultation

• Obtain immediate endocrinology consult for every case of pituitary apoplexy, regardless of severity, to guide hormone replacement and long-term follow-up 1

Patient Education and Safety

• Prior to discharge, provide education on "sick-day rules" and stress-dose steroids 1
• Prescribe an emergency intramuscular steroid kit with clear instructions 1
• All patients with adrenal insufficiency must obtain and carry a medical alert bracelet 1

Post-Operative Monitoring

• Strict monitoring of fluid and electrolyte balance is essential post-operatively 1
• Watch for diabetes insipidus and SIADH, which are common complications after pituitary surgery 1
• Monitor for CSF leak, which is a risk factor for postoperative complications 1

Special Populations

Pediatric Considerations

• Pituitary apoplexy is probably more common in pediatric non-functioning adenomas than in adults and may be more severe 2, 1
• Adult guidelines should be adopted for children and adolescents, with consideration for early surgery in selected patients 1
• The presence of arginine vasopressin deficiency (central diabetes insipidus) at diagnosis strongly suggests an alternative diagnosis such as craniopharyngioma, histiocytosis, or germ-cell tumor rather than typical NFPA apoplexy 2

Common Pitfalls to Avoid

• Never replace thyroid hormone before corticosteroids—this can trigger adrenal crisis by increasing cortisol metabolism 2, 1
• Do not wait for laboratory confirmation before starting stress-dose steroids in suspected cases 1
• Do not assume posterior pituitary function remains stable—diabetes insipidus can develop suddenly even with initially concentrated urine 1
• Not all intrapituitary hemorrhage on imaging represents symptomatic apoplexy—correlation with acute clinical syndrome is required 6, 7