Classical pituitary apoplexy is a medical emergency and rapid replacement with hydrocortisone may be lifesaving. It is caused by haemorrhage and/or infarction of a tumour within the pituitary gland. A high index of clinical suspicion is essential to diagnose this condition as prompt management may be life and vision saving. This guideline aims to take the non-specialist through the initial phase of assessment and management.
The diagnosis of pituitary apoplexy is often delayed as ~80% of these patients will have no previous history of a pituitary problem and the clinical features mimic other more common neurological conditions.
A diagnosis of pituitary apoplexy should be considered in all patients who have acute severe headache and any of the following:
patients in whom subarachnoid haemorrhage (SAH) and meningitis have been excluded
patients with neuro-ophthalmic signs
patients with pre-existing pituitary tumours
Acute severe headache is the most common and earliest manifestation. Headache may be accompanied by nausea and vomiting
Ocular palsies, most commonly a third nerve palsy, can occur due to involvement of the cavernous sinus
Reduced visual acuity and visual field defects, most commonly a bi-temporal hemianopia, are due to optic chiasmal compression
Fever, neck stiffness, photophobia or reduced consciousness (similar to signs/symptoms of SAH or meningitis) may occur
Hypertension, major surgery, especially coronary artery bypass grafting, dynamic testing of the pituitary gland, anticoagulation therapy, coagulopathies, pregnancy and head trauma.
SAH due to ruptured intracranial aneurysm or arteriovenous malformation
Cavernous sinus thrombosis
Ensure haemodynamic stability through supportive measures
Urgent bloods: urea and electrolytes, full blood count, renal and liver function tests, clotting profile
Indications for empirical steroid therapy in patients with pituitary apoplexy are haemodynamic instability, altered consciousness level, reduced visual acuity and severe visual field defects. Steroid replacement is potentially lifesaving in these patients
In adults, hydrocortisone 100 mg i.m. bolus followed by 50–100 mg six hourly by intramuscular injection or 100–200 mg as an intravenous bolus followed by 2–4 mg per hour by continuous i.v. infusion can be used
Careful assessment of fluid and electrolyte balance
Ideally, endocrine evaluation with blood samples for random serum cortisol, TSH, free T4, prolactin, IGF1, LH, FSH, testosterone (men), oestradiol (women) for later analysis
Bedside assessment of visual acuity and fields
Further neuro-ophthalmic assessments can be undertaken when the patient is clinically stable
CT brain (± LP) to exclude SAH and meningitis should be undertaken if not already done
Magnetic resonance imaging (MRI) is the investigation of choice and has been shown to confirm the diagnosis in over 90% of patients. A pituitary CT is indicated if MRI is contraindicated or not possible
Urgent referral to the joint neurosurgical/endocrine unit for definitive management
After emergency care: where should patients with pituitary apoplexy be managed?
Once the diagnosis has been confirmed, it is recommended that all patients be transferred once medically stabilised, following liaison and advice from the specialist neurosurgical/endocrine team, to the local neurosurgical/endocrine team as soon as possible. Neurosurgical high dependency unit (HDU) facilities must be available. This team must have access to specialist endocrine and ophthalmological expertise. These patients should then be managed according the Society for Endocrinology UK guidelines for the management of pituitary apoplexy (1).
Indications for surgery
Patients should first be stabilised medically with steroid replacement, if needed, before surgical intervention. Studies have shown significantly greater improvement in visual acuity and visual field defects in patients who had early surgery (within 8 days).
Surgical intervention should be considered in patients with:
Severely reduced visual acuity
Severe and persistent visual field defects
Deteriorating level of consciousness
All patients with pituitary apoplexy need follow-up by endocrine and neurosurgical teams. They require repeat assessment of pituitary and visual function, at 4–6 weeks. Thereafter, 6–12 monthly follow-up to optimise hormonal replacement and to monitor tumour progression/recurrence.
See Fig. 1 for an emergency management summary.
Pituitary apoplexy is a rare and potentially lethal endocrine emergency, characterised by acute severe headache, visual defects, and/or reduced consciousness.
The clinical presentation often mimics other more common neurological emergencies.
Prompt resuscitation and corticosteroid replacement may be lifesaving.
MRI scan is the investigation of choice.
Urgent discussion with the regional neurosurgical/endocrine team is essential.
Surgical intervention should be considered in patients with severe and persisting visual defects or in those with deteriorating level of consciousness after medical stabilisation and steroid replacement.
The authors would like to thank Senthil Rajasekaran and Marian Lanyon for their contributions to the UK Guidelines for the Management of Pituitary Apoplexy.
The document should be considered as a guideline only; it is not intended to determine an absolute standard of medical care. The doctors concerned must make the management plan for an individual patient.