Pituitary apoplexy

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- Section Writer: Dr. Om J Lakhani
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Q. What is Pituitary apoplexy?
- Clinical syndrome caused by acute ischemic infarction or hemorrhage within a pituitary adenoma.
- Typically occurs in the setting of an existing pituitary adenoma.
- Leads to rapid expansion of the adenoma.
- Causes acute onset of symptoms like headache, visual impairment, ocular paresis, altered mental status, and hormonal dysfunction.
- Results from compression of the normal pituitary gland or the hypothalamus.
Q. Is it because of infarct or hemorrhage in the pituitary ?
- Could be either
Q. Can it be asymptomatic ?
- So the infarct or hemorraghe can be asymptomatic
- However, it is not under the umbrella definition of Pituitary apoplexy
Q. Is Sheehan a form of pituitary apoplexy ?
- Since Sheehan is subclinical- it does not fall in the said definition
- However it is also a form of Pituitary infarction
Q. Is it reported in which most common subtype of Pituitary adenoma?
- It is most commonly reported in NON-FUNCTIONING PITUITARY ADENOMAS
- Amongst the functional tumors it is most common in Prolactinoma
Q. Can it also occurs in conditions not associated with Pituitary adenoma?
- Rarely, it can occur in the absence of a pituitary adenoma in conditions such as Rathke's cleft cysts, pituitary hyperplasia, or inflammatory lesions.
Q. What are the outcomes of pituitary apoplexy ?
- Detoriation- SAH or cerebral vasospasm
- Improvement-
  - With sequel – Pituitary insufficiency, Neurological diorder or visual deficiet
  - Without sequel
Q. What are the Precipitating factors ?
- Angiographic procedures
- Anticoagulants
- Increase of blood pressure
- Dopamine agonists
- Pituitary dynamic testing
- High dose of estrogens
- Trauma
- Bleeding disorder
- Cardiac surgery
- Increase of intracranial pressure
Q. What are the pathophysiological reasons for pituitary apoplexy ?
- Tumor outgrowing its blood supply
- Superior hypophyseal artery compressing against the diaphragmatic sellae
- Inferior hypophyseal artrey – entrapment against the diaphragmatic notch
Q. Is there a change in the blood supply between a normal pituitary and that of an adenoma ?
- Yes
- Normal pituitary is supplied by Superior hypophyseal artrey
- However, when there is a pituitary tumor- the blood supply comes from a inferior hypophyseal artrey
- The later blood vessel is more vulnerable to pressure changes
Q. Enlist the Clinical features of pituitary apoplexy ?
- Headache
- Visual disorder
- Loss of consciousness
- Cranial nerve involvement- 3,4,6 , V1
- Focal neurological deficiet- due to internal carotid involvement in cavernous sinus
- Endocrine dysfunction
Q. What is the typical headache seen in patients with pituitary apoplexy ?
- It is frontal and retriorbital in nature
- It is often severe and described as "thunderclap" or "worst ever" headache.
- This headache is frequently associated with nausea and vomiting.
Q. What are the features of 3rd nerve palsy ?
- Ptosis
- Loss of light and accomodation reflex
- Eyeball is downward and outward
- Pupil- mid-dilated
Q. Can it produce facial nerve palsy ?
- Yes
Q. What are the points in pituitary apoplexy score ?
- Consciousness
  - Normal – GCS- 15 - 0
  - Mild – 8-14 - 2
  - Severe <8 – 4
- Visual acuity
  - Normal- 0
  - Unilateral – 1
  - Bilateral -2
- Ocular paresis
  - Absent- 0
  - Present – unilateral – 1
  - Bilateral- 2
- Visual field defect
  - Absent- 0
  - Unilateral- 1
  - Bilateral – 2
Q. What is the use of this score ?
- Score is useful for conservatively managed patients
- Score of >/= 4 or increasing score would suggest need for surgical intervention
Q. Can DI occur in pituitary apoplexy ?
- Yes
Q . In case of suspected apoplexy- what is done first- CT or MRI ?
- CT is done first as MRI cannot diagnose fresh blood early
- MRI is more useful in subacute setting (4 day to 1 month)
Q. How will blood appear in CT scan in various time frames ?
- Acute 0-10 days - Hyperdense- 60-80 HU
- Subacute- 10-20 days- isodense- 40 HU
- Chronic > 20 days- hypodense- 10 HU
Q. What about MRI?
- In T1
  - Acute- <24 hrs - Isointense
  - Subacute- 3-5 days - Hyperintense
  - Chronic > 3 weeks – hypointense
Q. What suggest hemorraghe and necrosis in subacute MRI ?
- In T1-
  - Hyperintense- hemorrhage
  - Hypointense- necrosis
Q. What is the importance of knowing the timing of origin of symptoms ?
- To determine the best imaging approach
Q. Which imaging must be done when the patient presents with symptoms within 6 hrs or origin ?
- CT scan
Q. What do you see in this picture ?
- So what you see here is that the CT scan is able to show a hemorrhage in the pituitary while the MRI of the same patient done within the same time frame is missing the bleed
Q. Enlist the DD of pituitary apoplexy ?
- Subarachnoid hemorrhage
- Infectious meningitis
- Migraine
- Rathke's cyst hemorrhage
- Cavernous sinus thrombosis
Management
Q. Give the management guidelines for pituitary apoplexy ?
- Step 1.1 – Assessment
  - 1. Note time of onset of symptoms
    - a. if <4 days- go for CT scan
    - b. >4 days- go for MRI sella directly
- 1. Note the following features on clinical examination
  - a. Consciousness- GCS
  - b. Visual acuity
  - c. Visual field
  - d. Ocular paresis
- 1. Calculate the Pituitary symptom score
- Step 1.2- Cortisol management
  - 1. Collect sample for Cortisol
  - 1. Start empirical corticosteroid if following conditions met
    - a. Loss of consciousness
    - b. Hemodynamically unstable
    - c. Reduced visual acuity
    - d. Severe visual field defect
    - e. Lower cortisol documented previously
    - f. Patient already on glucocorticoid replacement
- 1. Hydrocortisone
  - a. 100 mg IV bolus followed by 50 mg IV /6 hourly OR
  - b. 50 mg in 50 ml of Normal saline @4 ml/hr (4mg/hr infusion) – continuous
- 1. If condition for empirical hydrocortisone not met
  - a. Send 8:00 am Cortisol next morning
  - b. If cortisol <20 ug/dl – Start Hydrocortisone
- Step 2- Conservative vs Surgical management
  - Surgical management is considered in following conditions:
    - 1. Loss of consciousness
    - 1. Severe loss of visual acuity
    - 1. Severe visual field defects
    - 1. Pituitary symptom score >/= 4
  - Timing of surgery- must be done within 1 week
- Step 3.1 Conservative management
  - 1. Daily visual field and visual acuity monitoring
  - 1. Hemodynamic monitoring and neurological status monitoring
- Step 3.1- Surgical management
  - Transsphenoidal –preferred approach
- Step 4- Post operative management
  - 1. Continue glucocorticoids post operative
  - 1. Post op day 3- w/h hydrocortisone dose of afternoon and evening and cortisol next day morning 8:00 am
    - a. Cortisol >20 ug/dl- discontinue Gc
    - b. Cortisol 14-20 ug/dl- Gc only during stress
    - c. Cortisol <14 ug/dl- Gc required
  - 1. Thyroid function on day 3- if low replace thyroid
  - 1. Visual assessment 48 hours after surgery bedside- reassess with perimetry later date
- Step 5- Long term post operative management
  - 1. 6 weeks post op- Reassess anterior pituitary function
  - 1. MRI- 3 months after surgery – then annually for 5 years
Q. How common is secondary adrenal insufficiency in patients with pituitary apoplexy ?
- It is very common
- Found in about 70% of cases
Q. What are the signs of secondary adrenal insufficiency ?
- Hyponatremia
- Hypoglycemia
- Hypotension
Q. When surgery is considered , how are the outcome of various complications ?
- 1. Endocrine abnormalities- recover in 2 weeks
- 1. Visual field defect- 8 weeks
- 1. Cranial nerve abnormality- 2 months
Q. What is the importance of preoperative prolactin levels in predicting recovery of endocrine function ?
- Preoperative prolactin levels have impact on the recover of the endocrine function
  - Preoperative prolactin >8.8 ng/ml- predicts higher likelihood of recovery of the endocrine function post-operatively
Q. Give me the difference in outcome in early surgery versus late surgery ?
- Improvement in Visual Deficits:
  - Early Surgery: A substantial improvement in visual deficits, ranging from 57% to 95%.
  - Late Surgery: No specific data provided, but studies indicate no significant difference in visual outcomes between early and late surgery.
- Recovery of Cranial Nerve Palsies:
  - Early Surgery: Improvement in cranial nerve palsies observed in 63% to 100% of cases.
  - Late Surgery: No specific data provided, but similar to visual deficits, no significant difference reported in cranial neuropathy recovery between early and late intervention.
- Pituitary Function Recovery:
  - Early Surgery: Lower proportion of patients experience pituitary function improvement, ranging from 19% to 57%.
  - Late Surgery: No specific data on pituitary function recovery, but studies suggest no significant difference in pituitary hormone recovery between early and delayed surgery.
- Recovery of Visual Acuity:
  - Early Surgery: Improvement in visual acuity measured by logMAR improved after surgery.
  - Late Surgery: No clear association between visual acuity improvement and time between symptom onset and surgery.
- Timing of Surgery and Visual Field Deficits:
  - Early Surgery: A higher rate of improvement in visual field deficits when surgery is performed within 7 days of symptom onset.
  - Late Surgery: A lower rate of improvement in visual field deficits when surgery is delayed beyond 7 days.
- General Considerations:
  - Early Surgery: Often considered in cases with substantial visual deficits, but only after addressing electrolyte and hemodynamic abnormalities.
  - Late Surgery: Selection bias in treatment decisions for PA; outcomes must be interpreted with caution.
- It's important to note that the data varies across different studies, and the decision on the timing of surgery should be based on individual patient circumstances and clinical judgment.
Q. Give the summary of data on surgery versus conservative management ?
- The summary of data on surgery versus conservative management for Pituitary Apoplexy (PA) includes:
  - Surgical Decompression:
    - Historically, surgical decompression was the recommended treatment for patients with PA.
    - Surgery provides rapid relief from headache and other symptoms.
    - A substantial proportion of patients show improvement in visual deficits and cranial nerve palsies post-surgery.
    - Pituitary function recovery varies, with a lower proportion of patients experiencing improvement.
  - Conservative Management:
    - Recent retrospective studies show similar outcomes with conservative management compared to surgical intervention.
    - Conservative management may be feasible in highly selected clinical scenarios, particularly with mild visual deficits and improving symptoms.
    - Involves stabilization of the patient, hormonal replacement, and reversal of electrolyte abnormalities.
  - Comparative Outcomes:
    - Several meta-analyses and studies indicate notable heterogeneity in outcomes between surgical and conservative approaches.
    - No definitive evidence suggests superiority of one approach over the other in all cases.
  - Factors Influencing Decision:
    - The choice between surgery and conservative management depends on individual patient factors, including the severity of symptoms and the presence of complications.
    - Early or emergent surgical intervention is often considered for patients with substantial visual deficits, post addressing electrolyte and hemodynamic abnormalities.
  - Limitations in Data:
    - Due to the rarity of the condition and the acuity of the clinical scenarios, further studies are needed to better stratify patients for appropriate treatment options.
- In summary, while surgical intervention has historically been the standard treatment for PA, recent evidence suggests that conservative management can yield similar outcomes in certain cases. The choice of treatment should be personalized based on individual patient characteristics and the clinical scenario.
Q. What percentage of patient has complete regression after conservative management ?]
- Complete regression is seen in 28% of case sand the mean time of regression is 18 months
Q. What percentage of patients have progression or recurrence ?
- 20%
Q.Does the recurrence or progression depend on whether surgery was done or conservative management was done ?
- No

References:

Revisiting Pituitary Apoplexy: Donegan D, Erickson D. Revisiting pituitary apoplexy. Journal of the Endocrine Society. 2022 Sep 1;6(9):bvac113.