review pituitary apoplexy...pathophysiology of pituitary apoplexy 5 hemorrhage 1.suri, h.,...
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1
Date:2020.2.27
Speaker:R1 An Che, Cheng
Supervisor: VS Chih Jen, Cheng
Review
Pituitary Apoplexy
Introduction
2
Pituitary apoplexy is a clinical syndrome, characterized by
sudden onset of headache, visual impairment and
decreased consciousness caused by abrupt hemorrhage
and/or infarction of the pituitary gland, generally within a
pituitary adenoma
Rajasekaran S, Vanderpump M, Baldeweg S, et al. UK guidelines for the management of pituitary apoplexy. Clin Endocrinol (Oxf). 2011;74:9–20. .
Hormone Secretion of Pituitary Gland
3 Modified from The Netter Collection of Medical Illustrations: Endocrine System Vol.2
TSH
ACTH
LH
FSH
Prolactin
GH
Oxytocin
ADH
Posterior pituitary gland Anterior pituitary gland
Hypothalamus
Anatomy of Pituitary Gland
4 Modified from Netter, Netter’s Atlas of Human Anatomy
III cranial nerve
Optic chiasm Pituitary gland
IV cranial nerve
V1 cranial nerve
V2 cranial nerve
VI cranial nerve
Internal carotid artery
Sphenoidal sinus
Pathophysiology of Pituitary Apoplexy
5
Hemorrhage
1.Suri, H., Dougherty, C. Presentation and Management of Headache in Pituitary Apoplexy. Curr Pain Headache Rep 23, 61 (2019)
2.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45..
Precipitating
factors
Reduced
perfusion
Increased
intrasellar pressure
Infarction
Vasculopathy
Tumor outgrowth of
blood supply
Pathophysiology of Pituitary Apoplexy
Sudden increased
intrasellar pressure
Increased pressure on
adjacent vascular and
neural structure
Neural palsy
(CN.III, IV, V, VI)
1.Suri, H., Dougherty, C. Presentation and Management of Headache in Pituitary Apoplexy. Curr Pain Headache Rep 23, 61 (2019)
2.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45..
Pathophysiology of Pituitary Apoplexy
Sudden increased
intrasellar pressure
Increased pressure on
adjacent vascular and
neural structure
Pressure transmitted on
brain stem/hypothalamus Consciousness
impairment
Neural palsy
(CN.III, IV, V, VI)
1.Suri, H., Dougherty, C. Presentation and Management of Headache in Pituitary Apoplexy. Curr Pain Headache Rep 23, 61 (2019)
2.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45..
Pathophysiology of Pituitary Apoplexy
Sudden increased
intrasellar pressure
Increased pressure on
adjacent vascular and
neural structure
Pressure transmitted on
brain stem/hypothalamus
Optic chiasm
compression
Visual
Defect
Consciousness
impairment
Neural palsy
(CN.III, IV, V, VI)
1.Suri, H., Dougherty, C. Presentation and Management of Headache in Pituitary Apoplexy. Curr Pain Headache Rep 23, 61 (2019)
2.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45..
Pathophysiology of Pituitary Apoplexy
Sudden increased
intrasellar pressure
Compression of
normal pituitary gland
Increased pressure on
adjacent vascular and
neural structure
Hypopituitarism
Pressure transmitted on
brain stem/hypothalamus
Optic chiasm
compression
Visual
Defect
Consciousness
impairment
Neural palsy
(CN.III, IV, V, VI)
1.Suri, H., Dougherty, C. Presentation and Management of Headache in Pituitary Apoplexy. Curr Pain Headache Rep 23, 61 (2019)
2.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45..
Epidemiology
10
1.Raappana A, Koivukangas J, Ebeling T, Pirilä T. Incidence of pituitary adenomas in Northern Finland in 1992–2007. J Clin Endocrinol Metab.
2010;95:4268–4275
2.FernandezAKaravitakiNWassJA. Prevalence of pituitary adenomas: a community-based, cross-sectional study in Banbury (Oxfordshire, UK).
Clinical Endocrinology 201072377–382.
More than 80% have an underlying pituitary adenoma, but it
can occur in non-adenomatous lesions, such as sellar
tuberculoma, and sellar metastasis, or normal pituitary gland
Prevalence is about 6.2 cases/100 000 population
Incidence is about 0.17 episodes/100,000 person-years
Most frequent in the fifth or sixth decade
Male : Female is about 2:1
Precipitating Factors
11
Systemic hypertension (26%) Dopamine agonists
Anticoagulation therapy (25%) Radiation therapy
Major surgery, in particular Cardiac
surgery(18%)
Dengue haemorrhagic fever
Pituitary function tests with GnRH,
TRH and CRH
Coagulopathies
Pregnancy and Delivery Cerebral angiopathy
Estrogen therapy
Head trauma
Precipitating factors have been found in 40% of cases The majority of cases occur spontaneously
1.Rajasekaran S, Vanderpump M, Baldeweg S, et al. UK guidelines for the management of pituitary apoplexy. Clin Endocrinol (Oxf). 2011;74:9–20
2.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45... .
Clinical Presentation
Clinical manifestation Frequency
Headache >90%
Nausea and vomiting 43~80%
Visual impairment
Visual fields defects or decreased visual acuity 36~71%
Diplopia >50%
Altered mental state 13–42%
Hypopituitarism
ACTH deficiency 70~76%
Gonadotrophin deficiency 76~79%
Central hypothyroidism 50~57%
Panhypopituitarism 70%
Hyponatremia 12~44%
Cristina Capatina ,Warrick Inder ,Niki Karavitaki et.al1 MANAGEMENT OF ENDOCRINE DISEASE: Pituitary tumour apoplexyin
European Journal of Endocrinology May 2015 Volume 172: Issue 5, European Society of Endocrinology
Clinical Presentation
Himanshu Suri & Carrie Dougherty. Presentation and Management of Headache in Pituitary Apoplexy; Current Pain and Headache Reports
volume 23, Article number: 61 .2019.
.
Presentations of Headache in Pituitary Apoplexy
Nearly half (45.8%) in a retrospective series of 60 patients
presented with severe acute thunderclap headache with
maximum intensity at onset.
Some presented with a gradual onset, unremitting
headache of several weeks duration.
Few cases throbbing and associated with nausea,
phonophobia, and mild photophobia, mimicking migraine
Diagnostic Evaluation
14
Patients presenting with acute severe headache with or without
neuro‐ophthalmic signs
Patients known to have a pituitary tumor
Patients known to have a pituitary tumor when performing pituitary
stimulation tests, anticoagulation therapy or undertaking coronary
artery bypass or other major surgery
Who Should be Suspected
Rajasekaran S, Vanderpump M, Baldeweg S, et al. UK guidelines for the management of pituitary apoplexy. Clin Endocrinol (Oxf). 2011;74:9–20.
Diagnostic Evaluation
15
Role of Computerized Tomography(CT)
Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45...
To rule out SAH in patients with severe, sudden onset
headace
It shows an intrasellar mass in 80% of cases, but was
diagnostic in only 21–28% of cases
In non-hemorrhage apoplexy, CT may be non- specific
CT provides better hemorrhage detection in hyperacute
stage(few hours from onset) than MRI.
Diagnostic Evaluation
16
Image Findings Non-contrast CT in Hemorrhage of Pituitary Apoplexy
Pradeep Goyal1, Michael Utz2, Nishant Gupta. Et.al Clinical and imaging features of pituitary apoplexy and role of imaging in differentiation of
clinical mimics Vol 8, No 2 March 2018
Hyperdense lesion in acute hemorrhage(few hours from onset)
Diagnostic Evaluation
17
Role of Magnetic Resonance Imaging(MRI)
1.Cristina Capatina, Warrick Inder , Niki Karavitaki et.al. MANAGEMENT OF ENDOCRINE DISEASE: Pituitary tumour apoplexy Volume 172:
Issue 5 May 2015
2.Rajasekaran S, Vanderpump M, Baldeweg S, et al. UK guidelines for the management of pituitary apoplexy. Clin Endocrinol (Oxf). 2011;74:9–20.
Confirm the diagnosis of pituitary apoplexy in over 90% of the patients
MRI can identify hemorrhagic and necrotic areas and show the relationship
between the tumor and neighboring structures
In the first few hours, hemorrhage can be missed on MRI and is better
identified with CT
Diagnostic Evaluation
18
Image Findings
Normal Pituitary Gland in MRI
Dr Mostafa El-Feky and Assoc Prof Frank Gaillard et al. Pituitary MRI . Radiopaedia. 23rd Apr 2012.
Iso-intense
Sagittal T1
Pre-contrast
Diagnostic Evaluation
19
Image findings
Normal Pituitary Gland in MRI
Dr Mostafa El-Feky and Assoc Prof Frank Gaillard et al. Pituitary MRI . Radiopaedia. 23rd Apr 2012.
Contrast enhanced
Hyper-intense
Sagittal T1
Post-contrast
Diagnostic Evaluation
20
Image findings
Normal Pituitary Gland in MRI
Dr Mostafa El-Feky and Assoc Prof Frank Gaillard et al. Pituitary MRI . Radiopaedia. 23rd Apr 2012.
Coronal T2
Hyper-intense
Diagnostic Evaluation
21
Image Findings
CT and MRI in Hemorrhage Apoplexy
Modified from Dr Mostafa El-Feky and Assoc Prof Frank Gaillard et al. Timeline diagram of MRI and CT characteristics of intracerebral hemorrhage
.Radiopaedia.. 19th Sep 2017
Hyperacute
<12 hours
Acute
12-48 hours
Subacute
>2 days
Chronic
>14 days
Isodense
Isointense
Isointense
Diagnostic Evaluation
22
Image Findings MRI of Hemorrhage Apoplexy in Acute Stage(12~48hours)
Pradeep Goyal1, Michael Utz2, Nishant Gupta. Et.al Clinical and imaging features of pituitary apoplexy and role of imaging in differentiation of
clinical mimics Vol 8, No 2 March 2018
Hyper-intense
Sagittal T1
Pre-contrast
Hypo-intense
Sagittal T1
post-contrast
Hypo-intense
Coronal T2
Diagnostic Evaluation
23
Image Findings
MRI in Non-hemorrhage Apoplexy
In acute phase, both hemorrhagic and non-hemorrhagic
apoplexy show high signal on diffusion weighted
imaging(DWI)
After intravenous contrast, the most common finding in
both hemorrhagic and non-hemorrhagic apoplexy is
peripheral rim enhancement in acute phase
Pradeep Goyal1, Michael Utz2, Nishant Gupta. Et.al Clinical and imaging features of pituitary apoplexy and role of imaging in differentiation of
clinical mimics Vol 8, No 2 March 2018
Diagnostic Evaluation
24
Image Findings MRI in Non-hemorrhage Apoplexy in Hyepracute stage
Pradeep Goyal1, Michael Utz2, Nishant Gupta. Et.al Clinical and imaging features of pituitary apoplexy and role of imaging in differentiation of
clinical mimics Vol 8, No 2 March 2018
High signal
DWI
Rim enhancement
SagittalT1
post-contrast
Iso-intense
SagittalT1
pre-contrast
Diagnostic Evaluation
25 Modified from Dr Mostafa El-Feky and Assoc Prof Frank Gaillard et al. Timeline diagram of MRI and CT characteristics of intracerebral hemorrhage
.Radiopaedia.. 19th Sep 2017
Hyperacute
<12 hours
Acute
12-48 hours
Subacute
>2 days
Chronic
>14 days
Isodense
Isointense
Isointense
Back to Our Case
Diagnostic Evaluation
26
Axial T1
Pre-contrast
Heterogonous
Hyper-intense
Back to Our Case
Diagnostic Evaluation
27
Axial T1
Post-contrast
Hypo-intense
Back to Our Case
Diagnostic Evaluation
28
Axial T2
Heterogonous
Hyper-intense
Back to Our Case
Diagnostic Evaluation
29
Differential Diagnosis
Pregnancy Hyperplasia
During pregnancy there is progressive enlargement of the pituitary gland. Patient may
present with headache with visual field deficits and can have acute hypopituitarism.
Sagittal T1
pre-contrast
Large pituitary gland
Axial T1
pre-contrast
No focal
Hyper-intense
Diagnostic Evaluation
30
Differential Diagnosis
Rathke's Cleft Cyst(RCC)
Epithelial cysts arising from remnants of the Rathke pouch in the pituitary
gland. A large sellar RCC may mimic pituitary apoplexy clinically
Coronal T2
Heterogeneously
Hypo-intense
Coronal CT
Sellar mass with
fluid debris
level
Management
31
Supportive measures to ensure hemodynamic stability
Empiric corticosteroids replacement
Conservative treatment
Surgical intervention
Best approach is controversial
Therapeutic strategies
Rajasekaran S, Vanderpump M, Baldeweg S, et al. UK guidelines for the management of pituitary apoplexy. Clin Endocrinol (Oxf). 2011;74:9–20.
Management
32
Empiric Corticosteroids Replacement
Corticotropic deficiency is the most life-threatening complication,
potentially causing severe hemodynamic problems
Indication:
Haemodynamic instability, impaired consciousness and
visual function
Treatment:
Hydrocortisone bolus 100–200 mg followed by 50–100 mg
every 6 hours
Tapered to maintenance dose of 20–30 mg/day, orally
1.Rajasekaran S, Vanderpump M, Baldeweg S, et al. UK guidelines for the management of pituitary apoplexy. Clin Endocrinol (Oxf). 2011;74:9–20. 2.Bancos I, Hahner S, Tomlinson J, Arlt W. Diagnosis and management of adrenal insufficiency. Lancet Diabetes Endocrinol. 2015;3:216–226.
Management
33
Conservative Treatment
Some retrospective studies have revealed that in patient with stable
visual deficits or improving, a conservative approach is safe, does not
result in poor visual or endocrine outcome
Indications
Patients without any neuro‐ophthalmic signs or improving
Operative high risk
Treatment
Glucocorticoids, replacement of hormone deficits
Monitoring of visual function, keep fluids and electrolytes balance.
1.Ayuk, J., McGregor, E.J., Mitchell, R.D. et al. (2004) Acute management of pituitary apoplexy–surgery or conservative management?.
2. Gruber, A., Clayton, J., Kumar, S. et al. (2006) Pituitary apoplexy: retrospective review of 30 patients–is surgical intervention always necessary? British
Journal of Neurosurgery, 20, 379–385
3.Rajasekaran S, Vanderpump M, Baldeweg S, et al. UK guidelines for the management of pituitary apoplexy. Clin Endocrinol (Oxf). 2011;74:9–20. 4.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45...
Management
34
Surgical Intervention
Indications
Visual fields and acuity impairment
Impaired consciousness
Deteriorating neurological signs
Conservative treatment failure
Timing
Preferably within the first 7 days of onset of symptoms
Complications
CSF leakage
Damage of normal pituitary
1.Peter M., De Tribolet N. Visual outcome after transsphenoidal surgery for pituitary adenomas. British Journal of Neurosurgery. 1995;9(2):151–158
2.Rajasekaran S, Vanderpump M, Baldeweg S, et al. UK guidelines for the management of pituitary apoplexy. Clin Endocrinol (Oxf). 2011;74:9–20. 3.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45...
Management
Best approach is still controversial
1.Sibal L, Ball SG, Connolly V. et.al. Pituitary apoplexy: a review of clinical presentation, management and outcome in 45 cases. Pituitary20047157–163..
2.BujawansaS, Thondam SK, Steele C et.al. Presentation, management and outcomes in acute pituitary apoplexy: a large single-centre experience from
the United Kingdom. Clinical Endocrinology 201480419–424.
3.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45...
Available literature indicates that cases without severe, progressing
neuroophthalmic signs can safely be managed conservatively
There were selection bias to their retrospective design; the patients
in the conservative group had less severe condition than those in the
surgical group. Bearing this in mind, the endocrine and visual
outcomes are similar in operated and conservatively managed cases.
A randomized trial is needed for obtaining strong evidence.
Management
Adriana Albani, Francesco Ferraù, ,Filippo Flavio Angileri et.al Multidisciplinary Management of Pituitary Apoplexy Int J Endocrinol. 2016 Dec 15.
Suspected pituitary apoplexy
Multidisciplinary care (endocrinologist, neurosurgeon, ophthalmologist)
1. Keep hemodynamic stability, fluid electrolyte balance
2. Consider empiric steroid
3. Assessment of pituitary function
Urgent MRI(or CT if unavailable)
Assess for
Impairment of consciousness
Deterioration or visual disturbance
Surgery Conservative treatment
closely monitoring
No improvement
Management
Adriana Albani, Francesco Ferraù, ,Filippo Flavio Angileri et.al Multidisciplinary Management of Pituitary Apoplexy Int J Endocrinol. 2016 Dec 15.
Suspected pituitary apoplexy
Multidisciplinary care (endocrinologist, neurosurgeon, ophthalmologist)
1. Keep hemodynamic stability, fluid electrolyte balance
2. Consider empiric steroid
3. Assessment of pituitary function
Urgent MRI(or CT if unavailable)
Assess for
Impairment of consciousness
Deterioration or visual disturbance
Surgery Conservative treatment
closely monitoring
No improvement
Management
Adriana Albani, Francesco Ferraù, ,Filippo Flavio Angileri et.al Multidisciplinary Management of Pituitary Apoplexy Int J Endocrinol. 2016 Dec 15.
Suspected pituitary apoplexy
Multidisciplinary care (endocrinologist, neurosurgeon, ophthalmologist)
1. Keep hemodynamic stability, fluid electrolyte balance
2. Consider empiric steroid
3. Assessment of pituitary function
Urgent MRI(or CT if unavailable)
Assess for
Impairment of consciousness
Deterioration or visual disturbance
Surgery Conservative treatment
closely monitoring
No improvement
Management
Adriana Albani, Francesco Ferraù, ,Filippo Flavio Angileri et.al Multidisciplinary Management of Pituitary Apoplexy Int J Endocrinol. 2016 Dec 15.
Suspected pituitary apoplexy
Multidisciplinary care (endocrinologist, neurosurgeon, ophthalmologist)
1. Keep hemodynamic stability, fluid electrolyte balance
2. Consider empiric steroid
3. Assessment of pituitary function
Urgent MRI(or CT if unavailable)
Assess for
Impairment of consciousness
Deterioration or visual disturbance
Surgery Conservative treatment
closely monitoring
No improvement
Management
Adriana Albani, Francesco Ferraù, ,Filippo Flavio Angileri et.al Multidisciplinary Management of Pituitary Apoplexy Int J Endocrinol. 2016 Dec 15.
Suspected pituitary apoplexy
Multidisciplinary care (endocrinologist, neurosurgeon, ophthalmologist)
1. Keep hemodynamic stability, fluid electrolyte balance
2. Consider empiric steroid
3. Assessment of pituitary function
Urgent MRI(or CT if unavailable)
Assess for
Impairment of consciousness
Deterioration or visual disturbance
Surgery Conservative treatment
closely monitoring
No improvement
Outcome
41
Motality:5–15.3%
Visual disturbance
Complete or significant improvement in visual disturbance
occurs in 57–86%
Endocrine
Hypogonadism remains in 55–79%, hypothyroidism in 45–60%,
and ACTH deficiency in 60–87%
Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45..
Take Home Messages
42
Pituitary tumor apoplexy is a rare, potentially life-
threatening clinical syndrome caused by ischemic
infarction or hemorrhage, generally into a pituitary tumor
The diagnosis should be suspected in all cases with
sudden-onset severe headache, with or without
neuroophthalmic manifestations
Take Home Messages
43
CT imaging is the most common initial study during the
acute onset symptoms
MRI having better sensitivity should always be performed
in acute and subacute phase
Take Home Messages
44
Patient should be under the care of a multidisciplinary
team including endocrinologist, neurosurgeon, and
ophthalmologist
In cases with severe, progressing visual or neurological
manifestations, surgical decompression is indicated;
Patients with mild, stable clinical picture can be managed
conservatively
Take Home Messages
45
The visual and neurological outcomes are favorable in
most cases.
The endocrinological prognosis is less favorable with
many patients requiring replacement therapy and long-
term follow-up.
The optimal treatment is debate. Prospective randomized
trials are necessary
Thank you for listening
46
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