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==Overview== | ==Overview== | ||
Pituitary apoplexy is caused by [[hemorrhage]] into the [[pituitary gland]]. The [[pituitary gland]] is a small [[gland]] joined to the [[hypothalamus]] at the base of [[brain]]. The [[Pituitary gland|pituitary]] produces many of the [[hormone]]s that control essential body processes. Pituitary apoplexy is most commonly associated with [[pituitary adenoma]]. The common symptoms of pituitary apoplexy are severe [[headache]] with [[nausea and vomiting]]. Other [[symptoms]] depends upon the amount of [[hemorrhage]] and [[necrosis]] in the [[pituitary gland]]. [[Hemorrhage]] into the [[pituitary gland]] may compress the surrounding structures and present with ophthlamoplegia, [[cranial nerve palsies]], and signs of [[increased intracranial pressure]]. The initial [[diagnostic]] test is a [[CT scan]] without [[Contrast medium|contrast]] which will show the [[hemorrhage]] as a hyperintense lesion. In the case of inconclusive [[CT]], an [[MRI]] may be done to better visualize the lesion. Laboratory tests are done to identify specific [[hormone]] and [[electrolyte disturbances]]. The initial management of pituitary apoplexy includes rapid [[Hemodynamically|hemodynamic]] stabilization of the patient with replacement of [[hormones]]. Depending upon the patient's condition after initial management, [[neurological]] decompression may be done.<ref name="SempleWebb2005">{{cite journal|last1=Semple|first1=Patrick L.|last2=Webb|first2=Michael K.|last3=de Villiers|first3=Jacques C.|last4=Laws|first4=Edward R.|title=Pituitary Apoplexy|journal=Neurosurgery|volume=56|issue=1|year=2005|pages=65–73|issn=0148-396X|doi=10.1227/01.NEU.0000144840.55247.38}}</ref><ref name="pmid15531524">{{cite journal| author=Zayour DH, Selman WR, Arafah BM| title=Extreme elevation of intrasellar pressure in patients with pituitary tumor apoplexy: relation to pituitary function. | journal=J Clin Endocrinol Metab | year= 2004 | volume= 89 | issue= 11 | pages= 5649-54 | pmid=15531524 | doi=10.1210/jc.2004-0884 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15531524 }}</ref> | |||
==Historical Perspective== | ==Historical Perspective== | ||
Pituitary apoplexy was first discovered by Pearce Bailey in the year 1898. In 1905, Leopold Bleibtreu recorded the [[postmortem]] examination of a 21-year-old [[Acromegaly|acromegalic]] patient, in whom he discovered that the [[pituitary gland]] had been replaced by an old [[hemorrhage]]. The term "pituitary apoplexy" was coined by Brougham, Heusner, and Adams in the year 1950.<ref name="pmid14774761">{{cite journal |vauthors=BROUGHAM M, HEUSNER AP, ADAMS RD |title=Acute degenerative changes in adenomas of the pituitary body--with special reference to pituitary apoplexy |journal=J. Neurosurg. |volume=7 |issue=5 |pages=421–39 |year=1950 |pmid=14774761 |doi=10.3171/jns.1950.7.5.0421 |url=}}</ref> | |||
==Pathophysiology== | ==Pathophysiology== | ||
Pituitary apoplexy is an acute [[clinical]] [[syndrome]] caused by [[hemorrhage]] and [[necrosis]] in the [[pituitary gland]]. Most commonly, pituitary apoplexy is associated with [[pituitary adenoma]]. The [[pituitary adenoma]] predisposes the patient to an increased risk of [[bleeding]] within the [[pituitary gland]]. The [[pituitary adenoma]] has [[Fenestrated membrane|fenestrated]] [[endothelium]] surrounded by a variable number of [[smooth muscle cells]], which are not found in the normal [[pituitary gland]].Gene involved in the [[pathogenesis]] of pituitary apoplexy include a [[mutation]] in AIP gene, which is located on [[chromosome]] 11q13.2. On gross pathology, pituitary apoplexy presents with [[hemorrhage]] with or without [[necrosis]]. Electron microscopic shows evidence of abnormal [[fenestration]] of tumor vessels ([[pituitary adenoma]]) with fragmented [[basal membrane]]<nowiki/>s that may predispose the patient to [[hemorrhage]]. | |||
==Differentiating Pituitary apoplexy From Other Diseases== | ==Differentiating Pituitary apoplexy From Other Diseases== | ||
Pituitary apoplexy must be differentiated from other [[diseases]] that cause severe [[headache]] such as [[subarachnoid hemorrhage]], [[meningitis]], [[intracranial mass]], [[cerebral hemorrhage]], [[cerebral infarction]], [[intracranial venous thrombosis]], [[migraine]], [[head injury]], [[lymphocytic hypophysitis]], and [[radiation injury]].<ref name="pmid28575459">{{cite journal |vauthors=Rapalino O, Mullins ME |title=Intracranial Infectious and Inflammatory Diseases Presenting as Neurosurgical Pathologies |journal=Neurosurgery |volume= |issue= |pages= |year=2017 |pmid=28575459 |doi=10.1093/neuros/nyx201 |url=}}</ref><ref name="pmid28400981">{{cite journal |vauthors=Konakondla S, Schirmer CM, Li F, Geng X, Ding Y |title=New Developments in the Pathophysiology, Workup, and Diagnosis of Dural Venous Sinus Thrombosis (DVST) and a Systematic Review of Endovascular Treatments |journal=Aging Dis |volume=8 |issue=2 |pages=136–148 |year=2017 |pmid=28400981 |pmc=5362174 |doi=10.14336/AD.2016.0915 |url=}}</ref><ref name="pmid28653369">{{cite journal |vauthors=Yadav P, Bradley AL, Smith JH |title=Recognition of Chronic Migraine by Medicine Trainees: A Cross-Sectional Survey |journal=Headache |volume= |issue= |pages= |year=2017 |pmid=28653369 |doi=10.1111/head.13133 |url=}}</ref> | |||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
The worldwide [[prevalence]] of | The worldwide [[prevalence]] of pituitary apoplexy is 6.2 per 100,000 persons. The [[incidence]] of pituitary apoplexy is 0.7 per 100,000 persons.<ref name="pmid19650784">{{cite journal| author=Fernandez A, Karavitaki N, Wass JA| title=Prevalence of pituitary adenomas: a community-based, cross-sectional study in Banbury (Oxfordshire, UK). | journal=Clin Endocrinol (Oxf) | year= 2010 | volume= 72 | issue= 3 | pages= 377-82 | pmid=19650784 | doi=10.1111/j.1365-2265.2009.03667.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19650784 }} </ref> | ||
==Risk Factors== | ==Risk Factors== | ||
Common [[risk factors]] in the development of | Common [[risk factors]] in the development of pituitary apoplexy include [[bleeding disorders]], [[diabetes]], use of a breathing machine, [[Radiation therapy|radiation]] to the [[pituitary gland]], [[angiography]], [[head injury]], [[surgery]], [[Pituitary gland|pituitary]] stimulation, and [[pregnancy]] induced lactotroph hyperplasia.<ref name="pmid26414232">{{cite journal |vauthors=Briet C, Salenave S, Bonneville JF, Laws ER, Chanson P |title=Pituitary Apoplexy |journal=Endocr. Rev. |volume=36 |issue=6 |pages=622–45 |year=2015 |pmid=26414232 |doi=10.1210/er.2015-1042 |url=}}</ref> | ||
==Screening== | ==Screening== | ||
There are no [[Screening (medicine)|screening]] guidelines for | There are no [[Screening (medicine)|screening]] guidelines for pituitary apoplexy. | ||
==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
If left untreated, | If left untreated, pituitary apoplexy is an [[Acute (medicine)|acute]] life threatening condition. Pituitary apoplexy may lead to a sudden decline in [[pituitary hormone]] production. The most life threatening [[endocrinopathy]] is acute [[adrenal crisis]]. Complications of pituitary apoplexy include [[vision loss]], [[optic neuritis]], [[diplopia]], [[ptosis]], [[increased intracranial pressure]], [[hypothyroidism]], [[hypogonadism]], and [[growth hormone deficiency]]. The [[prognosis]] of pituitary apoplexy depends upon presentation and initiation of therapy. Emergent application of [[medical]] and surgical treatment is associated with greater improvement in [[Visual field defect|visual field defects]], [[visual acuity]], and [[diplopia]]. The outlook is good for people who are diagnosed early and treated. Patients require [[hormone]](s) replacement therapy for life.<ref name="pmid21082047">{{cite journal |vauthors=Woo HJ, Hwang JH, Hwang SK, Park YM |title=Clinical outcome of cranial neuropathy in patients with pituitary apoplexy |journal=J Korean Neurosurg Soc |volume=48 |issue=3 |pages=213–8 |year=2010 |pmid=21082047 |pmc=2966721 |doi=10.3340/jkns.2010.48.3.213 |url=}}</ref> | ||
==Diagnosis== | ==Diagnosis== | ||
===History and Symptoms=== | ===History and Symptoms=== | ||
Pituitary apoplexy usually has a short period of symptoms ([[Acute (medicine)|acute]]). [[Symptoms]] usually include severe [[headache]], [[paralysis]] of eye muscles, visual disturbances, [[Nausea and vomiting|nausea, and vomiting]].<ref name="pmid28437813">{{cite journal |vauthors=Pyrgelis ES, Mavridis I, Meliou M |title=Presenting Symptoms of Pituitary Apoplexy |journal=J Neurol Surg A Cent Eur Neurosurg |volume= |issue= |pages= |year=2017 |pmid=28437813 |doi=10.1055/s-0037-1599051 |url=}}</ref> | |||
===Physical Examination=== | ===Physical Examination=== | ||
Patients with | Patients with pituitary apoplexy appear ill and usually look [[tired]]. [[Physical examination]] of patients with pituitary apoplexy is usually remarkable for [[orthostatic hypotension]], [[visual acuity]] and [[Visual field defect|visual field defects]], [[cranial nerve palsies]], H[[horner syndrome|orner syndrome]], [[meningeal irritation]], altered level of consciousness, severe [[mental status]] change, and other [[signs]] of [[hypopituitarism]]. | ||
===Laboratory Findings=== | ===Laboratory Findings=== | ||
Laboratory findings consistent with the diagnosis of pituitary apoplexy include endocrinopathies from hypofunction of the [[pituitary gland]]. [[Blood tests]] will be done to check levels of [[ACTH]], [[cholesterol]], [[cortisol]] ,[[growth hormone]], [[LH]], [[prolactin]] and somatomedin C [[IGF-1|(IGF-1]]). | Laboratory findings consistent with the diagnosis of pituitary apoplexy include endocrinopathies from hypofunction of the [[pituitary gland]]. [[Blood tests]] will be done to check levels of [[ACTH]], [[cholesterol]], [[cortisol]], [[growth hormone]], [[LH]], [[prolactin]] and somatomedin C [[IGF-1|(IGF-1]]). | ||
===X-ray=== | ===X-ray=== | ||
[[X-ray]] is an inexpensive method for evaluating pituitary apoplexy. However, [[x-ray]] is neither the best initial test nor the most accurate test in evaluating pituitary apoplexy. | [[X-ray]] is an inexpensive method for evaluating pituitary apoplexy. However, [[x-ray]] is neither the best initial test, nor the most accurate test in evaluating pituitary apoplexy. | ||
===CT=== | ===CT=== | ||
[[CT]] scan without [[Contrast medium|contrast]] is the initial test of choice in [[emergency department]] patients who | [[CT]] scan without [[Contrast medium|contrast]] is the initial test of choice in [[emergency department]] patients who present with sudden-onset severe [[headache]], [[Vision loss|visual loss]] or [[ophthalmoplegia]] suggestive of pituitary apoplexy. A [[CT]] scan can also help to differentiate whether [[subarachnoid hemorrhage]] is arising from pituitary [[hemorrhage]] or an [[aneurysm]]. | ||
===MRI=== | ===MRI=== | ||
[[MRI]] is done if the [[CT-scans|CT scan]] is suspicious for pituitary apoplexy. [[MRI]] is more sensitive than [[CT-scans|CT scan]]. [[MRI]] is more accurate in distinguishing the soft tissues of the [[Pituitary gland|pituitary]] from the surrounding bony structures. [[MRI]] is also superior to [[CT]] scan for detecting [[ischemia]] and [[infarction]] in brain tissue. | [[MRI]] is done if the [[CT-scans|CT scan]] is suspicious for pituitary apoplexy. [[MRI|MRI]] is more sensitive than [[CT-scans|CT scan]]. [[MRI|MRI]] is more accurate in distinguishing the soft tissues of the [[Pituitary gland|pituitary]] from the surrounding bony structures. [[MRI|MRI]] is also superior to [[CT]] scan for detecting [[ischemia]] and [[infarction]] in brain tissue. | ||
===Ultrasound=== | ===Ultrasound=== | ||
There are no ultrasound findings associated with pituitary apoplexy. | There are no ultrasound findings associated with pituitary apoplexy. | ||
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==Treatment== | ==Treatment== | ||
The optimal therapy for | The optimal therapy for pituitary apoplexy depends upon presentation of the patient. The emphasis is on early [[hemodynamic]] stabilization of the patient, with evaluation for signs of deficiency of pituitary [[hormones]]. Life threatening [[hypopituitarism]] must be treated with replacement of [[hormones]]. | ||
===Surgery=== | ===Surgery=== | ||
[[Neurological]] decompression is done once the patient is [[hemodynamically]] stable. [[Surgery]] relieves [[pressure]] on the [[Pituitary gland|pituitary]] and improves [[Visual field defect|visual field defects]] and ocular palsy. Early decompression has been associated with better [[visual]] and [[endocrine]] outcome. | [[Neurological]] decompression is done once the patient is [[hemodynamically]] stable. [[Surgery]] relieves [[pressure]] on the [[Pituitary gland|pituitary]] and improves [[Visual field defect|visual field defects]] and ocular palsy. Early decompression has been associated with better [[visual]] and [[endocrine]] outcome. | ||
Latest revision as of 07:45, 30 July 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Akshun Kalia M.B.B.S.[2]
Overview
Pituitary apoplexy is caused by hemorrhage into the pituitary gland. The pituitary gland is a small gland joined to the hypothalamus at the base of brain. The pituitary produces many of the hormones that control essential body processes. Pituitary apoplexy is most commonly associated with pituitary adenoma. The common symptoms of pituitary apoplexy are severe headache with nausea and vomiting. Other symptoms depends upon the amount of hemorrhage and necrosis in the pituitary gland. Hemorrhage into the pituitary gland may compress the surrounding structures and present with ophthlamoplegia, cranial nerve palsies, and signs of increased intracranial pressure. The initial diagnostic test is a CT scan without contrast which will show the hemorrhage as a hyperintense lesion. In the case of inconclusive CT, an MRI may be done to better visualize the lesion. Laboratory tests are done to identify specific hormone and electrolyte disturbances. The initial management of pituitary apoplexy includes rapid hemodynamic stabilization of the patient with replacement of hormones. Depending upon the patient's condition after initial management, neurological decompression may be done.[1][2]
Historical Perspective
Pituitary apoplexy was first discovered by Pearce Bailey in the year 1898. In 1905, Leopold Bleibtreu recorded the postmortem examination of a 21-year-old acromegalic patient, in whom he discovered that the pituitary gland had been replaced by an old hemorrhage. The term "pituitary apoplexy" was coined by Brougham, Heusner, and Adams in the year 1950.[3]
Pathophysiology
Pituitary apoplexy is an acute clinical syndrome caused by hemorrhage and necrosis in the pituitary gland. Most commonly, pituitary apoplexy is associated with pituitary adenoma. The pituitary adenoma predisposes the patient to an increased risk of bleeding within the pituitary gland. The pituitary adenoma has fenestrated endothelium surrounded by a variable number of smooth muscle cells, which are not found in the normal pituitary gland.Gene involved in the pathogenesis of pituitary apoplexy include a mutation in AIP gene, which is located on chromosome 11q13.2. On gross pathology, pituitary apoplexy presents with hemorrhage with or without necrosis. Electron microscopic shows evidence of abnormal fenestration of tumor vessels (pituitary adenoma) with fragmented basal membranes that may predispose the patient to hemorrhage.
Differentiating Pituitary apoplexy From Other Diseases
Pituitary apoplexy must be differentiated from other diseases that cause severe headache such as subarachnoid hemorrhage, meningitis, intracranial mass, cerebral hemorrhage, cerebral infarction, intracranial venous thrombosis, migraine, head injury, lymphocytic hypophysitis, and radiation injury.[4][5][6]
Epidemiology and Demographics
The worldwide prevalence of pituitary apoplexy is 6.2 per 100,000 persons. The incidence of pituitary apoplexy is 0.7 per 100,000 persons.[7]
Risk Factors
Common risk factors in the development of pituitary apoplexy include bleeding disorders, diabetes, use of a breathing machine, radiation to the pituitary gland, angiography, head injury, surgery, pituitary stimulation, and pregnancy induced lactotroph hyperplasia.[8]
Screening
There are no screening guidelines for pituitary apoplexy.
Natural History, Complications, and Prognosis
If left untreated, pituitary apoplexy is an acute life threatening condition. Pituitary apoplexy may lead to a sudden decline in pituitary hormone production. The most life threatening endocrinopathy is acute adrenal crisis. Complications of pituitary apoplexy include vision loss, optic neuritis, diplopia, ptosis, increased intracranial pressure, hypothyroidism, hypogonadism, and growth hormone deficiency. The prognosis of pituitary apoplexy depends upon presentation and initiation of therapy. Emergent application of medical and surgical treatment is associated with greater improvement in visual field defects, visual acuity, and diplopia. The outlook is good for people who are diagnosed early and treated. Patients require hormone(s) replacement therapy for life.[9]
Diagnosis
History and Symptoms
Pituitary apoplexy usually has a short period of symptoms (acute). Symptoms usually include severe headache, paralysis of eye muscles, visual disturbances, nausea, and vomiting.[10]
Physical Examination
Patients with pituitary apoplexy appear ill and usually look tired. Physical examination of patients with pituitary apoplexy is usually remarkable for orthostatic hypotension, visual acuity and visual field defects, cranial nerve palsies, Horner syndrome, meningeal irritation, altered level of consciousness, severe mental status change, and other signs of hypopituitarism.
Laboratory Findings
Laboratory findings consistent with the diagnosis of pituitary apoplexy include endocrinopathies from hypofunction of the pituitary gland. Blood tests will be done to check levels of ACTH, cholesterol, cortisol, growth hormone, LH, prolactin and somatomedin C (IGF-1).
X-ray
X-ray is an inexpensive method for evaluating pituitary apoplexy. However, x-ray is neither the best initial test, nor the most accurate test in evaluating pituitary apoplexy.
CT
CT scan without contrast is the initial test of choice in emergency department patients who present with sudden-onset severe headache, visual loss or ophthalmoplegia suggestive of pituitary apoplexy. A CT scan can also help to differentiate whether subarachnoid hemorrhage is arising from pituitary hemorrhage or an aneurysm.
MRI
MRI is done if the CT scan is suspicious for pituitary apoplexy. MRI is more sensitive than CT scan. MRI is more accurate in distinguishing the soft tissues of the pituitary from the surrounding bony structures. MRI is also superior to CT scan for detecting ischemia and infarction in brain tissue.
Ultrasound
There are no ultrasound findings associated with pituitary apoplexy.
Other Imaging Findings
There are no other imaging findings associated with pituitary apoplexy.
Other Diagnostic Studies
There are no other diagnostic studies associated with pituitary apoplexy.
Treatment
The optimal therapy for pituitary apoplexy depends upon presentation of the patient. The emphasis is on early hemodynamic stabilization of the patient, with evaluation for signs of deficiency of pituitary hormones. Life threatening hypopituitarism must be treated with replacement of hormones.
Surgery
Neurological decompression is done once the patient is hemodynamically stable. Surgery relieves pressure on the pituitary and improves visual field defects and ocular palsy. Early decompression has been associated with better visual and endocrine outcome.
References
- ↑ Semple, Patrick L.; Webb, Michael K.; de Villiers, Jacques C.; Laws, Edward R. (2005). "Pituitary Apoplexy". Neurosurgery. 56 (1): 65–73. doi:10.1227/01.NEU.0000144840.55247.38. ISSN 0148-396X.
- ↑ Zayour DH, Selman WR, Arafah BM (2004). "Extreme elevation of intrasellar pressure in patients with pituitary tumor apoplexy: relation to pituitary function". J Clin Endocrinol Metab. 89 (11): 5649–54. doi:10.1210/jc.2004-0884. PMID 15531524.
- ↑ BROUGHAM M, HEUSNER AP, ADAMS RD (1950). "Acute degenerative changes in adenomas of the pituitary body--with special reference to pituitary apoplexy". J. Neurosurg. 7 (5): 421–39. doi:10.3171/jns.1950.7.5.0421. PMID 14774761.
- ↑ Rapalino O, Mullins ME (2017). "Intracranial Infectious and Inflammatory Diseases Presenting as Neurosurgical Pathologies". Neurosurgery. doi:10.1093/neuros/nyx201. PMID 28575459.
- ↑ Konakondla S, Schirmer CM, Li F, Geng X, Ding Y (2017). "New Developments in the Pathophysiology, Workup, and Diagnosis of Dural Venous Sinus Thrombosis (DVST) and a Systematic Review of Endovascular Treatments". Aging Dis. 8 (2): 136–148. doi:10.14336/AD.2016.0915. PMC 5362174. PMID 28400981.
- ↑ Yadav P, Bradley AL, Smith JH (2017). "Recognition of Chronic Migraine by Medicine Trainees: A Cross-Sectional Survey". Headache. doi:10.1111/head.13133. PMID 28653369.
- ↑ Fernandez A, Karavitaki N, Wass JA (2010). "Prevalence of pituitary adenomas: a community-based, cross-sectional study in Banbury (Oxfordshire, UK)". Clin Endocrinol (Oxf). 72 (3): 377–82. doi:10.1111/j.1365-2265.2009.03667.x. PMID 19650784.
- ↑ Briet C, Salenave S, Bonneville JF, Laws ER, Chanson P (2015). "Pituitary Apoplexy". Endocr. Rev. 36 (6): 622–45. doi:10.1210/er.2015-1042. PMID 26414232.
- ↑ Woo HJ, Hwang JH, Hwang SK, Park YM (2010). "Clinical outcome of cranial neuropathy in patients with pituitary apoplexy". J Korean Neurosurg Soc. 48 (3): 213–8. doi:10.3340/jkns.2010.48.3.213. PMC 2966721. PMID 21082047.
- ↑ Pyrgelis ES, Mavridis I, Meliou M (2017). "Presenting Symptoms of Pituitary Apoplexy". J Neurol Surg A Cent Eur Neurosurg. doi:10.1055/s-0037-1599051. PMID 28437813.