Sheehan's syndrome overview: Difference between revisions

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{{Sheehan's syndrome}}
{{Sheehan's syndrome}}
{{CMG}}; {{AE}} {{IQ}}
{{CMG}}; {{AE}}{{IQ}}


==Overview==
==Overview==
Sheehan's syndrome is a life threatening [[complication]] of severe [[Postpartum haemorrhage|postpartum hemorrhage (PPH)]] and results in mild to severe [[hypopituitarism]]. It is less prevalent in developed countries but it is still one of the most common causes of [[hypopituitarism]] in underdeveloped and developing countries. During [[pregnancy]], enlarged size of [[pituitary gland]] and decreased blood supply to it due to any cause can result in [[ischemic necrosis]] of [[pituitary gland]]. Severe [[Postpartum Hemorrhage|PPH]], [[Glandular tissue|glandular]] [[hypertrophy]] and [[hyperplasia]], smaller [[Sella turcica|sella]] size, [[autoimmunity]] and [[Disseminated Intravascular Coagulation|disseminated vascular coagulation (DIC)]] are thought to play an important role in the [[pathogenesis]] of Sheehan's syndrome. The most common causes include [[massive hemorrhage]] during [[parturition]], [[vascular]] compression and vascular [[occlusion]]. Sheehan's syndrome needs to be differentiated from [[diseases]] causing [[hypopituitarism]] and other diseases like [[lymphocytic hypophysitis]], [[pituitary apoplexy]], [[hypothyroidism]], [[addison's disease]], [[empty sella syndrome]], [[Hypogonadotrophic hypogonadism|hypogonadotropic hypogonadism]], [[Simmond's Disease|simmond's disease]], hypoprolactinemia and [[menopause]]. [[Risk factors]] causing Sheehan's syndrome include [[pregnancy]], [[Massive hemorrhage|severe/massive PPH]], pituitary mass, pre-existing [[Vascular disease|vascular diseases]], smaller and rigid [[Sella turcica|sella]], traumatic delivery, multiple [[Gestation|gestations]], [[placental]] abnormalities and [[type 1 Diabetes]]. Sheehan's syndrome, if left untreated lead to [[panhypopituitarism]] and [[empty sella syndrome]]. Common [[complications]] include [[adrenal crisis]], [[hypotension]], [[hypothyroidism]] and [[hypopituitarism]]. [[Prognosis]] is generally excellent provided early [[diagnosis]] and management resulting in complete reversal of [[symptoms]]. [[Diagnosis]] of Sheehan's syndrome is made on clinical basis with a recent/remote history of traumatic [[delivery]] or delivery complicated by [[hypotension]]. The most common presentation is [[postpartum]] lactation failure, [[amenorrhea]], loss of sexual hair, [[fatigue]], [[anorexia]] and [[weight loss]]. Clinical features depend upon the severity of [[hypopituitarism]] that results from Sheehan's syndrome. Almost all the patients have [[Growth hormone|GH]], [[prolactin]] and [[gonadotropin]] deficiency and the majority has [[Adrenocorticotropic hormone|ACTH]] and [[TSH]] deficiency. Laboratory evaluation gives a picture of partial or [[panhypopituitarism]] and laboratory findings consistent with the [[diagnosis]] of [[Sheehan's syndrome]] include [[hyponatremia]], [[hypokalemia]], [[hypocalcemia]], [[hypomagnesemia]], [[hypophosphatemia]], [[anemia]], [[pancytopenia]], [[eosinophilia]], [[hypoalbuminemia]], low [[fasting plasma glucose]] and decreased levels of [[anterior pituitary]] [[hormones]] in blood (low free [[thyroxine]], low [[estradiol]], decreased [[cortisol]] levels. The most sensitive test is inadequate [[prolactin]] and [[gonadotropin]] responses to stimulation. [[ECG]] findings associated with [[Sheehan's syndrome]] can include [[QT interval prolongation]], [[Brugada Syndrome|Type-1 Brugada-like ECG pattern]] (due to [[adrenal crisis]]), findings suggestive of [[cardiac tamponade]] ([[sinus tachycardia]], [[Low QRS voltage|low voltage QRS]] and [[electrical alternans]]), and [[dilated cardiomyopathy]]. [[Computed tomography|CT]] scan findings in case of [[acute]] presentation of Sheehan's syndrome may show non-[[hemorrhagic]] [[pituitary gland]] enlargement while [[chronic]] presentation may show an [[empty sella]] or decreased [[Sella turcica|sellar]] volume. Findings on [[Magnetic resonance imaging|MRI]] suggestive of [[Sheehan's syndrome]] include decreased [[Sella turcica|sellar]] volume, [[empty sella]], [[pituitary]] remnant tissue or [[Cerebrospinal fluid|CSF]] in the [[sella]].Treatment involves appropriate [[hormone replacement therapy]], which must be taken for the rest of your life that results in significant improvement and reversal of not only the physical symptoms but also the [[psychological]] symptoms.
Sheehan's syndrome is a life-threatening [[complication]] of severe [[Postpartum haemorrhage|postpartum hemorrhage (PPH)]] that results in mild to severe maternal [[hypopituitarism]]. Sheehan's syndrome is less prevalent in developed countries, but it is still one of the most common causes of [[hypopituitarism]] in underdeveloped and developing countries. During [[pregnancy]], enlarged size of the [[pituitary gland]] and decreased blood supply due to any cause can result in [[ischemic necrosis]] of [[pituitary gland]]. Severe [[Postpartum Hemorrhage|PPH]], [[Glandular tissue|glandular]] [[hypertrophy]] and [[hyperplasia]], smaller [[Sella turcica|sella]] size, [[autoimmunity]], and [[Disseminated Intravascular Coagulation|disseminated vascular coagulation (DIC)]] are thought to play an important role in the [[pathogenesis]] of Sheehan's syndrome. The most common causes of Sheehan's syndrome include [[massive hemorrhage]] during [[parturition]], [[vascular]] compression, and vascular [[occlusion]]. Sheehan's syndrome needs to be differentiated from [[diseases]] causing [[hypopituitarism]] and other diseases like [[lymphocytic hypophysitis]], [[pituitary apoplexy]], [[hypothyroidism]], [[addison's disease]], [[empty sella syndrome]], [[Hypogonadotrophic hypogonadism|hypogonadotropic hypogonadism]], [[Simmond's Disease|simmond's disease]], hypoprolactinemia, and [[menopause]]. [[Risk factors]] for Sheehan's syndrome include [[pregnancy]], [[Massive hemorrhage|severe/massive PPH]], pituitary mass, pre-existing [[Vascular disease|vascular diseases]], smaller and rigid [[Sella turcica|sella]], traumatic delivery, multiple [[Gestation|gestations]], [[placental]] abnormalities, and [[type 1 Diabetes|type 1 diabetes]]. If left untreated, Sheehan's syndrome can lead to [[panhypopituitarism]] and [[empty sella syndrome]]. Common [[complications]] of Sheehan's syndrome include [[adrenal crisis]], [[hypotension]], [[hypothyroidism]], and [[hypopituitarism]]. [[Prognosis]] is generally excellent, providing early [[diagnosis]] and management may result in complete reversal of [[symptoms]]. [[Diagnosis]] of Sheehan's syndrome is made on clinical basis with a recent/remote history of traumatic [[delivery]] or delivery complicated by [[hypotension]]. The most common presentations include [[postpartum]] lactation failure, [[amenorrhea]], loss of sexual hair, [[fatigue]], [[anorexia]], and [[weight loss]]. Clinical features depend on the severity of [[hypopituitarism]] that results from Sheehan's syndrome. Almost all the patients have [[Growth hormone|growth hormone (GH)]], [[prolactin]], and [[gonadotropin]] deficiency; and the majority of the patients have [[Adrenocorticotropic hormone|ACTH]] and [[TSH]] deficiency. Laboratory evaluation in patients with Sheehan's syndrome gives a picture of partial or [[panhypopituitarism]]. Other laboratory findings include [[hyponatremia]], [[hypokalemia]], [[hypocalcemia]], [[hypomagnesemia]], [[hypophosphatemia]], [[anemia]], [[pancytopenia]], [[eosinophilia]], [[hypoalbuminemia]], low [[fasting plasma glucose]], and decreased levels of [[anterior pituitary]] [[hormones]] (free [[thyroxine]], [[estradiol]], and [[cortisol]] levels). The most sensitive test is inadequate [[prolactin]] and [[gonadotropin]] response to stimulation. [[ECG]] findings associated with [[Sheehan's syndrome]] include [[QT interval prolongation]], [[Brugada Syndrome|type-1 Brugada-like ECG pattern]] (due to [[adrenal crisis]]), findings suggestive of [[cardiac tamponade]] ([[sinus tachycardia]], [[Low QRS voltage|low voltage QRS]], and [[electrical alternans]]), and [[dilated cardiomyopathy]]. [[Computed tomography|CT]] scan findings in case of [[acute]] Sheehan's syndrome may show non-[[hemorrhagic]] [[pituitary gland]] enlargement, while [[chronic]] presentation may show an [[empty sella]] or decreased [[Sella turcica|sella]] volume. Findings on [[Magnetic resonance imaging|MRI]] suggestive of [[Sheehan's syndrome]] include decreased [[Sella turcica|sella]] volume, [[empty sella]], [[pituitary]] remnant tissue or [[Cerebrospinal fluid|CSF]] in the [[sella]]. The treatment of Sheehan's syndrome involves appropriate [[hormone replacement therapy]], which must be taken for the rest of the patient's life, results in significant improvement and reversal of not only the physical symptoms, but also the [[psychological]] symptoms.


==Historical Perspective==
==Historical Perspective==
[[Sheehan's syndrome]] was first discovered by Leon Konrad Gliński about a century ago and it was named after Harold Sheehan (1900-1988).<ref>{{WhoNamedIt|synd|2009}}</ref><ref>H. L. Sheehan. Post-partum necrosis of anterior pituitary. The Journal of Pathology and Bacteriology, Chichester, 1937, 45: 189-214.</ref>
Sheehan's syndrome was first discovered by Leon Konrad Gliński about a century ago and it was named after Harold Sheehan (1900-1988).


==Classification==
==Classification==
There is no established system for the [[classification]] of Sheehan's syndrome
Sheehan's syndrome may be classified based on the onset or presentation of [[symptoms]] and the degree of [[glandular]] damage. Sheehan's syndrome may be classified into acute and chronic subtypes based on the time period after [[delivery]] and also extent of [[Gland|glandular]] damage. 


==Pathophysiology==
==Pathophysiology==
It is thought that Sheehan's syndrome is the result of [[ischemic necrosis]] of [[pituitary gland]] due to [[pituitary gland]] [[Enlargement of organs|enlargement]] during [[parturition]] precipitated by [[hypotension]] due to [[massive hemorrhage]]. Apart from [[pituitary gland]] enlargement during and before [[parturition]], [[vasospasm]], generalized [[Schwartzman phenomenon]],[[thrombosis]] and compression of the hypophyseal arteries, [[autoimmunity]], [[DIC]] and smaller size of [[Sella turcica|sella]] are thought to play a contributing role in [[pathogenesis]] of Sheehan syndrome.<ref name="pmid15237929">{{cite journal |vauthors=Keleştimur F |title=Sheehan's syndrome |journal=Pituitary |volume=6 |issue=4 |pages=181–8 |year=2003 |pmid=15237929 |doi= |url=}}</ref><ref>{{cite journal |last=Apitz |first=Kurt |date=September 1, 1935 |title=A Study of the Generalized Shwartzman Phenomenon|url=http://www.jimmunol.org/content/29/3/255.short|journal=The Journal of Immunology |volume=29 |issue=3 |pages=255-266}}</ref><ref name="McKayMerrill1953">{{cite journal|last1=McKay|first1=Donald G.|last2=Merrill|first2=Samuel J.|last3=Weiner|first3=Albert E.|last4=Hertig|first4=Arthur T.|last5=Reid|first5=Duncan E.|title=The pathologic anatomy of eclampsia, bilateral renal cortical necrosis, pituitary necrosis, and other acute fatal complications of pregnancy, and its possible relationship to the generalized Shwartzman phenomenon|journal=American Journal of Obstetrics and Gynecology|volume=66|issue=3|year=1953|pages=507–539|issn=00029378|doi=10.1016/0002-9378(53)90068-4}}</ref> [[Occlusion]] and other [[Vascular anomaly|vascular anomalies]] of the [[hypophyseal portal system]] can also cause [[complications]] in the exchange of [[hormones]] between the [[hypothalamus]] and the [[pituitary gland]] leading to [[hypopituitarism]]. Sheehan's syndrome results in mild to severe [[pituitary]] dysfunction resulting in partial or [[panhypopituitarism]] such as growth hormone ([[GH|GH)]], [[thyroid hormone]], [[glucocorticoid]], [[gonadotropins]] and [[prolactin]] [[Hormone|hormone deficiencies]] that manifests as a wide spectrum of presentation.<ref name="pmid8043090">{{cite journal |vauthors=Vance ML |title=Hypopituitarism |journal=N. Engl. J. Med. |volume=330 |issue=23 |pages=1651–62 |year=1994 |pmid=8043090 |doi=10.1056/NEJM199406093302306 |url=}}</ref> Usually, [[Growth hormone|GH]] is the earliest one to be lost.<ref name="pmid15237929">{{cite journal |vauthors=Keleştimur F |title=Sheehan's syndrome |journal=Pituitary |volume=6 |issue=4 |pages=181–8 |year=2003 |pmid=15237929 |doi= |url=}}</ref>
It is believed that Sheehan's syndrome is the result of [[ischemic necrosis]] of [[pituitary gland]], due to [[pituitary gland]] [[Enlargement of organs|enlargement]] during [[parturition]] that is precipitated by [[hypotension]] due to [[massive hemorrhage]]. Apart from [[pituitary gland]] enlargement during and before [[parturition]], [[vasospasm]], generalized [[Schwartzman phenomenon]], [[thrombosis]] and compression of the hypophyseal arteries, [[autoimmunity]], [[DIC]], and smaller size of [[Sella turcica|sella]] play a contributing role in [[pathogenesis]] of Sheehan's syndrome. [[Occlusion]] and other [[Vascular anomaly|vascular anomalies]] of the [[hypophyseal portal system]] can also complicate the exchange of [[hormones]] between the [[hypothalamus]] and the [[pituitary gland]], leading to [[hypopituitarism]]. Sheehan's syndrome may result in mild to severe [[pituitary]] dysfunction (partial or [[panhypopituitarism]]), which is identified with growth hormone ([[GH|GH)]], [[thyroid hormone]], [[glucocorticoid]], [[gonadotropins]], and [[prolactin]] [[Hormone|hormone deficiencies]], and manifests as a wide spectrum of presentation. Usually, [[Growth hormone|GH]] deficiency is the earliest one to develop.
 
==Causes==
==Causes==
Common causes of Sheehan's syndrome include [[massive hemorrhage]], [[Hypotension|hypotensio]]<nowiki/>n during pregnancy, [[vascular]] compression, and vascular [[occlusion]] ([[thrombosis]], [[DIC]]). Less common causes include [[vascular insufficiency]] due to [[CABG]] in older patients and snake bites (Russell's [[viper]] bites).
Common causes of Sheehan's syndrome include [[massive hemorrhage]], [[Hypotension|hypotensio]]<nowiki/>n during pregnancy, [[vascular]] compression, and vascular [[occlusion]] ([[thrombosis]], [[DIC]]). Less common causes are [[vascular insufficiency]] due to [[CABG|coronary artery bypass grafting (CABG)]] in older patients and [[Snake bite|snake bites]] (Russell's [[viper]] bites).


==Differentiating [[Sheehan's syndrome]] from Other Diseases==
==Differentiating Sheehan's syndrome from Other Diseases==
Sheehan syndrome must be differentiated from [[lymphocytic hypophysitis]], [[pituitary apoplexy]], [[hypothyroidism]], [[Addison's disease]], [[panhypopituitarism]], [[empty sella syndrome]], [[hypogonadotropic hypogonadism]], [[Simmond's Disease|Simmond's disease]], hypoprolactinemia and [[menopause]].<ref name="pmid8325288">{{cite journal |vauthors=Rolih CA, Ober KP |title=Pituitary apoplexy |journal=Endocrinol. Metab. Clin. North Am. |volume=22 |issue=2 |pages=291–302 |year=1993 |pmid=8325288 |doi= |url=}}</ref><ref name="pmid1520058">{{cite journal |vauthors=Vidal E, Cevallos R, Vidal J, Ravon R, Moreau JJ, Rogues AM, Loustaud V, Liozon F |title=Twelve cases of pituitary apoplexy |journal=Arch. Intern. Med. |volume=152 |issue=9 |pages=1893–9 |year=1992 |pmid=1520058 |doi= |url=}}</ref><ref name="pmid8183446">{{cite journal |vauthors=Lazaro CM, Guo WY, Sami M, Hindmarsh T, Ericson K, Hulting AL, Wersäll J |title=Haemorrhagic pituitary tumours |journal=Neuroradiology |volume=36 |issue=2 |pages=111–4 |year=1994 |pmid=8183446 |doi= |url=}}</ref>
Sheehan's syndrome must be differentiated from [[lymphocytic hypophysitis]], [[pituitary apoplexy]], [[hypothyroidism]], [[Addison's disease]], [[panhypopituitarism]], [[empty sella syndrome]], [[hypogonadotropic hypogonadism]], [[Simmond's Disease|Simmond's disease]], hypoprolactinemia, and [[menopause]] on the basis of hypo-functioning of the pituitary.


==Epidemiology and Demographics==
==Epidemiology and Demographics==
The [[incidence]] of Sheehan's syndrome is difficult to assess.<ref name="pmid303183">{{cite journal |vauthors=Asaoka K |title=[A study on the incidence of post-partum hypopituitarism, (Sheehan's syndrome)] |language=Japanese |journal=Nihon Naibunpi Gakkai Zasshi |volume=53 |issue=7 |pages=895–909 |year=1977 |pmid=303183 |doi= |url=}}</ref> It was found to be the 6th most common cause of [[Growth hormone|GH]] deficiency with an [[incidence]] of 3.1% of cases.<ref name="pmid10468941">{{cite journal |vauthors=Abs R, Bengtsson BA, Hernberg-Stâhl E, Monson JP, Tauber JP, Wilton P, Wüster C |title=GH replacement in 1034 growth hormone deficient hypopituitary adults: demographic and clinical characteristics, dosing and safety |journal=Clin. Endocrinol. (Oxf) |volume=50 |issue=6 |pages=703–13 |year=1999 |pmid=10468941 |doi= |url=}}</ref> In 2009, the [[prevalence]] of Sheehan's syndrome was estimated to be 5.1 per 100,000 women.It is less [[Prevalence|prevalent]] in [[Developed country|developed countries]] due to better [[obstetrical]] care and [[maternal health]] awareness.<ref name="pmid16213852">{{cite journal |vauthors=Feinberg EC, Molitch ME, Endres LK, Peaceman AM |title=The incidence of Sheehan's syndrome after obstetric hemorrhage |journal=Fertil. Steril. |volume=84 |issue=4 |pages=975–9 |year=2005 |pmid=16213852 |doi=10.1016/j.fertnstert.2005.04.034 |url=}}</ref><ref name="pmid26817341">{{cite journal |vauthors=Krysiak R, Okopień B |title=[Sheehan's syndrome--a forgotten disease with 100 years' history] |language=Polish |journal=Prz. Lek. |volume=72 |issue=6 |pages=313–20 |year=2015 |pmid=26817341 |doi= |url=}}</ref> It is still one of the most common [[causes]] of [[hypopituitarism]] in developing countries.<ref name="pmid26817341">{{cite journal |vauthors=Krysiak R, Okopień B |title=[Sheehan's syndrome--a forgotten disease with 100 years' history] |language=Polish |journal=Prz. Lek. |volume=72 |issue=6 |pages=313–20 |year=2015 |pmid=26817341 |doi= |url=}}</ref>
The [[incidence]] of Sheehan's syndrome is difficult to assess. It was found to be the 6th most common cause of [[Growth hormone|growth hormone (GH)]] deficiency with an [[incidence]] of 3.1% of cases. In 2009, the [[prevalence]] of Sheehan's syndrome was estimated to be 5.1 per 100,000 women. Sheehan's syndrome is less [[Prevalence|prevalent]] in [[Developed country|developed countries]] due to better [[obstetrical]] care and [[maternal health]] awareness. Sheehan's syndrome is one of the most common [[causes]] of [[hypopituitarism]] in developing countries.


==Risk Factors==
==Risk Factors==
Common risk factors in the development of Sheehan's syndrome include pregnancy, severe/massive PPH, pituitary mass, pre-existing vascular diseases, autoimmunity,type 1 Diabetes, DIC, smaller and rigid sella, multiple gestations, placental abnormalities and traumatic delivery.
Common risk factors in the development of Sheehan's syndrome include [[pregnancy]], severe/massive [[Postpartum hemorrhage|postpartum hemorrhage (PPH)]], [[pituitary]] mass, pre-existing [[Vascular disease|vascular diseases]], [[autoimmunity]], [[Disseminated intravascular coagulation|DIC]], smaller and rigid [[sella]], and traumatic [[Childbirth|delivery]].


==Screening==
==Screening==
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==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
Sheehan's syndrome, if left untreated lead to hypopituitarism and empty sella syndrome. Common complications include adrenal crisis, hypotension, hypothyroidism and hypopituitarism. Prognosis is generally excellent provided early diagnosis and management resulting in complete reversal of symptoms.
If left untreated, Sheehan's syndrome leads to [[hypopituitarism]] and [[empty sella syndrome]]. Common complications are [[adrenal crisis]], [[hypotension]], [[hypothyroidism]], and [[hypopituitarism]]. [[Prognosis]] is generally excellent; early [[diagnosis]] and management often results in a complete reversal of symptoms.


==Diagnosis==
==Diagnosis==
Diagnosis is made on clinical basis with a recent/remote history of traumatic delivery or delivery complicated by hypotension. Diagnosis is mostly clinical but detailed medical history, measurement of pituitary hormone levels in blood, pituitary hormone stimulation tests and imaging (MRI preferred on CT) studies can help in making the diagnosis.
Diagnosis of Sheehan's syndrome is made on clinical basis with a recent or remote history of traumatic delivery or delivery complicated by [[hypotension]]. Diagnosis is mostly clinical but detailed medical history, measurement of [[pituitary hormone]] levels in blood, [[pituitary hormone]] stimulation tests and imaging ([[MRI]] preferred on [[CT]]) studies can help in making the diagnosis.
===History and Symptoms===
===History and Symptoms===
The most common symptoms of Sheehan's sydrome include agalactorrhea and failure to resume menstruation after parturition. Common symptoms include hot flushes, decreased pubic/axillary hair, hypotension, hypoglycemia, features of hypothyroidism, hypoadrenalism and hypogonadism.
The most common symptoms of Sheehan's sydrome are agalactorrhea and failure to resume [[menstruation]] after [[parturition]]. Common symptoms are [[hot flushes|hot flashes]], decreased [[Pubic hair|pubic]] or [[axillary hair]], [[hypotension]], [[hypoglycemia]], features of [[hypothyroidism]], [[hypoadrenalism]], and [[hypogonadism]].


===Physical Examination===
===Physical Examination===
Patients with syndrome usually appear fatigued and lethargic. Physical examination is usually remarkable for bradycardia, hypotension, pallor and signs suggestive of respective hormonal deficiency.
Patients with Sheehan's syndrome usually appear [[Fatigue|fatigued]] and [[lethargic]]. Physical examination is remarkable for [[bradycardia]], [[hypotension]], [[pallor]], and signs suggestive of respective pituitary hormonal deficiency.


===Laboratory Findings===
===Laboratory Findings===
Laboratory findings consistent with the diagnosis of Sheehan's syndrome include hyponatremia, hypokalemia, hypocalcemia, hypomagnesemia, hypophosphatemia, anemia, pancytopenia, eosinophilia, hypoalbuminemia, low fasting plasma glucose etc.
Laboratory findings consistent with the diagnosis of Sheehan's syndrome include [[hyponatremia]], [[hypokalemia]], [[hypocalcemia]], [[hypomagnesemia]], [[hypophosphatemia]], [[anemia]], [[pancytopenia]], [[eosinophilia]], [[hypoalbuminemia]] and low [[fasting plasma glucose]].


===Electrocardiogram===
===Electrocardiogram===
ECG findings associated with Sheehan's syndrome can include QT interval prolongation,Type-1 Brugada-like ECG pattern(due to adrenal crisis),findings suggestive of cardiac tamponade, dilated cardiomyopathy(multifactorial).<ref name="pmid25771803">{{cite journal |vauthors=Komuro J, Kaneko M, Ueda K, Nitta S, Kasao M, Shirai T |title=Adrenal insufficiency causes life-threatening arrhythmia with prolongation of QT interval |journal=Heart Vessels |volume=31 |issue=6 |pages=1003–5 |year=2016 |pmid=25771803 |pmc=4893060 |doi=10.1007/s00380-015-0660-6 |url=}}</ref><ref name="pmid24718975">{{cite journal |vauthors=Anselm DD, Baranchuk A |title=Confirmed Brugada phenocopy in the setting of hypopituitarism |journal=Herz |volume=40 |issue=4 |pages=639–40 |year=2015 |pmid=24718975 |doi=10.1007/s00059-014-4075-4 |url=}}</ref><ref name="pmid28365624">{{cite journal |vauthors=Martin-Grace J, Ahmed M, Mulvihill N, Feeney ER, Crowley RK |title=Getting to the heart of hypopituitarism |journal=Clin Med (Lond) |volume=17 |issue=2 |pages=140–142 |year=2017 |pmid=28365624 |doi=10.7861/clinmedicine.17-2-140 |url=}}</ref><ref name="pmid23513050">{{cite journal |vauthors=Doshi S, Roy A, Ramamoorthy A, Kothari SS, Bahl VK |title=Dilated cardiomyopathy: a ghost from the past |journal=Circ Heart Fail |volume=6 |issue=2 |pages=e19–21 |year=2013 |pmid=23513050 |doi=10.1161/CIRCHEARTFAILURE.112.000062 |url=}}</ref>
[[ECG|Electrocardiogram (ECG)]] findings associated with Sheehan's syndrome may include [[QT interval prolongation]], [[Brugada Syndrome|type-1 Brugada-like]] [[ECG]] pattern (due to [[adrenal crisis]]), or findings suggestive of [[cardiac tamponade]] or [[dilated cardiomyopathy]].


===X-ray===
===X-ray===
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===CT scan===
===CT scan===
Acute presentation shows non-hemorrhagic pituitary gland enlargement while chronic presentation shows an empty sella or decreased sellar volume.<ref name="pmid2750772">{{cite journal |vauthors=Barkan AL |title=Pituitary atrophy in patients with Sheehan's syndrome |journal=Am. J. Med. Sci. |volume=298 |issue=1 |pages=38–40 |year=1989 |pmid=2750772 |doi= |url=}}</ref><ref name="pmid2591059">{{cite journal |vauthors=Sherif IH, Vanderley CM, Beshyah S, Bosairi S |title=Sella size and contents in Sheehan's syndrome |journal=Clin. Endocrinol. (Oxf) |volume=30 |issue=6 |pages=613–8 |year=1989 |pmid=2591059 |doi= |url=}}</ref><ref name="pmid1906495">{{cite journal |vauthors=Bakiri F, Bendib SE, Maoui R, Bendib A, Benmiloud M |title=The sella turcica in Sheehan's syndrome: computerized tomographic study in 54 patients |journal=J. Endocrinol. Invest. |volume=14 |issue=3 |pages=193–6 |year=1991 |pmid=1906495 |doi=10.1007/BF03346787 |url=}}</ref>
CT scans in patients with Sheehan's syndrome shows non-hemorrhagic [[pituitary]] gland enlargement in acute cases while chronic cases present as an [[empty sella]] or decreased [[Sella turcica|sella]] volume.


===MRI===
===MRI===
Findings on MRI suggestive of Sheehan's syndrome include decreased sellar volume, empty sella, pituitary remnant tissue or CSF fluid in sella.<ref name="pmid2750772">{{cite journal |vauthors=Barkan AL |title=Pituitary atrophy in patients with Sheehan's syndrome |journal=Am. J. Med. Sci. |volume=298 |issue=1 |pages=38–40 |year=1989 |pmid=2750772 |doi= |url=}}</ref><ref name="pmid2591059">{{cite journal |vauthors=Sherif IH, Vanderley CM, Beshyah S, Bosairi S |title=Sella size and contents in Sheehan's syndrome |journal=Clin. Endocrinol. (Oxf) |volume=30 |issue=6 |pages=613–8 |year=1989 |pmid=2591059 |doi= |url=}}</ref><ref name="pmid1906495">{{cite journal |vauthors=Bakiri F, Bendib SE, Maoui R, Bendib A, Benmiloud M |title=The sella turcica in Sheehan's syndrome: computerized tomographic study in 54 patients |journal=J. Endocrinol. Invest. |volume=14 |issue=3 |pages=193–6 |year=1991 |pmid=1906495 |doi=10.1007/BF03346787 |url=}}</ref>
Findings on [[MRI]] suggestive of Sheehan's syndrome are decreased [[sella]] volume, [[empty sella]], [[pituitary]] remnant tissue, or [[CSF|cerebrospinal fluid (CSF)]] in [[sella]].


===Ultrasound===
===Ultrasound and Echocardiography===
Echo findings associated with Sheehan's syndrome may include reversible dilated cardiomyopathy and pericardial effusion.<ref name="pmid1553916">{{cite journal |vauthors=Frustaci A, Perrone GA, Gentiloni N, Russo MA |title=Reversible dilated cardiomyopathy due to growth hormone deficiency |journal=Am. J. Clin. Pathol. |volume=97 |issue=4 |pages=503–11 |year=1992 |pmid=1553916 |doi= |url=}}</ref><ref name="pmid21668725">{{cite journal |vauthors=Hsieh CY, Liu BY, Yang YN, Yin WH, Young MS |title=Massive pericardial effusion with diastolic right ventricular compression secondary to hypothyroidism in a 73-year-old woman |journal=Emerg Med Australas |volume=23 |issue=3 |pages=372–5 |year=2011 |pmid=21668725 |doi=10.1111/j.1742-6723.2011.01425.x |url=}}</ref>
[[Echocardiography]] findings associated with Sheehan's syndrome may include reversible [[dilated cardiomyopathy]] and [[pericardial effusion]].


===Other Imaging Findings===
===Other Imaging Findings===
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===Other Diagnostic Studies===
===Other Diagnostic Studies===
There are no additional diagnostic findings for Sheehan's syndrome.
Surgical intervention may be considered when there is emergency presentation, such as [[pituitary apoplexy]] or [[subarachnoid hemorrhage]] causing Sheehan's syndrome to prevent [[hypopituitarism]].


==Treatment==
==Treatment==
===Medical Therapy===
===Medical Therapy===
Treatment involves appropriate hormone replacement therapy that results in complete recovery and reversal of symptoms.
The treatment of Sheehan's syndrome involves [[hormone replacement therapy]], which generally results in complete recovery and reversal of symptoms.


===Surgery===
===Surgery===
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===Primary Prevention===
===Primary Prevention===
Effective measures for the primary prevention of Sheehan's syndrome include; improved obstetrical care and peri-natal monitoring, prevention of pregnancy related complications, maternal awareness about Sheehan's syndrome and risk factors causing it and post-puerperal follow up.
Effective measures for the primary prevention of Sheehan's syndrome include improved [[obstetrical]] care and [[perinatal]] monitoring, prevention of [[pregnancy]] related complications such as [[hypotension]] or [[hemorrhage]], maternal awareness about risk factors of Sheehan's syndrome, and post-puerperal follow up.


===Secondary Prevention===
===Secondary Prevention===
Effective measures for the secondary prevention include early diagnosis and treatment to prevent life threatening complications.
Effective measures for the secondary prevention of sheehan's syndrome are early [[diagnosis]] and treatment to prevent life-threatening complications, such as [[adrenal]] crisis and [[infertility]]. Other measures of secondary prevention include frequent prenatal visits and prevention of development of [[hypotension]] due to any reason.  


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Latest revision as of 00:09, 30 July 2020

Sheehan's syndrome Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Sheehan's syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

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History and Symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2]

Overview

Sheehan's syndrome is a life-threatening complication of severe postpartum hemorrhage (PPH) that results in mild to severe maternal hypopituitarism. Sheehan's syndrome is less prevalent in developed countries, but it is still one of the most common causes of hypopituitarism in underdeveloped and developing countries. During pregnancy, enlarged size of the pituitary gland and decreased blood supply due to any cause can result in ischemic necrosis of pituitary gland. Severe PPH, glandular hypertrophy and hyperplasia, smaller sella size, autoimmunity, and disseminated vascular coagulation (DIC) are thought to play an important role in the pathogenesis of Sheehan's syndrome. The most common causes of Sheehan's syndrome include massive hemorrhage during parturition, vascular compression, and vascular occlusion. Sheehan's syndrome needs to be differentiated from diseases causing hypopituitarism and other diseases like lymphocytic hypophysitis, pituitary apoplexy, hypothyroidism, addison's disease, empty sella syndrome, hypogonadotropic hypogonadism, simmond's disease, hypoprolactinemia, and menopause. Risk factors for Sheehan's syndrome include pregnancy, severe/massive PPH, pituitary mass, pre-existing vascular diseases, smaller and rigid sella, traumatic delivery, multiple gestations, placental abnormalities, and type 1 diabetes. If left untreated, Sheehan's syndrome can lead to panhypopituitarism and empty sella syndrome. Common complications of Sheehan's syndrome include adrenal crisis, hypotension, hypothyroidism, and hypopituitarism. Prognosis is generally excellent, providing early diagnosis and management may result in complete reversal of symptoms. Diagnosis of Sheehan's syndrome is made on clinical basis with a recent/remote history of traumatic delivery or delivery complicated by hypotension. The most common presentations include postpartum lactation failure, amenorrhea, loss of sexual hair, fatigue, anorexia, and weight loss. Clinical features depend on the severity of hypopituitarism that results from Sheehan's syndrome. Almost all the patients have growth hormone (GH), prolactin, and gonadotropin deficiency; and the majority of the patients have ACTH and TSH deficiency. Laboratory evaluation in patients with Sheehan's syndrome gives a picture of partial or panhypopituitarism. Other laboratory findings include hyponatremia, hypokalemia, hypocalcemia, hypomagnesemia, hypophosphatemia, anemia, pancytopenia, eosinophilia, hypoalbuminemia, low fasting plasma glucose, and decreased levels of anterior pituitary hormones (free thyroxine, estradiol, and cortisol levels). The most sensitive test is inadequate prolactin and gonadotropin response to stimulation. ECG findings associated with Sheehan's syndrome include QT interval prolongation, type-1 Brugada-like ECG pattern (due to adrenal crisis), findings suggestive of cardiac tamponade (sinus tachycardia, low voltage QRS, and electrical alternans), and dilated cardiomyopathy. CT scan findings in case of acute Sheehan's syndrome may show non-hemorrhagic pituitary gland enlargement, while chronic presentation may show an empty sella or decreased sella volume. Findings on MRI suggestive of Sheehan's syndrome include decreased sella volume, empty sella, pituitary remnant tissue or CSF in the sella. The treatment of Sheehan's syndrome involves appropriate hormone replacement therapy, which must be taken for the rest of the patient's life, results in significant improvement and reversal of not only the physical symptoms, but also the psychological symptoms.

Historical Perspective

Sheehan's syndrome was first discovered by Leon Konrad Gliński about a century ago and it was named after Harold Sheehan (1900-1988).

Classification

Sheehan's syndrome may be classified based on the onset or presentation of symptoms and the degree of glandular damage. Sheehan's syndrome may be classified into acute and chronic subtypes based on the time period after delivery and also extent of glandular damage. 

Pathophysiology

It is believed that Sheehan's syndrome is the result of ischemic necrosis of pituitary gland, due to pituitary gland enlargement during parturition that is precipitated by hypotension due to massive hemorrhage. Apart from pituitary gland enlargement during and before parturition, vasospasm, generalized Schwartzman phenomenon, thrombosis and compression of the hypophyseal arteries, autoimmunity, DIC, and smaller size of sella play a contributing role in pathogenesis of Sheehan's syndrome. Occlusion and other vascular anomalies of the hypophyseal portal system can also complicate the exchange of hormones between the hypothalamus and the pituitary gland, leading to hypopituitarism. Sheehan's syndrome may result in mild to severe pituitary dysfunction (partial or panhypopituitarism), which is identified with growth hormone (GH), thyroid hormone, glucocorticoid, gonadotropins, and prolactin hormone deficiencies, and manifests as a wide spectrum of presentation. Usually, GH deficiency is the earliest one to develop.

Causes

Common causes of Sheehan's syndrome include massive hemorrhage, hypotension during pregnancy, vascular compression, and vascular occlusion (thrombosis, DIC). Less common causes are vascular insufficiency due to coronary artery bypass grafting (CABG) in older patients and snake bites (Russell's viper bites).

Differentiating Sheehan's syndrome from Other Diseases

Sheehan's syndrome must be differentiated from lymphocytic hypophysitis, pituitary apoplexy, hypothyroidism, Addison's disease, panhypopituitarism, empty sella syndrome, hypogonadotropic hypogonadism, Simmond's disease, hypoprolactinemia, and menopause on the basis of hypo-functioning of the pituitary.

Epidemiology and Demographics

The incidence of Sheehan's syndrome is difficult to assess. It was found to be the 6th most common cause of growth hormone (GH) deficiency with an incidence of 3.1% of cases. In 2009, the prevalence of Sheehan's syndrome was estimated to be 5.1 per 100,000 women. Sheehan's syndrome is less prevalent in developed countries due to better obstetrical care and maternal health awareness. Sheehan's syndrome is one of the most common causes of hypopituitarism in developing countries.

Risk Factors

Common risk factors in the development of Sheehan's syndrome include pregnancy, severe/massive postpartum hemorrhage (PPH), pituitary mass, pre-existing vascular diseases, autoimmunity, DIC, smaller and rigid sella, and traumatic delivery.

Screening

There is insufficient evidence to recommend routine screening for Sheehan's syndrome.

Natural History, Complications, and Prognosis

If left untreated, Sheehan's syndrome leads to hypopituitarism and empty sella syndrome. Common complications are adrenal crisis, hypotension, hypothyroidism, and hypopituitarism. Prognosis is generally excellent; early diagnosis and management often results in a complete reversal of symptoms.

Diagnosis

Diagnosis of Sheehan's syndrome is made on clinical basis with a recent or remote history of traumatic delivery or delivery complicated by hypotension. Diagnosis is mostly clinical but detailed medical history, measurement of pituitary hormone levels in blood, pituitary hormone stimulation tests and imaging (MRI preferred on CT) studies can help in making the diagnosis.

History and Symptoms

The most common symptoms of Sheehan's sydrome are agalactorrhea and failure to resume menstruation after parturition. Common symptoms are hot flashes, decreased pubic or axillary hair, hypotension, hypoglycemia, features of hypothyroidism, hypoadrenalism, and hypogonadism.

Physical Examination

Patients with Sheehan's syndrome usually appear fatigued and lethargic. Physical examination is remarkable for bradycardia, hypotension, pallor, and signs suggestive of respective pituitary hormonal deficiency.

Laboratory Findings

Laboratory findings consistent with the diagnosis of Sheehan's syndrome include hyponatremia, hypokalemia, hypocalcemia, hypomagnesemia, hypophosphatemia, anemia, pancytopenia, eosinophilia, hypoalbuminemia and low fasting plasma glucose.

Electrocardiogram

Electrocardiogram (ECG) findings associated with Sheehan's syndrome may include QT interval prolongation, type-1 Brugada-like ECG pattern (due to adrenal crisis), or findings suggestive of cardiac tamponade or dilated cardiomyopathy.

X-ray

There are no x-ray findings associated with Sheehan's syndrome.

CT scan

CT scans in patients with Sheehan's syndrome shows non-hemorrhagic pituitary gland enlargement in acute cases while chronic cases present as an empty sella or decreased sella volume.

MRI

Findings on MRI suggestive of Sheehan's syndrome are decreased sella volume, empty sella, pituitary remnant tissue, or cerebrospinal fluid (CSF) in sella.

Ultrasound and Echocardiography

Echocardiography findings associated with Sheehan's syndrome may include reversible dilated cardiomyopathy and pericardial effusion.

Other Imaging Findings

There are no other imaging findings associated with Sheehan's syndrome.

Other Diagnostic Studies

Surgical intervention may be considered when there is emergency presentation, such as pituitary apoplexy or subarachnoid hemorrhage causing Sheehan's syndrome to prevent hypopituitarism.

Treatment

Medical Therapy

The treatment of Sheehan's syndrome involves hormone replacement therapy, which generally results in complete recovery and reversal of symptoms.

Surgery

Surgical intervention is not recommended for the management of Sheehan's syndrome.

Primary Prevention

Effective measures for the primary prevention of Sheehan's syndrome include improved obstetrical care and perinatal monitoring, prevention of pregnancy related complications such as hypotension or hemorrhage, maternal awareness about risk factors of Sheehan's syndrome, and post-puerperal follow up.

Secondary Prevention

Effective measures for the secondary prevention of sheehan's syndrome are early diagnosis and treatment to prevent life-threatening complications, such as adrenal crisis and infertility. Other measures of secondary prevention include frequent prenatal visits and prevention of development of hypotension due to any reason.

References

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