Sheehan's syndrome overview: Difference between revisions
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==Epidemiology and Demographics== | ==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. | 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== | ||
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==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
Sheehan's syndrome, if left untreated | Sheehan's syndrome, if left untreated, leads to [[hypopituitarism]] and [[empty sella syndrome]]. Common complications are [[adrenal crisis]], [[hypotension]], [[hypothyroidism]], and [[hypopituitarism]]. [[Prognosis]] is generally excellent provided early [[diagnosis]] and management resulting in complete reversal of symptoms. | ||
==Diagnosis== | ==Diagnosis== | ||
Diagnosis is made on clinical basis with a recent | 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 are agalactorrhea and failure to resume [[menstruation]] after [[parturition]]. Common symptoms are [[hot flushes|hot flashes]], decreased [[Pubic hair|pubic]] | 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 | 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]] | 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 | [[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=== | ||
CT scan in 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 are decreased [[sella]] volume, [[empty sella]], [[pituitary]] remnant tissue, or [[CSF]] | 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=== | ||
[[Echocardiography]] findings associated with Sheehan's syndrome may include reversible [[dilated cardiomyopathy]] and [[pericardial effusion]]. | [[Echocardiography]] findings associated with Sheehan's syndrome may include reversible [[dilated cardiomyopathy]] and [[pericardial effusion]]. | ||
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==Treatment== | ==Treatment== | ||
===Medical Therapy=== | ===Medical Therapy=== | ||
Treatment involves appropriate [[hormone replacement therapy]] that results in complete recovery and reversal of symptoms. | Treatment of Sheehan's syndrome involves appropriate [[hormone replacement therapy]] that 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 | 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 | Effective measures for the secondary prevention include early diagnosis and treatment to prevent life threatening complications. | ||
==References== | ==References== | ||
Revision as of 16:33, 11 September 2017
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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) and results in mild to severe 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. Sheehan's syndrome, if left untreated, 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. Treatment of Sheehan's syndrome involves appropriate hormone replacement therapy, which must be taken for the rest of the 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
There is no established system for the classification of Sheehan's syndrome.
Pathophysiology
It is thought that Sheehan's syndrome is the result of ischemic necrosis of pituitary gland, due to pituitary gland 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 play a contributing role in pathogenesis of Sheehan 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; manifests as a wide spectrum of presentation. Usually, GH is the earliest one to be lost.
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 PPH, pituitary mass, pre-existing vascular diseases, autoimmunity, type 1 diabetes, DIC, smaller and rigid sella, multiple gestations, placental abnormalities, and traumatic delivery.
Screening
There is insufficient evidence to recommend routine screening for Sheehan's syndrome.
Natural History, Complications, and Prognosis
Sheehan's syndrome, if left untreated, leads to hypopituitarism and empty sella syndrome. Common complications are adrenal crisis, hypotension, hypothyroidism, and hypopituitarism. Prognosis is generally excellent provided early diagnosis and management resulting in 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 scan in 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
There are no additional diagnostic findings for Sheehan's syndrome.
Treatment
Medical Therapy
Treatment of Sheehan's syndrome involves appropriate hormone replacement therapy that 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 include early diagnosis and treatment to prevent life threatening complications.