Sheehan's syndrome: what is it, symptoms, treatment, prognosis

What is Sheehan Syndrome?

Sheehan's syndrome - postpartum hypopituitarismcaused by pituitary necrosis. This disorder is usually the result of severe hypotension or shockcaused by massive bleeding during or after childbirth. Excessive bleeding can reduce blood flow to the pituitary gland, causing damage or death of pituitary cells (necrosis). As a result, the production of conventional pituitary hormones is reduced, possibly by a significant amount.

During pregnancy, the pituitary gland will swell and can double in size. During this time, the gland is especially vulnerable to a sharp drop in blood pressure (sometimes called "shock"), excessive bleeding in the mother can cause "shock" and damage to the gland cells. During this time, the amount of hormones produced by the pituitary gland may decrease, leading to symptoms associated with hypopituitarism.

There seem to be two forms of the disorder; chronic form and acute form, depending on the degree of damage to the cells of the gland. The acute form reflects significant damage, so symptoms become apparent shortly after delivery. In chronic cases, the extent of the lesions is much smaller and symptoms may not appear until months or years after delivery.

Signs and symptoms

The clinical signs of Sheehan's syndrome vary greatly and depend on the degree of impairment in the secretion of pituitary hormones, including:

• prolactin, a hormone that stimulates lactation;
• gonadotropins (luteinizing hormone [LH] and follicle-stimulating hormone [FSH]), which regulate ovarian function;
• thyroid-stimulating hormone (TSH), which stimulates work thyroid gland;
• ACTH, adrenocorticotropin, which stimulates the cortex adrenal glands;
• growth hormone (GH).

How much of the pituitary gland dies and how much the level of hormones in the bloodstream decreases determines what happens to the mother. Chronic patients have less damaged pituitary tissue and symptoms may not appear until weeks or even years after birth.

In its most severe form, the condition is associated with impaired lactation after the birth of a child. Menstruation does not start again, sexual interest (libido) decreases, armpit hair slowly disappears, the breasts shrink (atrophy) and the vaginal lining becomes thinner, sometimes causing pain during intercourse act. Some women menstrual cycle repeated and subsequent pregnancies reported.

Typical symptoms (fatigue, dry skin, constipation, weight gain, lethargy) hypothyroidism usually develop gradually. Severe ACTH deficiency is associated with fatigue, chronic hypotension with fainting and an inability to respond to stress. If these symptoms occur, they usually appear within weeks or months after the baby is born.

Since Sheehan syndrome is a disease that affects adults, the consequences of growth hormone deficiency limited to some loss of muscle strength, increased body fat and increased sensitivity to insulin.

A less common acute or more severe form is potentially very dangerous. In these cases, less than 10 percent of the normal volume of pituitary tissue remains. Patients may have persistent low blood pressure (hypotension), irregular and rapid heartbeat (tachycardia), as well as lactate deficiency and low blood sugar (hypoglycemia) immediately after childbirth.

In both chronic and acute forms, symptoms may be observed diabetes insipidussuch as an irresistible thirst and excessive water intake, as well as a large volume of urine.

Causes and risk factors

In developed countries, Sheehan's syndrome is a rare complication of pregnancy, usually resulting from excessive blood loss after childbirth. The presence of disseminated intravascular coagulation (eg, amniotic fluid embolism or HELLP syndrome) is also a factor in its development. A retrospective study in Turkey showed that the prevalence of Sheehan's syndrome is directly proportional to the number of home births in each decade. This may be due to previously limited obstetric methods used at home. For example, an area of ​​improvement in preventing the condition may be to improve the efficacy of blood transfusion therapy for hypovolemic shock.

Pituitary necrosis may be a direct result of insufficient blood flow to the pituitary artery as a result of enlargement of the pituitary gland during pregnancy. One of the reasons for pituitary gland growth associated with the risk of developing Sheehan's syndrome is hyperplasia of lactotrophs (responsible for the production of prolactin). Ischemia can occur as a result of vasospasm, thrombosis, or vascular compression, sometimes as a result an increase in the number of lactotrophic cells throughout pregnancy (which contributes to an increase pituitary gland). Necrosis can result from severe hypotension or shock from excessive uterine bleeding after delivery. Sheehan's syndrome can result from narrowing of the artery and abnormal hypotension in combination with insufficient satisfaction of the increased needs of the pituitary gland for blood supply, observed during pregnancy. This increased blood flow and metabolic demand is associated with the previously mentioned hyperplasia of lactotrophs.

Some possible predisposing factors to Sheehan's syndrome may include: hereditary or acquired disseminated intravascular coagulation (DIC syndrome), restriction of blood supply to the pituitary gland, small size of the sella turcica, vasospasm or thrombosis. Postpartum haemorrhage (PPH) is considered a predictor of Sheehan's syndrome, so symptoms anemia, obesity and older mothers may increase the risk of Sheehan's syndrome. Uterine atony can be associated with PPH, which can cause Sheehan syndrome. This results in abnormally prolonged blood flow to the placenta after delivery.

Diagnostics

The diagnosis of Sheehan's syndrome is usually made when a woman has symptoms of hypopituitarism and has a history of profuse bleeding during labor. The doctor may also order blood tests to measure pituitary hormone levels to confirm the diagnosis.

In addition, magnetic resonance imaging (MRI) or computed tomography (CT) may be ordered to obtain images of the pituitary gland and exclude the possibility of other problems with the pituitary gland, such as a tumor, since pituitary tumors are the main cause of hypopituitarism and its attendant symptoms.

In situations where Sheehan's syndrome is acute and symptoms begin to appear immediately, the diagnosis is made before the woman leaves the hospital and treatment begins immediately.

Epidemiology

A study of 1,034 adults with Sheehan's syndrome found the disease ranked sixth in terms of the incidence of growth hormone deficiency and is the cause of 3.1% of cases (compared with 53.9% due to tumor pituitary gland).

Sheehan's syndrome is more common in developing countries than in developed ones. In addition, it was found that most women who had the disease gave birth at home rather than in a hospital.

Symptomatic disorders

Symptoms of the following disorders may be similar to those of Sheehan's syndrome. Comparisons can be useful for differential diagnosis:

• Antiphospholipid syndrome (AFS) - rare autoimmune diseasecharacterized by recurring blood clots that usually appear before the age of 45. APS can also be associated with repeated miscarriages for no apparent reason in young women. In some cases, there may be a family history of bleeding disorders. Antiphospholipid syndrome can occur in people with lupus erythematosus or related autoimmune diseases or as a primary syndrome in healthy people.
• Pituitary (autoimmune lymphocytic hypophysitis - ALH) is an inflammation of the pituitary gland. Irritation caused by an inflammatory response may interfere with the production of one or more hormones of the pituitary gland, and in this regard, pituitary may mimic the symptoms of the syndrome to some extent Shihan. Likewise, pituitary tumors such as pituitary adenomas can also mimic Sheehan's syndrome.

Standard treatments

Sheehan's syndrome is treated in the same way as normal hypopituitarism - hormone replacement therapy. The doctor will assess the patient's hormone levels and determine which ones need hormone replacement therapy as it depends on the individual.

• Estrogen and Progesterone: it is usually taken until the age of menopause, after which the doctor will assess if they are still needed. In cases where a woman has had a hysterectomy (removal of the uterus), only estrogen will be prescribed. Prescribing oral contraceptives is one of the most common ways to replenish these hormones.
• Levothyroxine: the drug increases the deficiency of thyroid hormones caused by low or insufficient production of thyroid-stimulating hormone (TSH).
• Cortisones such as prednisone and hydrocortisone: are used to replace adrenocorticotropic hormones (ACTH).
• Growth hormone (GH): Your doctor may prescribe it to help increase muscle mass, lower cholesterol levels, and generally improve your overall well-being.

Forecast

The prognosis for early diagnosis and treatment is excellent. The condition can be life-threatening if left untreated.