Sheehan syndrome

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The condition occurs due to ischemic pituitary necrosis, usually secondary to a loss of a life-threatening amount of blood (uterine haemorrhage) or a severe drop in blood pressure (hypotension). The reduced blood supply and oxygen deficiency that result from these events can damage the pituitary gland (small gland located at the base of the brain), which is no longer able to produce enough hormones. state of pituitary insufficiency (hypopituitarism).

After the baby is born, lack of milk supply and failure to resume menstruation (post-pregnancy amenorrhea) are the most common presenting symptoms.

The diagnosis of Sheehan's syndrome requires the execution of neuroradiological tests and the dosage of pituitary hormones, in basal conditions and after various types of stimulus tests. These investigations aim to determine which pituitary hormones are deficient and if it is necessary to replace them. pharmacologically.

Treatment of Sheehan's syndrome usually involves hormone replacement therapy, to be modulated on the basis of the hormones that the woman is lacking.

and from a state of shock, which occurs during or immediately after childbirth.

Also called postpartum hypopituitarism, this condition is characterized by reduced or absent secretion of one or more hormones by the pituitary (or pituitary gland). The presentation of Sheehan syndrome varies from one patient to another (clinical manifestations range from from non-specific symptoms to coma). Usually, milk discharge (agalease) does not appear and the patient may complain of fatigue, amenorrhea, cold intolerance and loss of pubic and axillary hair.

Most patients have mild symptoms, so Sheehan's syndrome is not diagnosed and treated for a long time.

, fertility, blood pressure, breast milk production and many other life processes. Therefore, a lack of these hormones can cause problems throughout the body.

To better understand how damage to the pituitary gland is involved in Sheehan's syndrome, it is necessary to remember some notions about the hormones produced by it:

  • ACTH (adrenocorticotropic hormone): stimulates the adrenal glands to produce cortisol. ACTH deficiency results in a cortisol deficiency due to the hypoactivity of the adrenal glands.
  • Oxytocin: hormone necessary for labor, childbirth (stimulates contractions) and breastfeeding.
  • PRL (prolactin or lactotropic hormone): responsible for the production of milk by mothers after childbirth. Prolactin deficiency is associated with the reduction or total absence of lactation. In Sheehan's syndrome, lactation does not occur, as pituitary necrosis causes the destruction of the galactotropic cells responsible for the production of prolactin (PRL).
  • TSH (thyroid stimulating hormone or thyrotropic): regulates the production of hormones by the thyroid gland. The lack or deficiency of thyrotropic hormone affects the activity of the thyroid gland (in particular, the production of T3 and T4), resulting in hypothyroidism.
  • LH (luteinizing hormone) and FSH (follicle stimulating hormone): control fertility in both sexes (ovulation in women, sperm production in men) and stimulate the secretion of sex hormones from the ovaries and testes (estrogen and progesterone in women; testosterone in women). "man). In Sheehan's syndrome, post-pregnancy amenorrhea occurs due to the ischemic involvement of the area responsible for the production of pituitary gonadotropic hormones, with a lack of secretion of LH and FSH.
  • GH (growth hormone or somatotropic): it is necessary for development in children (bones and muscle mass), but has effects on the whole organism throughout life. In adults, growth hormone deficiency can lead to lack of physical energy , changes in body composition (increase in fat and decrease in muscle mass) and increased cardiovascular risk.
  • ADH (antidiuretic hormone or vasopressin): helps maintain a normal water balance. Deficiency of antidiuretic hormone (or vasopressin) affects the kidneys and can result in diabetes insipidus. This condition typically manifests itself with excessive thirst, diluted urine, and frequent urination (polyuria), particularly during the night.

Risk factors

Any condition that increases the chance of severe blood loss (bleeding) or low blood pressure during childbirth can increase the risk of Sheehan's syndrome.

The main risk factors include twin pregnancies and placental complications.

In women suffering from severe postpartum hemorrhage, other conditions that could play a role in the pathogenesis of Sheehan's syndrome include physiological hypertrophy of the pituitary gland in pregnancy (so the pituitary gland needs an increased blood supply to support its activity. ), disseminated intravascular coagulation and autoimmunity.

To know

Postpartum uterine haemorrhage is a rather rare complication and it should be noted that Sheehan syndrome occurs even less frequently. Both risks are greatly reduced with care and monitoring of the woman during labor and delivery.

It should also be noted that Sheehan's syndrome is not common in industrialized nations, largely due to improved obstetric care and the availability of rapid blood transfusions. The risk remains, however, for women in countries in way of development.

, or they manifest themselves with an abrupt onset hypopituitarism.

Manifestations of Sheehan's syndrome occur due to the deficiency of hormones produced by the pituitary gland. In addition to the production of breast milk, the effects of this dysfunction affect the activities of the thyroid and adrenal glands and the menstrual cycle.

The deficiency or absence of one, many or all of the pituitary hormones can lead to major changes in the body (with the exception of oxytocin and prolactin).

The clinical picture of Sheehan's syndrome can be clinically evident (i.e. the hormonal deficiency is clinically evident) or latent (it occurs only in certain situations, such as severe stress, or is detected only through some specific hormone tests).

Sheehan syndrome: how it manifests itself

Sheehan's syndrome can produce various signs and symptoms, which depend on which pituitary hormone is missing or deficient and include:

  • Difficulty or inability to breastfeed
  • Absence of menstrual flows (amenorrhea) or infrequent menstruation (oligomenorrhea);
  • Decrease in blood pressure values;
  • Low blood sugar (glucose) levels (hypoglycemia)
  • Irregular heartbeat;
  • Difficulty concentrating and sleepiness
  • Abdominal pain;
  • Tiredness;
  • Generalized swelling;
  • Cold intolerance;
  • Weight gain or, conversely, weight loss.

It should be noted that, for many women, the symptoms of Sheehan's syndrome are non-specific and are often attributed to other situations (for example: fatigue can be interpreted as a consequence of becoming a mother). There are cases, then, in which it is possible that no disturbances appear at all: the extent of the symptoms of Sheehan's syndrome are correlated, in fact, to the extent of the damage to the pituitary gland. Postpartum hypopituitarism can therefore affect the production of one or more hormones.

Some women live for years without knowing that their pituitary gland is not functioning properly. However, extreme physical stress, such as a severe infection or surgery, can suddenly trigger thyroid or adrenal insufficiency.

Complications

Pituitary hormones control many aspects of metabolism, so Sheehan's syndrome can cause a number of problems, including:

  • Adrenal crisis (severe condition in which the adrenal glands produce too little cortisol hormone)
  • Low blood pressure (hypotension);
  • Reduction of plasma sodium levels;
  • Menstrual irregularities;
  • Infertility;
  • Weakness and reduced exercise tolerance.

Adrenal crisis: a life-threatening situation

The most serious complication of Sheehan's syndrome is adrenal crisis, which is a sudden and life-threatening state in which extremely low blood pressure, shock, coma and death occur in succession.

. In gathering information related to the patient's medical history, it is important to mention any complications of childbirth, regardless of the time the baby was born, as disorders related to Sheehan's syndrome may have a late onset. Other useful anamnestic data to report to the doctor are amenorrhea and lack of milk production, two key signs of Sheehan's syndrome.
  • Blood and urine tests. Laboratory tests allow to check whether the levels of hormones secreted by the pituitary and their target organs are within the reference range (eg free thyroxine, TSH, prolactin, LH, FSH and testosterone in men or estradiol in women). If Sheehan's syndrome is suspected, a pituitary hormone stimulation test may also be required to assess the response of the gland. This test is usually done after consulting a physician who specializes in hormonal disorders (endocrinologist) Hormonal assays are performed on the blood and, sometimes, on the urine.
  • Neuroradiological examinations. To check the size of the pituitary gland or to rule out other possible conditions that could justify the symptom picture (such as a pituitary tumor), the patient may be subjected to imaging tests. In the majority of patients with Sheehan's syndrome, computed tomography (CT) High resolution and contrast-enhanced magnetic resonance imaging (MRI) demonstrate secondary empty sella syndrome.
  • Giulia Bertelli

    Graduated in Medical-Pharmaceutical Biotechnology, she worked as a Research and Development Officer in Food Supplements and Dietary Food companies
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