Sheehan’s Syndrome - Causes, Symptoms, Diagnosis, and Treatment

Introduction:

Sheehan's syndrome is a condition exclusively associated with pregnancy. It occurs due to excess loss of blood during or after childbirth. This extra blood loss causes damage to the pituitary gland and leads to hypopituitarism. The condition's symptoms are variable and reflect at different times depending upon the degree of damage to the pituitary gland. In addition, Sheehan's syndrome affects the production of several hormones. This condition is therefore also known as post-partum hypopituitarism or post-partum pituitary gland necrosis.

What Is Sheehan's Syndrome?

Sheehan's syndrome is a condition in which excessive blood loss during or after childbirth may lead to dysfunctioning of the pituitary gland and cause maternal hypopituitarism. Sheehan's syndrome is also known as post-partum pituitary gland necrosis. The blood loss during or after childbirth causes reduced blood flow and deprives the pituitary gland of the oxygen it requires to function correctly. Thus, the production of pituitary hormones is reduced by a significant amount. Sheehan's syndrome affects the production and release of the following hormones:

1. Thyroid-Stimulating Hormone (TSH)- This hormone instructs the thyroid gland to release its hormones.

2. Luteinizing Hormone (LH)- This hormone works with follicle-stimulating hormone in menstrual cycle regulation and egg production.

3. Follicle-Stimulating Hormone (FSH)- Menstrual cycle regulation and egg production in females.

4. Growth-Hormone (GH)- This hormone controls the growth and development of the body, especially in bones and muscles.

5. Adrenocorticotropic Hormone (ACTH)- Stimulates the adrenal gland to release its hormone-like cortisol, etc.

6. Prolactin- This hormone helps in milk production during lactation.

What Is the Common Cause of Sheehan's Syndrome?

During pregnancy, the pituitary gland increases in size to almost double. This is due to increased estrogen levels in the body. Therefore, blood loss makes the pituitary gland more susceptible to damage. Excess blood loss during or after childbirth can cause a severe drop in blood pressure, may induce shock, and cause damage to the cells of the pituitary gland. A severe spasm of the blood vessels supplying the pituitary gland leads to a lack of oxygen in the gland. In addition, necrosis may occur because of severe hypotension due to excessive uterine bleeding postpartum. Usually, Sheehan's syndrome affects the anterior part of the pituitary gland. There are two forms of the disorders; acute and chronic. The acute form reflects considerable damage to the gland cells, so the symptoms become apparent very soon. In the chronic form, there is considerably less amount of blood loss. Therefore, the symptoms may not appear for months after delivery. Multiple factors also contribute to the condition:

1. Anemia.

2. Obesity.

3. Advanced maternal age.

4. Failure of the uterus to contract after childbirth.

5. Placental abruption (placenta detached from the uterus).

6. When the placenta completely covers the cervix.

7. Giving birth to a large baby or multiple babies at one time.

8. High blood pressure during pregnancy.

9. Delivery of child at home or assisted delivery like forceps delivery or vacuum-assisted delivery.

How Much Blood Loss Will Cause Sheehan's Syndrome?

Sheehan's syndrome occurs in one to two percent of women who lose about one to two liters of blood during childbirth.

Who Is Affected by Sheehan's Syndrome?

Sheehan's syndrome is rare in developed countries like the United States, where good medical and maternal care is available during and after childbirth. However, this condition can still affect women in developing countries with inadequate medical help. In addition, women who give birth at home are also more susceptible to this condition. However, the exact incidence of the disease is still unknown because of the fewer reported cases.

Is Sheehan's Syndrome Inherited?

Sheehan's syndrome is not a hereditary condition, meaning it is not genetically passed from parents to their children. Instead, this condition is exclusively associated with pregnancy and its complications.

What Are the Symptoms of Sheehan's Syndrome?

The symptoms and clinical features of Sheehan's syndrome are highly variable. In addition, the symptoms may reflect at different times depending upon the severity of the damage done to the pituitary gland. Some women remain asymptomatic for years after the delivery. However, the most common symptoms of Sheehan's syndrome are:

1. Difficulty in milk production and inability to feed.

2. Menstruation does not begin again or in irregular cycles.

3. Decreased sex drive.

4. Armpit hair slowly disappears.

5. Breasts get smaller in size.

6. Fatigue and sluggishness.

7. Dry skin.

8. Weight gain.

9. Constipation.

10. Joint pain.

11. Low blood sugar level.

12. Low blood pressure.

13. Irregular heartbeat.

14. Increased thirst and frequent urination.

15. Intolerance to cold.

How Is Sheehan's Syndrome Diagnosed?

The symptoms of Sheehan's syndrome cannot be the only diagnostic criteria because of the similarity of the syndrome to many other diseases. Therefore, the diagnosis is only partially based on the patient's medical history and symptoms. The following tests are done to confirm the diagnosis:

1. Blood Tests- Thyroxine levels, thyroid-stimulating hormone (TSH), estrogen, gonadotropin, cortisol, and adrenocorticotropic hormone (ACTH) are checked to determine if the pituitary gland functions correctly. The extent of necrosis of the gland is also determined by assaying these hormones.

2. Hormone-Stimulation Test- This test checks how well the pituitary gland responds to different hormones.

3. Imaging Tests- Computed tomography scan (CT scan) or magnetic image resonance (MRI) may also be done to check the presence of any pituitary gland tumor causing similar symptoms. Also, these imaging tests help evaluate the size and structure of the pituitary gland.

How Is Sheehan's Syndrome Treated?

Lifelong replacement of the hormones is the standard and definitive treatment of Sheehan's syndrome. These will be the hormones that the body is no longer producing. The following hormones are given:

1. Corticosteroids- Prednisone or Hydrocortisone are given as a replacement for adrenal hormones.

2. Levothyroxine- These hormones are given as a replacement for thyroid hormones.

3. Estrogen and Progesterone- These hormones help regulate the absent or irregular menstrual cycles.

4. Luteinizing Hormone and Follicle-Stimulating Hormone- These hormones will regulate ovulation and treat infertility after Sheehan's syndrome.

5. Growth Hormone- These hormones are also given after Sheehan's syndrome to improve bone density, balance muscle to fat ratio in the body, and lower cholesterol levels.

What Are the Complications of Sheehan's Syndrome?

If left untreated, Sheehan's syndrome can cause the following compilations:

1. Persistent low blood pressure.

2. Irregular and missing periods.

3. Infertility.

4. Adrenal crisis (this is a life-threatening condition where adrenal glands do not produce enough adrenal hormones).

What Is the Prognosis of Sheehan's Syndrome?

The prognosis for Sheehan's syndrome is excellent. However, the patient will require hormonal replacement therapy for the rest of their life. If the patient is on steroids, they must carry emergency Hydrocortisone injections. They should also wear medical identification and alert jewelry stating they are on steroid medications.

Conclusion:

Sheehan's syndrome is a rare postpartum complication leading to pituitary gland dysfunction. It is the sixth most common cause of growth hormone deficiency in children. This condition is more common in developing countries where medical facilities are compromised. In addition, treatment of the syndrome is lifelong thyroid replacement therapy. If left untreated, the condition can worsen and become life-threatening. However, the prognosis of Sheehan's syndrome is good. The condition is manageable with continued hormone replacement therapy, and the individual affected by the syndrome can lead a normal and healthy life.