Sheehan Syndrome Definition
Sheehan Syndrome is a condition characterized by hypopituitarism or decreased functioning of pituitary gland which occurs as a result of ischemic necrosis caused by hypovolemic shock and blood loss during as well as after childbirth.
This syndrome is also referred to as:
- Sheehan’s Syndrome
- Simmonds’ syndrome
- Postpartum pituitary necrosis
- Postpartum panhypopituitarism
- Postpartum panhypopituitary syndrome
- Postpartum hypopituitarism
Pituitary damage that is not related to pregnancy is known as Simmonds’ disease.
Sheehan Syndrome ICD-9 Code
The ICD-9 Code for this syndrome is 253.2.
Sheehan Syndrome Epidemiology
The prevalence of this disease was around 10 to 20 in per 100000 women around 50 years ago. With developments in medical research and obstetrics, the current rate of occurrence of this condition is around 5 in per 100000 women. The mean age of diagnosis is around 36.6 years. Although improvements in obstetrics have brought down the figures of women suffering from this syndrome, the condition can be noticed both in underdeveloped countries as well as in first-world countries that are equipped with the most state-of-the-art developments in medical science.
Sheehan Syndrome Causes
Sheehan syndrome occurs as a very rare pregnancy-related complication. Although many health problems can give rise to lowered pituitary functions, this condition normally develops after too much loss of blood during or after the childbirth. Blood loss at these stages can be particularly detrimental to pituitary gland, damaging the hormone-producing tissues which disrupt the normal functioning of the gland.
The pituitary gland can be found at the base of the brain. Its function is to produce hormones that facilitate growth, assist in reproductive functions and production of breast milk, regulate metabolism, heal wounds as well as stimulate the thyroid and adrenal glands. An absence or lack of the above mentioned hormones may give rise to numerous types of symptoms.
Although the exact causes that give rise to this condition is not known, medical experts suspect certain probable influences. These include:
- Signs of disseminated intravascular coagulation such as in HELLP syndrome or amniotic fluid embolism
- Enlargement of the pituitary glands during pregnancy
- Severe drop in the blood pressure or hypotension that happens during hemorrhage
Sheehan Syndrome Pathophysiology
Hyperplasia and hypertrophy of the lactotrophs during pregnancy leads to the enlargement of anterior pituitary, without corresponding increase in the supply of blood. Secondly, the anterior pituitary gland of patients is supplied by one low-pressure portal venous system. When these vulnerabilities are affected by hypotension or a major hemorrhage during peripartum period, it can lead to ischemia of affected pituitary regions thereby giving rise to necrosis. The posterior pituitary gland of patients is generally unaffected because of the direct arterial supply to it.
Sheehan Syndrome Risk Factors
There are certain conditions that may increase the propensity of bleeding during or after childbirth. These include multiple pregnancies (i.e. having twins or triplets) and/or abnormalities of the placenta.
Sheehan Syndrome Symptoms
In some instances, women suffering from Sheehan syndrome may be relatively asymptomatic, thereby making a diagnosis difficult until the later years. Sometimes, signs of Hypopituitarism might be present. Patients of this syndrome may exhibit the following symptoms:
- Hair loss
- Hot flushes
- Visual field loss
- Slowed thinking
- Decreased libido
- Slowed heart rate
- Intolerance to cold
- Low blood pressure
- Adrenal insufficiency
- Extreme hypovolemia
- Decreased muscle mass
- Gonadotropin deficiency
- Difficulty in staying warm
- Coma (in some rare cases)
- Weight loss or weight gain
- Optic chiasm compression
- Secondary hypothyroidism
- Growth hormone deficiency
- Thyrotrophin or TRH deficiency
- Loss of pubic and/or axillary hair
- Hyponatremia or low sodium levels
- Adrenocorticotrophin or ACTH deficiency
- Hypoglycemia or lowered blood sugar levels
- Decreased free water clearance caused by glucocorticoid deficiency
- Secondary adrenal insufficiency that may resemble Addison’s disease
- Agalactorrhea or absence of lactation or other difficulties with lactation
- Syndrome of inappropriate antidiuretic hormone or ADH hypersecretion
- Acute exacerbation caused by stress that occurs due to surgery or severe infection developing years after delivery, a condition that is equivalent to an Addisonian crisis
Sheehan Syndrome Diagnosis
Diagnosing this syndrome can prove to be challenging. Doctors may inquire about the medical history of patient. Naturally, it is very important to cite any childbirth complications the patient may have had before. Problems relating to menstruation and/or lactation are required to be mentioned as well, as they are the two key symptoms of Sheehan’s syndrome.
If the signs and symptoms indicate pituitary insufficiency, doctors may recommend having blood tests done to check the pituitary hormone levels. Laboratory tests may show decreased levels of the following hormones:
- Thyroid stimulating hormone (TSH)
- Adrenocorticotropic hormone (ACTH)
- Follicle stimulating hormone (FSH)
- Luteinizing hormone (LH)
- Growth hormone (GH)
Patients may require diagnostic evaluation of pituitary hormones by specialized stimulation testing, which is generally done after consultation with an endocrinologist. Imaging tests, like MRI (magnetic resonance imaging) and/or CT (computerized tomography) scans may be required to evaluate the size of the pituitary gland as well as to check if there are any signs of other abnormalities, such as pituitary tumors.
Sheehan Syndrome Differential Diagnosis
A number of conditions may show signs and symptoms similar to that of Sheehan syndrome. Hence, while determining the presence of this condition, a doctor must differentiate it from such similar conditions so that proper treatment measures can be taken.
The differential diagnoses of Sheehan Syndrome include distinguishing its symptoms from those of conditions such as:
- Pituitary ischemia
- Pituitary adenoma
- Sellar mass apoplexy
- Macroadenoma apoplexy
- Lymphocytic hypophysitis
- Pituitary adenoma necrosis
- Subarachnoidal hemorrhage
Sheehan Syndrome Treatment
Treatment for this condition is carried out by hormone replacement therapy that is to be continued throughout life. The doctor might recommend any or all of the following medicines:
Drugs like prednisone or hydrocortisone can replace adrenal hormones that are not being manufactured due to the deficiency of an adrenocorticotropic hormone (ACTH). Adjustments in the medications might be required if the patient becomes seriously ill or experiences major physical distress. In such times, the body will generally produce extra amounts of a stress hormone called cortisol. A similar kind of fine-tuning of the dosage is required when the patient is having flu, diarrhea, is vomiting or having dental or surgical procedures. The dosage of corticosteroid medications may also require adjustments during months of pregnancy or when the patient has undergone marked weight loss or weight gain. Close monitoring of a patient’s condition is necessary to make sure that the correct dose is administered. Avoiding doses that are higher than the required amount will eradicate the side effects that are associated with the higher doses of corticosteroids.
The doctor may recommend using estrogen medications alone if the patient has undergone a hysterectomy (removal of uterus) procedure. Alternatively, a combination of progesterone and estrogen can be administered if the patient is still having her uterus. Estrogen replacement therapy can be carried out by using either patches or pills. Possibilities of future pregnancies might be rendered by administering preparations that include follicle stimulating hormone (FSH) and luteinizing hormone (LH), also collectively known as gonadotropins. They can be given by injections to stimulate ovulation.
Medicines like Levoxyl, Synthroid and others can boost the levels of deficient thyroid hormones which are caused by deficient or low thyroid-stimulating hormone production. Using Levothyroxine is a favorable option as these medications practically cause no side effects if applied in appropriate doses and is relatively inexpensive. The doctor should be informed if the medication brands are changed in order to make sure the right dosage is still maintained. The patient should also not skip doses or discontinue taking the drug just because she is feeling better, as this may gradually bring back the signs and symptoms.
Research has shown that treating patients of Sheehan syndrome as well as other variants of hypopituitarism can assist in bringing down the cholesterol levels, normalize the muscle to fat ratio of the body and improve the quality of life of sufferers.
An endocrinologist overseeing the treatment is most likely to test a patient’s blood regularly in order to make sure that she is getting appropriate but not excessive doses of required hormones. The hormone levels are generally checked after every couple of weeks or months during the earlier stages of treatment and then on a once-per-year basis.
Sheehan Syndrome Complications
As the pituitary hormones regulate numerous aspects of the metabolism of an individual, Sheehan syndrome can give rise to a number of complications, such as:
- High cholesterol
- Low blood pressure
- Menstrual problems
- Unintended weight loss
The most serious type of complication resulting from this syndrome is Addison’s disease, Addisonian crisis or adrenal crisis. It gives rise to a sudden, life-threatening situation that can result in very low blood pressure, coma, shock or even death. Adrenal crisis generally occurs when the body is under significant stress, like during a serious illness or surgery. In such situations, the adrenal glands are able to produce very little amounts of cortisol, a potent stress hormone.
Certain medications can have adverse side effects as well. Estrogen, when combined with progesterone, increases the risk of heart diseases as well as strokes and breast cancer. When used alone, Estrogen has been associated with abnormalities on mammograms, strokes and blood clots in legs.
Sheehan Syndrome Prognosis
The outcome for this condition is normally excellent with an early diagnosis and appropriate treatment. However, things might become critical with the development of adverse complications. Death or serious life-threatening disability may occur under such circumstances.