Sheehan syndrome (HVL necrosis) is the term used to describe a deficiency of ACTH. It is caused by medications or by an alteration of the anterior pituitary gland and is nowadays easily treatable.
What is Sheehan syndrome?
Sheehan syndrome is a loss of function of the anterior pituitary gland, which usually occurs after childbirth. The cause is high blood loss and resulting cell death. Other shock conditions such as burns can also lead to HVL necrosis. The syndrome can be clearly diagnosed on the basis of various symptoms. Treatment is also possible thanks to modern medical techniques. Often, the anterior pituitary gland recovers on its own. Sheehan syndrome cannot normally be prevented. However, hemophiliacs can take appropriate precautions to avoid severe blood loss and associated shock.
Causes
Sheehan syndrome is caused by high blood loss with hypovolemic shock. This results in an inferior hemorrhage and, a short time later, ischemic-related necrosis of the anterior pituitary. The exact cause of the shock can be quite different. Most often, the syndrome occurs as a result of childbirth, where blood loss is particularly high. Injuries that result in high blood loss also lead to necrosis. These can be burns, severe cuts and similar things. However, acute disease is usually the result of childbirth. Bleeding directly from the anterior pituitary gland can also cause Sheehan’s syndrome. It can result from a head injury and is often not noticed at all by affected individuals. It is only when headaches and hormonal problems occur that affected individuals can intervene. Surgery is then an effective way to remove the bleeding and relieve the discomfort.
Symptoms, complaints and signs
Sheehan syndrome occurs primarily after a complication-laden delivery of a child with high blood loss in the mother. Symptomatically, it presents with the following complaints: After failure of the anterior pituitary gland, there is an absence of milk let-down (agalactorrhea) because there is no longer a secretion of prolactin. Other symptoms include absence of menstruation (secondary amenorrhea) due to loss of the hormones follicle-stimulating hormone (FSH) and luteinizing hormone (LH), hypothyroidism, an abnormally increased urine excretion (polyuria) as well as an abnormally increased thirst (polydipsia), a tendency to hypoglycemia (hypoglycemia) due to the loss of growth hormone and the deficiency of adrenocorticotropin (a hormone that is also responsible for growth). There may also be loss of secondary body hair, lack of sexual desire (loss of libido), pallor of the skin (hypopigmentation) due to the absence of melanocyte-stimulating hormone, and in rare cases, short stature during growth. In Sheehan’s syndrome, the anterior pituitary gland is partially or totally dysfunctional and therefore many of the necessary hormones cannot be produced. Often patients are apathetic, indifferent and limited in their circumstances before diagnosis. In rare cases, Sheehan syndrome can also lead to secondary adrenocortical insufficiency.
Diagnosis and course
Although Sheehan syndrome is a serious impairment, diagnosis can be difficult. It often occurs years later, but it can be made in the postpartum period. A clear sign is a loss of secondary body hair. Lack of sexual desire and pallor of the skin can also be symptoms. The same applies to the absence of the milk supply. In most cases, this is the first symptom triggered by the loss of prolactin secretion. Loss of the hormones FSH and LH, which are associated with necrosis of the anterior lobe of the pituitary gland, results in absence of menstruation. Hypothyroidism is also a common symptom. Likewise, a tendency to hypoglycemia may be an indication. The clearest sign, however, is discomfort in the area of the eyes. If visual disturbances occur after delivery, Sheehan syndrome is suspected. The reason for this is the course of the optic nerve, which is located in the immediate vicinity of the pituitary gland.Disturbances in eye mobility are also known symptoms. The exact diagnosis is made by a physician. The doctor examines the blood count, obtains information about the patient’s medical history and excludes other diseases as the cause. The diagnosis can then be made relatively quickly. The course of Sheehan’s syndrome can vary greatly. In some cases, the damaged area of the body recovers on its own. In other cases, the deficiency can lead to serious disorders, even coma. Early treatment is therefore essential.
Complications
The complications and symptoms of Sheehan syndrome depend very much on its severity, so a general course cannot usually be predicted. However, affected individuals primarily suffer from failure to express milk if the patient has been pregnant. This can lead to growth disturbances in the child, although the absence of milk may be replaced by other products. Menstruation is also negatively affected due to Sheehan’s syndrome, and bleeding may stop completely. Affected individuals continue to suffer from significant hypothyroidism, which has a negative impact on health. There is hair loss and sexual unwillingness. The sexual disorders can have a very negative effect on the relationship with the partner and possibly lead to social discomfort. In many cases, the skin of the patients is very pale. If Sheehan syndrome occurs in childhood, it often leads to short stature. As a rule, Sheehan’s syndrome can be treated relatively easily with the administration of hormones. There are no particular complications and in most cases the course of the disease is positive. The life expectancy of those affected is also usually not negatively affected by the syndrome.
When should one go to the doctor?
Sheehan syndrome always requires treatment by a physician. Only early detection and treatment of the symptoms can limit or completely prevent further complications. The earlier Sheehan syndrome is detected, the better the further course of this disease. A doctor should be consulted if the affected person suffers from a significant underactivity of the thyroid gland. This is usually noticeable through various complaints and symptoms and should always be treated by a doctor. Furthermore, permanent hypoglycemia or very slow growth in children may also indicate the disease and must also be examined by a doctor. Insufficiency or other kidney complaints also often indicate Sheehan’s syndrome and should be investigated if they occur over a long period of time and do not go away on their own. Self-cure usually cannot occur with Sheehan syndrome. Sheehan syndrome can be detected by a general practitioner. However, further treatment depends on the exact symptoms and is then carried out by a specialist.
Treatment and therapy
After the diagnosis has been clearly established with the help of a blood test, Sheehan syndrome can be effectively treated. An important pillar of the therapy is the supply of the missing hormones. This is done by adding various preparations. Furthermore, secondary diseases that have already occurred must be treated, which sometimes takes a lot of time. The hormonal changes can also cause psychological problems that make treatment more difficult. It is then necessary to refer the patient to a psychiatrist. In most cases, however, it is sufficient to supply the missing hormones in order to restore the normal condition. Sheehan’s syndrome can also be treated surgically. In particular, if the pituitary gland is injured, neurosurgery can bring improvement. The surgery is performed through the nose and is very promising.
Prevention
Because Sheehan syndrome usually results from sudden blood loss, it is difficult to prevent. However, it is possible to closely monitor delivery and especially anterior pituitary function to prevent necrosis from occurring in the first place. In the case of an accident, of course, this is not possible. However, if initial symptoms are detected early, the syndrome can be diagnosed before the tissue is completely dead.Often it is then possible to restore the function through therapy. Lastly, a doctor should be consulted in the event of falls on the head. Magnetic resonance imaging allows injuries to the pituitary gland to be detected at an early stage and subsequently treated. This will at least prevent secondary diseases.
Follow-up care
In Sheehan syndrome, the patient usually has only very limited and also very few measures of direct aftercare available. The affected person should therefore ideally consult a physician at the first symptoms and signs of the disease to prevent the occurrence of other complaints and complications. The earlier a doctor is consulted, the better the further course of the disease usually is. Since Sheehan syndrome is a genetic disease, it usually cannot be completely cured. Therefore, if the affected person wishes to have children, he or she should undergo genetic testing and counseling to prevent the recurrence of the syndrome in the children. In most cases, sufferers of Sheehan syndrome are dependent on taking various medications that can limit the symptoms. It is always important to ensure that the medication is taken regularly and also in the correct dosage to alleviate the symptoms. If there are any uncertainties or questions, a doctor should be consulted first. The disease itself does not limit the life expectancy of the patient.
This is what you can do yourself
For the treatment of Sheehan syndrome, special preparations are used, which replace the missing hormones. In the context of self-help, it is also a matter of considering the subsequent secondary diseases. These are often psychological complaints that are closely linked to the therapy itself and place a heavy burden on the patients. Targeted therapy with a psychiatrist helps those affected to come to terms with their situation. It can take place over a long period of time and promotes the patient’s self-esteem. At the same time, those affected should observe their own bodies so that they can recognize any changes at an early stage. In this way, serious accidents or falls can be avoided. Such situations can be avoided by regularly taking the prescribed hormones. This drug therapy can be easily integrated into everyday duties. Thus, the usual bodily functions are ensured and the affected person can lead a normal life. A good contact with the doctor is also helpful. Patients should make an appointment at short notice in case of already known difficulties as well as in case of newly emerging problems. In this way, worse consequences can be avoided at an early stage.
