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Sheehan syndrome

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NORD is very grateful to Mark E. Molitch, MD, Professor of Medicine, Division of Endocrinology, Metabolism and Molecular Medicine, Northwestern University Feinberg School of Medicine, for assistance in the preparation of this report.

Synonyms of Sheehan syndrome

Disorder Subdivisions

General Discussion

Excessive blood loss during or after delivery of a baby may affect the function of the pituitary gland, leading to a form of maternal hypopituitarism known as Sheehan syndrome. Such extensive bleeding may reduce the blood flow to the pituitary gland and hence the amount of oxygen available to the cells of the brain and the gland. In such circumstances the pituitary cells may be damaged or die (necrosis) and thus the production of the usual pituitary hormones will be reduced, perhaps by a significant amount.

During pregnancy the pituitary gland will enlarge and may double in size. At this time the gland is especially vulnerable to "shock" and excessive maternal bleeding may induce the "shock" and the damage to the cells of the gland. At that time the balance of hormones produced by the pituitary may be thrown off giving rise to the symptoms associated with Sheehan syndrome.

There appear to be two forms of the disorder; a chronic form and an acute form, depending on the amount of damage to the gland's cells. The acute form reflects considerable damage so that symptoms become apparent soon after delivery. In chronic cases, the volume of damage is much less and symptoms may not appear for months or years after delivery.

Symptoms

The clinical features of Sheehan Syndrome are highly variable and depend on the degree of failure of secretion of pituitary hormones including:

Prolactin, the hormone that stimulates lactation
Gonadotrophin, which regulates the function of the gonads
TSH, which stimulates the thyroid gland
ACTH, adrenocorticotrophin, which stimulates the adrenal cortex
Growth hormone (GH)

How much pituitary tissue is killed, and by how much the hormone circulation is decreased, determines what happens to the mother. Patients with the chronic form have a smaller proportion of the pituitary tissue damaged and may not develop symptoms until weeks or even years after the birth.

In its most inclusive form, the condition is associated with failure of lactation after a woman has a baby. Menstruation does not begin again, sexual interest (libido) is diminished, pubic hair does not grow back, hair in the armpits (axilla) slowly disappears, and breasts and genitalia atrophy (diminish in size). For some women, menstrual periods do recur and subsequent pregnancies have been reported.

The characteristics of hypothyroidism usually develop gradually. A dry, waxy type of swelling (myxedema) may take years or decades to become apparent. Severe ACTH deficiency is associated with fatigue, chronic hypotension with fainting, and the inability to respond to stress. If these symptoms occur, they usually appear within weeks or months after the baby is born.

Since SS is a disorder affecting adults, the effects of growth hormone deficiency are limited to some loss of muscle strength, increased body fat and increased insulin sensitivity.

The 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 present with persistent low blood pressure (hypotension), irregular and fast heartbeat (tachycardia), as well as failure to lactate and low blood sugar (hypoglycemia).

In both the chronic and acute forms, there may be signs of diabetes insipidus (DI) such as abnormal thirst for and intake of water, as well as high volume of output of urine.

Causes

In most instances, a precipitous drop in blood pressure and consequent shock, due to obstetrical bleeding, precede the onset of symptoms. According to many physicians the amount of damage that must be done to the anterior pituitary before Sheehan Syndrome occurs varies from 75 to 90 percent. The enlarged pituitary requires more than normal volumes of oxygen, and any disruption of blood flow is a threat to the gland.

A severe spasm of the blood vessels feeding the pituitary (associated with shock) leads to lack of oxygen in the pituitary (pituitary ischemia) and various degrees of cellular damage depending on the severity and duration of arteriolar spasm.

Pituitary necrosis is found in association with other disorders but far less frequently. These disorders include sickle cell anemia, giant cell arteritis and a couple of others including trauma.

Affected Populations

Sheehan Syndrome affects women with excessive blood loss and circulatory collapse following childbirth. The incidence of Sheehan Syndrome is not known.

Related Disorders

Antiphospholipid Syndrome (APLS) is a rare autoimmune disorder characterized by recurring blood clots that usually appear before 45 years of age. It may also be associated with repeated spontaneous abortions for no apparent reason in young women. There may be a family history of blood clotting disorders in some cases. APS may occur in individuals with lupus or related autoimmune diseases or as a primary syndrome in otherwise healthy individuals.

Hypophysitis means inflammation of the pituitary gland. The irritation caused by the inflammatory reaction may interfere with the production of one or several of the pituitary hormones and in this respect hypophysitis may mimic, to some degree, the symptoms of Sheehan syndrome. Similarly, lesions of the pituitary gland that arise from pituitary adenomas, may also mimic Sheehan syndrome.

Standard Therapies

Diagnosis
In patients with severe hemorrhaging on delivery accompanied by long-lasting low blood pressure, treatment is started as soon as possible. Women believed to have the chronic form usually have blood drawn and the levels for several hormones are determined.

Standard Therapy
Treatment of Sheehan Syndrome consists of hormone replacement; i.e., ovarian, thyroid, and adrenocortical hormones (ACTH). Since in most cases ACTH deficiency is only partial, continuing cortisol replacement therapy may not be required. Hydrocortisone or prednisone are generally used to replace ACTH and cortisol; thyroxine replaces thyroid hormone; estrogen/progesterone replacement is usually achieved by administering oral contraceptives; while any indication of diabetes insipidus requires the use of demopressin. Growth hormone (GH) replacement therapy has been approved by the U.S. Food and Drug Administration (FDA) for adults with documented GH deficiency. Its use in cases of Sheehan Syndrome should be monitored and managed by a physician experienced in using GH. Benefits include increased muscle mass, reduction in the low density lipoproteins and an enhanced sense of well-being.

Information on replacement therapy in cases of hypopituitarism is readily available from:

The Pituitary Society
http://www.pituitarysociety.org/public

The Pituitary Foundation
http://www.pituitary.org.uk

The Hormone Foundation
http://www.hormone.org

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Organizations related to Sheehan syndrome

References

TEXTBOOKS
Molitch ME. Sheehan syndrome. In: NORD Guide to Rare Disorders. Philadelphia, PA: Lippincott Williams & Wilkins 2003.

Fauci AS, et al., eds. Harrison's Principles of Internal Medicine, 14th Ed. New York, NY: McGraw-Hill, Inc; 1998:2112-13.

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:216.

Suzuki K, Suzuki Y, Susuki K. Galactosylceramide Lipodosis: Globoid-Cell Leukodystrophy. In: Scriver CR, et al., eds. The Metabolic and Molecular Basis of Inherited Disease. 7th Ed. New York, NY; McGraw-Hill Companies, Inc; 1995:2671-92.

JOURNAL ARTICLES
Layden BT, Dubner S, Toft DJ, Kopp P, Grimm S, Molitch ME. Primary CNS lymphoma with bilateral symmetric hypothalamic lesions presenting with panhypopituitarism and diabetes insipidus. Pituitary. 2011 Jun;14(2):194-7.

Feinberg EC, Molitch ME, Endres LK, Peaceman AM. The incidence of Sheehan's syndrome after obstetric hemorrhage.Fertil Steril. 2005 Oct;84(4):975-9.

Syed AU, Al Figah MR, Fouda M. Coronary bypass surgery in patients with Sheehan's syndrome. Eur J Cardiothorac Surg. 2001;20:1264-66.

Schrager S, Sabo L. Sheehan syndrome: a rare complication of postpartum hemorrhage. J Am Board Fam Pract. 2001;11:245-49.

Huang YY, Ting MK, Hsu BR, et al. Demonstration of reserved anterior pituitary function among patients with amenorrhea after postpartum hemorrhage. Gynecol Endocrinol. 2000;14:99-104.

MacCagnanP, Oliveira JH, Castro V, et al. Abnormal circadian rhythm and increased non-pulsatile secretion of thyrotrophin in Sheehan's syndrome. Clin Endocrinol (Oxf). 1999;51:439-47.

Boulangier E, Pagniez D, Roueff S, et al. Sheehan syndrome presenting as early post-partum hyponatraemia. Nephrol Dial Transplant. 1999;14:2714-15.

Kan AK, Calligerous D. A case report of Sheehan syndrome presenting with diabetes insipidus. Aust N Z J Obstet Gynaecol. 1998;38:224-26.

Lavallee G, Morcos R, Palardy J, et al. MR of nonhemorrhagic postpartum pituitary apoplexy. AJNR Am J Neuroradiol. 1995;16:1939-41.

Zukor N, Bissessor M, Korber M, et al. Acute hypoglycaemic coma - a rare, potentially lethal form of early onset Sheehan syndrome. Aust J N Z Obstet Gynaecol. 1995;35:318-20.

Miki Y, Asato R, Okumura R, et al. Anterior pituitary gland in pregnancy: hyperintensity at MR. Radiology. 1993;187:229-31.

INTERNET
Corenblum, A. Pituitary Disease and Pregnancy. eMedicine. Last Updated: Jul 6, 2011.
www.emedicine.com/med/topic3264.htm Accessed on: January 26, 2012.

Report last updated: 2012/02/01 00:00:00 GMT+0