In addition to today’s resource, please carefully review A Case Report of Sheehan Syndrome: A Rare Cause of Hypopituitarism. [PubMed Abstract] [Full-Text HTML] [Full-Text PDF]. Cureus. 2024 Feb 4;16(2):e53544. doi: 10.7759/cureus.53544. eCollection 2024 Feb.
The above article reminds us that panhypopituitarism secondary to Sheehan Syndrome can go undiagnosed for years. It is critical to make this diagnosis and the above article will help.
Today, I review, link to, and excerpt from StatPearls‘ Sheehan Syndrome. Mark P. Schury1; Rotimi Adigun2. Last Update: September 4, 2023.
All that follows is from the above resource.
- Continuing Education Activity
- Introduction
- Etiology
- Epidemiology
- Pathophysiology
- History and Physical
- Evaluation
- Treatment / Management
- Differential Diagnosis
- Prognosis
- Complications
- Deterrence and Patient Education
- Enhancing Healthcare Team Outcomes
- Review Questions
- References
Continuing Education Activity
Sheehan syndrome which is also called post-partum pituitary necrosis refers to the necrosis of cells of the anterior pituitary gland following significant post-partum bleeding, hypovolemia, and shock. Advances in obstetrical care in developed countries have reduced the incidence of this condition. However, it is still a significant cause of morbidity and mortality in less developed countries. This activity reviews the evaluation and management of Sheehan syndrome and discusses the role of interprofessional team members in collaborating to provide care and enhance patient outcomes.
Etiology
Sheehan syndrome occurs when the anterior pituitary gland is damaged following significant blood loss. Classically, this condition develops after delivery complicated by severe maternal hemorrhage. The resulting ischemic injury impairs anterior pituitary hormone production. Diagnosis of Sheehan syndrome is not always apparent immediately after childbirth. Occasionally, signs and symptoms may not develop until months after delivery or another significant traumatic event. The first and most common symptom of Sheehan syndrome is an inability to lactate, also known as agalactorrhea. Other symptoms are associated with reduced pituitary hormone production and may include amenorrhea or oligomenorrhea, hot flashes, and decreased libido. Symptoms of hypothyroidism, such as fatigue, bradycardia, hypotension, weight gain, and constipation, may occur months later, along with loss of axillary and pubic hair. Adrenal insufficiency can also occur, with symptoms including fatigue and weight loss. Laboratory findings that may support a diagnosis of secondary adrenal insufficiency associated with Sheehan syndrome include hyponatremia, anemia, and hypoglycemia.[2][3]
Epidemiology
Sheehan syndrome is rare in high-resource countries because of advances in obstetric care and greater access to experienced healthcare professionals and medical facilities. However, the disorder remains a complication of childbirth in resource-limited and low-income countries. Results from some studies estimated the incidence of Sheehan syndrome at up to 5 cases per 100,000 births.[1]
Pathophysiology
During pregnancy, pituitary gland volume and cell number increase in the weeks preceding delivery. This increase is caused primarily by hyperplasia of prolactin-producing cells (lactotrophs) and other anterior pituitary cells. The resulting hyperplasia increases the nutritional and metabolic demands of the anterior pituitary gland without a corresponding increase in its blood supply. The anterior pituitary receives blood through a relatively low-pressure system, which may increase the susceptibility of pituitary cells to ischemia. Consequently, anterior pituitary cells are more prone to necrosis in pregnancies complicated by significant postpartum hemorrhage. The posterior pituitary gland receives a separate, higher-pressure blood supply and is therefore usually unaffected by shock or hypovolemia.[4]
Sheehan syndrome is the clinical manifestation of anterior pituitary cell necrosis and may present as panhypopituitarism or selective loss of pituitary function. Panhypopituitarism results when extensive pituitary cell necrosis occurs and is more common than selective loss of pituitary function. Prolactin and growth hormone are the hormones most commonly affected by selective pituitary necrosis and hypofunction. Posterior pituitary function is usually preserved. However, diabetes insipidus can occur as a rare manifestation of Sheehan syndrome.[5]
History and Physical
PPatients may present with acute or chronic manifestations of Sheehan syndrome. The chronic form is more common than the acute form. Diagnosis may be delayed because Sheehan syndrome can develop months to years after the initial event of hypovolemia and shock.[2]
The acute presentation of Sheehan syndrome is usually evident when the mother has difficulty breastfeeding or is unable to produce breast milk, a condition known as agalactorrhea.[6] However, many women remain asymptomatic for months to years after childbirth. Women may report menstrual irregularities after childbirth, and some may remain amenorrheic until Sheehan syndrome is diagnosed and treated. The acute form of Sheehan syndrome can be life-threatening if not recognized and treated promptly after childbirth. Persistent hypotension and tachycardia can mimic hypovolemia and shock, but hyponatremia and persistent hypoglycemia can support the diagnosis of Sheehan syndrome.[1]
Chronic Sheehan syndrome is diagnosed based on manifestations of hypopituitarism and may develop months to years after the initial vascular insult to the pituitary gland. Symptoms of hypothyroidism may occur months after childbirth. Patients may report fatigue, asthenia or weakness, hair loss, constipation, weight gain, difficulty concentrating, impaired attention, and cold intolerance. Clinicians may identify hypotension and bradycardia during the physical examination. Patients may also develop secondary adrenal insufficiency, with symptoms including fatigue and weight loss.[7] Laboratory findings may reflect hypoglycemia, hyponatremia, and/or anemia.
Treatment / Management
The basis of treatment for Sheehan syndrome is lifelong replacement of deficient hormones. Hypothyroidism can be treated with levothyroxine or liothyronine replacement therapy. Cortisol deficiency can be treated with prednisone or hydrocortisone replacement. Gonadotropin deficiency should be treated with estrogen alone if the uterus has been removed and with a combination of estrogen and progesterone if the uterus is present. Clinicians should discuss the risks and benefits of hormone replacement with each patient, including the risks associated with thrombophilia, cancer, and myocardial infarction. Growth hormone is probably the most common hormone that requires replacement, and the dosage must be individualized according to the patient’s needs. For patients who develop diabetes insipidus, desmopressin is the recommended treatment. Referral to an endocrinologist experienced in treating hypopituitarism and using growth hormone is recommended.[12]
Differential Diagnosis
The main differential diagnoses include:
Prognosis
The prognosis depends on timely diagnosis and hormone supplementation. If not diagnosed promptly, the prognosis can be grave.
Complications
Complications include:
Deterrence and Patient Education
Treatment requires lifelong replacement of deficient hormones. Pharmacists and nurses should educate patients about medication adherence because missed doses can precipitate life-threatening hormonal crises. Patients require lifelong care and close monitoring of hormone levels.[13][14]
Enhancing Healthcare Team Outcomes
Sheehan syndrome is rare but is associated with substantial morbidity. Therefore, an interprofessional team should coordinate care and may include an endocrinologist, neurologist or neurosurgeon, obstetrician, internal medicine clinician, primary care clinician, nurse practitioner, and radiologist.
