Late Onset Sheehan Syndrome

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sheehan syndrome

Case report

Sheehan’s syndrome (SS) is a kind of maternal hypopituitarism brought on by significant blood loss during or following childbirth. This severe bleeding may cause the pituitary gland to get less blood. That could lead to pituitary cell death and damage (necrosis). While Sheehan’s syndrome is still a significant source of morbidity and mortality among at-risk people in underdeveloped nations. Its incidence has declined in affluent nations.

Sheehan’s syndrome can happen months or years after delivery, or even during the postpartum phase.
Different degrees of anterior and occasionally posterior pituitary gland abnormalities are characteristics of SS. Adrenal insufficiency, central hypothyroidism, hypogonadism, growth hormone deficiency, and lactation failure can all result from hormone deficits that start in the anterior pituitary gland. Furthermore, central diabetic insipidus may result from diseases affecting the posterior pituitary region.

The hormones produced by the adenohypophysis were implicated in the atypical presentation of SS in a lady who developed the condition 38 years after experiencing postpartum hemorrhage. Literature rarely describes late-onset appearance.

Case Presentation

A 59-year-old lady of Mestizo ethnicity, the patient has no medical history, a history of postpartum hemorrhage at age 21. And no postpartum symptoms, including amenorrhea or agalactia. She arrived at the emergency room on June 15, 2023, after experiencing asthenia, adynamia, vomiting, and disorientation for eight days. Initially, hypertonic saline and dextrose were used to treat the hypotension, hyponatremia, and hypoglycemia that were noted at admission.

Laboratory investigations revealed central adrenal insufficiency and thyrotropin levels within the normal range at the time. The internal medicine unit defined the clinical symptoms as hypo-osmolar euvolemic hyponatremia. The patient began receiving intravenous bolus of hydrocortisone.

After the patient’s diagnosis of central hypothyroidism, levothyroxine was administered as part of the treatment.
Reduced pituitary gland size and a conspicuous suprasellar cistern in relation to the sella turcica were observed on contrast-enhanced magnetic resonance imaging of the sella turcica, indicating a partially empty sella. Growth hormone deficiency and hypogonadotropic hypogonadism were found in the remaining pituitary axis. The diagnosis of late-onset SS was made based on hypopituitarism and a smaller pituitary gland.

Discussion

Since the earliest symptoms of SS are typically minor and inconsequential. Therefore, diagnosing the condition requires a high level of clinical suspicion. As a result, the diagnosis is probably going to be delayed, which will make identification more difficult and endanger the patient’s life. Symptoms may appear suddenly during the postpartum phase or years following delivery. Furthermore, hormone insufficiency symptoms might manifest 3–32 years postpartum and, in this instance, up to 38 years postpartum.

This example illustrates the significant difficulty in diagnosing SS and the value of inquiring about a patient’s gestational history, including any postpartum hemorrhage, which typically necessitates the use of blood products.

Prolactin and growth hormone secretion are most frequently impacted, followed by luteinizing hormone and follicle-stimulating hormone secretion, depending on the secretory cells’ anatomical placement within the vasculature. Adrenocorticotropic hormone and thyroid-stimulating hormone secretion may also be impacted by severe pituitary gland necrosis.

It is difficult to diagnose SS because its symptoms might vary. Furthermore, its features are determined by the amount of time that has passed between delivery and the commencement of SS. The most common symptoms are agalactia, amenorrhea, and asthenia in the short term (less than five years); syncope, pericardial effusion, and abdominal pain in the intermediate term (6 to 15 years); and hypoglycemia, hyponatremia, dilated cardiomyopathy, hypotension, and confusion in the long term (more than fifteen years).

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