Sheehan's Syndrome
The most probable diagnosis is Sheehan's syndrome (option c), given the classic presentation of amenorrhea following primary postpartum hemorrhage requiring massive blood transfusion. 1, 2, 3
Clinical Reasoning
This patient presents with the pathognomonic triad for Sheehan's syndrome:
- History of severe postpartum hemorrhage requiring multiple blood transfusions 1, 2, 3
- Persistent amenorrhea since delivery (several years duration) 4, 5
- Negative pregnancy test excluding current pregnancy 5
The mechanism involves ischemic pituitary necrosis secondary to hypovolemic shock from massive PPH, as the enlarged pituitary gland during pregnancy is particularly vulnerable to hypoperfusion 1, 3, 4.
Why Other Options Are Less Likely
McCune-Albright syndrome (option a) presents in childhood with precocious puberty, café-au-lait spots, and polyostotic fibrous dysplasia—completely inconsistent with this postpartum presentation 1.
Acute tubular necrosis (option b) is a renal complication that may occur acutely after severe PPH but does not cause chronic amenorrhea years later 2.
Amenorrhea-galactorrhea syndrome (option d) typically involves elevated prolactin with galactorrhea; however, Sheehan's syndrome characteristically causes prolactin deficiency with agalactia (failure to lactate postpartum), not hyperprolactinemia 4, 5.
Asherman's syndrome (option e) involves intrauterine adhesions from uterine instrumentation (typically curettage), not from hemorrhage alone. This patient had no mention of postpartum curettage or instrumentation 5.
Key Diagnostic Features of Sheehan's Syndrome
The most sensitive early clinical indicators include 4, 5:
- Failure to lactate postpartum (agalactia)—present in essentially all assessable patients 4, 5
- Amenorrhea immediately following delivery—found in 55-76% of patients 4, 5
- Delayed diagnosis averaging 9-27 years after the causative delivery 4, 5
Critical Diagnostic Pitfall
Sheehan's syndrome is frequently underdiagnosed or diagnosed with significant delay (mean 9-27 years) even in developed countries 2, 5. The syndrome should be suspected in any woman with a history of severe PPH who develops signs of hypopituitarism, including 5:
- Persistent amenorrhea
- Failure to resume menses postpartum
- Hypothyroidism
- Chronic fatigue/asthenia
- Loss of axillary/pubic hair
Recommended Diagnostic Workup
For any woman failing to lactate after PPH, immediate evaluation should include 5:
- Prolactin levels (typically low or absent)
- Morning (0800h) ACTH and cortisol
- TSH and free T4
- IGF-1 levels
- LH and FSH
MRI findings typically show empty or partially empty sella turcica with a diminished, flattened pituitary gland 1, 3, 4.
Clinical Urgency
Early recognition is critical because untreated Sheehan's syndrome can lead to life-threatening adrenal crisis, particularly during physiologic stress 1, 2, 5. Appropriate hormone replacement therapy results in dramatic clinical improvement in cardiovascular function, energy levels, and prevention of acute adrenal insufficiency 1, 3.