(5.07) Late Onset Sheehan Syndrome Presenting With Panhypopituitarism

Sheehan syndrome (SS) is caused by ischemic pituitary necrosis from severe postpartum bleeding. Pregnancy enlarges the pituitary gland due to lactotroph hyperplasia, making it prone to ischemia in the confined space of the sella turcica. We hereby report a rare case of SS diagnosed 18 years after vaginal delivery.

Case(s) Description :

A 38-year-old female with unknown history arrived at the emergency room (ER) with altered mental status, confusion, vomiting, and a fall. EMS found her blood glucose below 20 mg/dl.She had no prior history of diabetes, thyroid or adrenal disease.Her vitals were BP 80/64 mmHg, HR 102 bpm.She was alert but confused, without hyperpigmentation of skin, and had repeated episodes of hypoglycemia.Further tests showed serum sodium 128 mmol/l (136-145 mmol/l), potassium 3.8 mmol/l (3.5-5.1 mmol/l), TSH 0.327mIU/ml (0.35-4.9 mIU/ml), free T4 0.54ng/dl (0.89-1.76 ng/dl).Random cortisol was 2.8µg/dL (3.7-19.4 µg/dL).Cosyntropin stimulation test showed baseline cortisol 2.5 µg/dL, at 30 minutes 5 µg/dL and at 60 minutes 6 µg/dL consistent with adrenal insufficiency and started on stress dose hydrocortisone. ACTH was 2.4pg/ml (7.2-63.3 pg/ml), prolactin 1.05ng/ml (2.8-29.2 ng/ml), FSH 0.5mIU/ml, LH < 0.3mIU/ml, IGF-1 13ng/ml (79-259 ng/ml), DHEAS 1.1ug/dl (57.3-279.2 ug/dl). She was started on levothyroxine.MRI of the pituitary showed an abnormal appearance of the pituitary with a curvilinear lack of enhancement between the anterior and posterior aspects of the pituitary.On her 3rd hospital day, her mental state improved. She reported a history of chronic fatigue, secondary amenorrhea and inability to lactate since her child’s birth 18 years ago.She sought medical attention for these symptoms, but a proper diagnosis was not made.A year ago, she was hospitalized for COVID and adrenal crisis, started on hydrocortisone and levothyroxine but didn’t follow-up.She restarted levothyroxine a week before this admission. She was diagnosed with panhypopituitarism secondary to SS, discharged on prednisone and levothyroxine with plan to start hormone replacement therapy as outpatient.

Discussion :

SS, prevalent in the developing world, accounts for 6% of hypopituitarism cases and is the most common cause in females.Over half of these patient have panhypopituitarism. Symptoms are subtle and progressive, requiring vigilance for diagnosis. The purpose of our case report is to consider SS as a diagnosis in women who develop non-specific symptoms or classic symptoms of amenorrhea and agalactia after childbirth. One retrospective study found a mean delay in the diagnosis of SS was 9 ± 9.7 years due to nonspecific symptoms. The treatment of SS is lifelong replacement of deficient hormones.