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Sheehan Syndrome: A Rare Complication of J Am Board Fam Pract: first published as on 1 September 2001. Downloaded from Postpartum Hemorrhage

Sarina Schrager, MD, and Laura Sabo, MD

Life-threatening postpartum hemorrhage is un- gency Department. She started feeling nauseous common as a result of the advances in obstetric the last 2 days of her initial hospitalization. The care. As this case illustrates, however, the conse- nausea worsened, and she developed , di- quences of postpartum hemorrhage can be severe. arrhea, dizziness, and . In the Emergency Department, the patient was Case Report normovolemic. Her blood pressure, pulse, respira- A healthy 39-year-old woman, gravida 2 para 1, tory rate, and temperature were normal, and she came to the Emergency Department with a 2-day did not have orthostatic . Her sodium history of nausea, vomiting, diarrhea, dizziness, and was 108 mEq/L. Her hemoglobin was 10.7 mL/dL, fatigue. Twelve days earlier, she had undergone a hematocrit 29%, white cell count 6,000/␮L, potas- primary low transverse cesarean section at term for sium 4.6 mEq/L, chloride 81 mEq/L, carbon diox- failure to descend after premature rupture of mem- ide content 16 mEq /L, blood urea nitrogen 5.0 branes, augmentation, and 21⁄2 hours of mg/L, and creatinine 0.7 mmol/L. She was alert active pushing. A viable male infant weighing 3,832 and oriented and had normal findings on neuro- g was delivered with Apgar scores of 9 and 9 at 1 logic examination. She was admitted to the hospital and 5 minutes, respectively. The patient’s for severe . Initially her hyponatre- was found to be boggy and patulous after delivery. mia rapidly corrected to 119 mEq/L by active hy- A lower uterine segment laceration was repaired, dration with normal saline solution and then furo- and the uterus and skin incisions were closed. She semide diuresis. The patient subsequently had http://www.jabfm.org/ had considerable postpartum and received fluids restricted; however, her sodium level was misoprostol and dinoprostone. slow to correct. At admission, her serum osmolality The patient continued to hemorrhage despite was 220 mOsm/L, and her osmolality was these interventions and was taken back to the op- 120 mOsm/L. Her level and -stim- erating room for an emergent supracervical hyster- ulating (TSH) level were also measured at ectomy. Because her right was hemorrhagic, admission and were low at 1.9 ␮g/dL and 0.44 she also had a right oophorectomy. She continued ng/dL, respectively. Later her free thyroxine and on 25 September 2021 by guest. Protected copyright. to hemorrhage after the hysterectomy and under- Ͻ (T3) levels were found to be 0.4 went an embolization of her right vaginal artery. ng/dL and 44 ng/dL, respectively. These labora- The embolization finally stopped her bleeding. tory values suggested pituitary failure. To ascertain During these procedures she was hypotensive and whether this patient did in fact have pituitary fail- coagulopathic and received numerous units of ure, an tolerance test was conducted on day blood, platelets, and fresh frozen plasma. After the 4 of her hospitalization; the results are displayed in embolization, the patient remained in the intensive Table 1. care unit for 2 days and spent a total of 10 days in Based on these results, pituitary failure caused by the hospital. A sodium level measured on the 5th Sheehan syndrome was diagnosed. The patient was day of her hospitalization was normal. She had started on cortisone acetate while still an inpatient been home for 2 days when she came to the Emer- and on and estrogen 1 week after her discharge. She was advised to wear a Medic Alert bracelet indicating adrenal insufficiency. The pa- Submitted, revised, 16 January 2001. From the Department of Family Medicine (SS, LS), Uni- tient will need to remain on both estrogen and versity of Wisconsin, Madison. Address reprint requests to levothyroxine therapy for the rest of her life. She Sarina Schrager, MD, Department of Family Medicine, University of Wisconsin, 777 S. Mills St, Madison, WI will need adrenocortical steroid replacement when- 53715. ever she has surgery or is severely ill.

Sheehan Syndrome 389 Table 1. Results, by Minutes itary. In this case, the patient responded slightly to J Am Board Fam Pract: first published as on 1 September 2001. Downloaded from After Insulin Administration. fluid restriction, but her sodium levels did not re-

30 45 60 90 120 turn to normal until she received Components Measured min min min min min replacement therapy. Diagnosis of Sheehan syndrome can be difficult. Corticotropin (ACTH) (pg/mL) 16 18 17 15 12 The diagnosis is based on clinical evidence of hy- Cortisol (␮g/dL) 1.6 1.8 3.2 3.3 3.0 popituitarism in a woman with a history of a post- Glucose (mg/dL) 47 38 50 74 104 (ng/mL) 0.5 0.8 2.4 1.7 1.3 partum hemorrhage. Deficiencies of specific ante- rior pituitary will cause varied symptoms. Corticotropin deficiency can cause Discussion weakness, fatigue, , or dizziness. Go- Sheehan syndrome, or necrosis of the pituitary nadotropin deficiency will often cause , gland, is a rare complication of postpartum hem- , hot flashes, or decreased . orrhage initially described in 1937.1 This case il- Growth hormone deficiency causes many vague lustrates an example of Sheehan syndrome at 2 symptoms including fatigue, decreased quality of weeks postpartum with severe hyponatremia. The life, and decreased muscle mass. is physiologically enlarged in preg- Secondary is clinically indistin- nancy and is therefore very sensitive to the de- guishable from primary hypothyroidism, but pa- creased blood flow caused by massive hemorrhage tients with hypothyroidism caused by hypopituitar- and hypovolemic . Women with Sheehan ism have low T3 and T4 levels with normal or even syndrome have varying degrees of hypopituitarism, inappropriately low TSH levels. Diagnosis of pan- ranging from panhypopituitarism to only selective hypopituitarism is straightforward, but partial de- pituitary deficiencies.2–4 The is ficiencies are often difficult to elicit.9 A woman more susceptible to damage than the posterior pi- with panhypopituitarism will have low levels of tuitary. pituitary hormones (, cortico- Failure to lactate or difficulties with are tropin, and thyrotropin) as well as the target hor- 5 common initial symptoms of Sheehan syndrome. mones (cortisol and thyroxine). Stimulation tests http://www.jabfm.org/ Many women also report amenorrhea or oligomen- (insulin-induced hypoglycemia or orrhea after delivery. In some cases, the diagnosis is stimulation test) are often necessary for diagnosis in not made until years later, when features of hypo- the acute phase or in situations where a partial pituitarism, such as secondary hypothyroidism or deficiency is suspected.10 secondary adrenal insufficiency, become evident in In this case, the diagnosis of Sheehan syndrome a woman who had a postpartum hemorrhage. A was suspected because of her history, hyponatre- on 25 September 2021 by guest. Protected copyright. woman with undiagnosed hypopituitarism from mia, and low baseline cortisol and thyroid hor- Sheehan syndrome might be relatively asymptom- mones levels. Neither cortisol, corticotropin, nor atic until her body is stressed by a severe growth hormone levels responded to a hypoglyce- or surgery years after her delivery, and she goes mic state. The insulin tolerance test induces tran- into an . sient hypoglycemia, which in a person with a nor- Hyponatremia is an uncommon acute presenta- mal pituitary gland stimulates corticotropin tion of Sheehan syndrome.6–8 There are several production and cortisol release. Normal stimulated possible mechanisms by which hypopituitarism can levels of cortisol range in the hundreds. This pa- result in hyponatremia. Hypothyroidism can cause tient’s cortisol level never went higher than 3 ␮g/ decreased free-water clearance and subsequent hy- dL. The insulin tolerance test can also test for ponatremia. deficiency can also growth hormone deficiency but is an unpleasant cause decreased free-water clearance independent test for the patient and is contraindicated in pa- of . Hypopituitarism itself can stimulate tients who have or . vasopressin secretion and can cause severe inappro- Radiologic imaging with either computed to- priate secretion of antidiuretic hormone, which can mography or magnetic resonance imaging is usu- also cause hyponatremia. The potassium level in ally not helpful in the acute phase and has not been these situations is normal, because adrenal produc- used frequently in acute diagnosis.11 Several studies tion of aldosterone is not dependent on the pitu- have shown an empty sella, however, in women

390 JABFP September–October 2001 Vol. 14 No. 5 with Sheehan syndrome further into the disease Rubin PC. Pituitary failure from Sheehan’s syn- J Am Board Fam Pract: first published as on 1 September 2001. Downloaded from process.12–14 Imaging can be helpful in situations drome in the puerperium. Two case reports. Br J where the diagnosis is not apparent. Obstet Gynaecol 1987;94:998–9. Treatment of young women with hypopituitar- 5. Roberts DM. Sheehan’s syndrome. Am Fam Phys 1988;37:223–7. ism usually includes replacement of hydrocortisone 6. Boulanger E, Pagniez D, Roueff S, et al. Sheehan first and then replacement of thyroid hormone and syndrome presenting as early post-partum hypona- estrogen with or without progesterone depending tremia. Nephrol Dial Transplant 1999;14:2714–5. on whether she has a uterus. Hydrocortisone is 7. Shoji M, Kimura T, Ota K, et al. Cortical laminar replaced first because thyroxine therapy can exac- necrosis and central pontine myelinolysis in a patient erbate glucocorticoid deficiency and theoretically with Sheehan syndrome and severe hyponatremia. induce an adrenal crisis.9,15 The standard dose of Intern Med 1996;35:427–31. hydrocortisone is 20 mg/d for an adult (15 mg 8. Putterman C, Almog Y, Caraco Y, Gross DJ, Ben- every morning and 5 mg every evening). Both thy- Chetrit E. Inappropriate secretion of antidiuretic hormone in Sheehan’s syndrome: a rare cause of roxine replacement and replacement postpartum hyponatremia. Am J Obstet Gynecol are common, and doses are titrated to each indi- 1991;165(5 Pt 1):1330–3. vidual. Replacement of growth hormone is neces- 9. Lamberts SW, de Herder WW, van der Lely AJ. sary in children with hypopituitarism but is contro- Pituitary insufficiency. Lancet 1998;352:127–34. versial in adults. Some people with severe growth 10. Vance ML. Hypopituitarism. N Engl J Med 1994; hormone deficiency derive great benefit from re- 330:1651–62. placement, but standard recommendations are not 11. Dejager S, Gerber S, Foubert L, Turpin G. Shee- available.16 han’s syndrome: differential diagnosis in the acute Although Sheehan syndrome is uncommon as a phase. J Intern Med 1998;244:261–6. result of improved obstetric care, it should be a 12. Banzal S, Ayoola EA, Banzal S. Sheehan’s syndrome in Saudi Arabia. Int J Gynaecol Obstet 1999;66: consideration in any woman who has a history of a 181–2. postpartum hemorrhage and who reports signs or 13. Otsuka F, Kageyama J, Ogura T, Hattori T, Makino symptoms of pituitary deficiency. H. Sheehan’s syndrome of more than 30 years’ du- ration: an endocrine and MRI study of 6 cases. En- http://www.jabfm.org/ References docr J 1998;45:451–8. 1. Sheehan HL. Postpartum necrosis of the anterior 14. Dash RJ, Gupta V, Suri S. Sheehan’s syndrome: pituitary. J Path Bacteriol 1937;45:189–214. clinical profile, pituitary hormone responses and 2. DiZerega G, Kletzky OA, Mishell DR. Diagnosis of computed sellar tomography. AustNZJMed1993; Sheehan’s syndrome using sequential pituitary stim- 23:26–31. ulation tests. Am J Obstet Gynecol 1978;132:348– 15. Orrego JJ, Barkan AL. Pituitary disorders. Drug

53. treatment options. Drugs 2000;59:93–106. on 25 September 2021 by guest. Protected copyright. 3. Ozbey N, Inanc S, Aral F, et al. Clinical and labo- 16. Davies JS, Obuobie K, Smith J, et al. A therapeutic ratory evaluation of 40 patients with Sheehan’s syn- trial of growth hormone in hypopituitary adults and drome. Isr J Med Sci 1994;30:826–9. its influence upon continued prescription by general 4. Lakhdar AA, McLaren EH, Davda NS, McKay EJ, practitioners. Clin Endocrinol 2000;52:295–303.

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