Sheehan's syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Iqra Qamar M.D.[2]
Overview
Historical Perspective
- Sheehan syndrome was first discovered by Leon Konrad Gliński about a century ago and it was named after Harold Sheehan(1900-1988).[1][2]
Classification
- There is no established system for the classification of Sheehan's syndrome.
Pathophysiology
Causes
- Common causes of Sheehan's syndrome include massive hemorrhage, hypotension during pregnancy, vascular compression, and vascular occlusion(thrombosis, DIC). Less common causes include vascular insufficiency due to CABG in older patients and snake bites(Russell's viper bites).
Differentiating ((Page name)) from Other Diseases
- Sheehan syndrome must be differentiated from lymphocytic hypophysitis, pituitary apoplexy, hypothyroidism, Addison's disease, panhypopititarism, empty sella syndrome, hypogonadotropic hypogonadism, Simmond's disease, hypoprolactinemia, menopause,female athlete triadand SAH.[3][4][5]
Epidemiology and Demographics
- The incidence of Sheehan's syndrome is difficult to assess.[6] It was found to be the 6th most common cause of GH deficiency with an incidence of 3.1% of cases.[7] In 2009, the prevalence of Sheehan's syndrome was estimated to be 5.1 per 100,000 women.It is less prevalent in developed countries due to better obstetrical care and maternal health awraeness.[8][9].It is still one of the most common causes of hypopituitarism in developing countries.[9].
Risk Factors
- Common risk factors in the development of Sheehan's syndrome include pregnancy, severe/massive PPH, pituitary mass, pre-existing vascular diseases, autoimmunity,type 1 Diabetes, DIC, smaller and rigid sella, multiple gestations, placental abnormalities and traumatic delivery.
Screening
- There is insufficient evidence to recommend routine screening for Sheehan's syndrome.
Natural History, Complications, and Prognosis
- Sheehan's syndrome, if left untreated lead to hypopituitarism and empty sella syndrome.
- Common complications include adrenal crisis, hypotension, hypothyroidism and hypopituitarism.
- Prognosis is generally excellent provided early diagnosis and management resulting in complete reversal of symptoms.
Diagnosis
- Diagnosis is made on clinical basis with a recent/remote history of traumatic delivery or delivery complicated by hypotension.
Diagnostic Criteria
- Diagnosis is made on clinical basis with a recent/remote history of traumatic delivery or delivery complicated by hypotension.
- Diagnosis is mostly clinical but following steps are required to make the established diagnosis.
History and Symptoms
- The most common symptoms of Sheehan's sydrome include agalactorrhea and failure to resume menstruation after parturition. Common symptoms include hot flushes, decreased pubic/axillary hair, hypotension, hypoglycemia, features of hypothyroidism, hypoadrenalism and hypogonadism.
Physical Examination
- Patients with syndrome usually appear fatigued, lethargic and weight gain. Physical examination is usually remarkable for bradycardia, hypotension, pallor and signs suggestive of respective hormonal deficiency.
Laboratory Findings
- Laboratory findings consistent with the diagnosis of Sheehan's syndrome include hyponatremia, hypokalemia, hypocalcemia, hypomagnesemia, hypophosphatemia, anemia, pancytopenia, eosinophilia, hypoalbuminemia, low fasting plasma glucose etc.
Electrocardiogram
- ECG findings associated with Sheehan's syndrome can include QT interval prolongation,Type-1 Brugada-like ECG pattern(due to adrenal crisis),findings suggestive of cardiac tamponade, dilated cardiomyopathy(multifactorial).[12][13][14][15]
X-ray
- There are no x-ray findings associated with Sheehan's syndrome.
CT scan
- Acute presentation shows non-hemorrhagic pituitary gland enlargement while chronic presentation shows an empty sella or decreased sellar volume.[16][17][18]
MRI
- Findings on MRI suggestive of Sheehan's syndrome include decreased sellar volume, empty sella, pituitary remnant tissue or CSF fluid in sella.[16][17][18]
Ultrasound
- Echo findings associated with Sheehan's syndrome may include reversible dilated cardiomyopathy and pericardial effusion.[19][20]
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
References
- ↑ Template:WhoNamedIt
- ↑ H. L. Sheehan. Post-partum necrosis of anterior pituitary. The Journal of Pathology and Bacteriology, Chichester, 1937, 45: 189-214.
- ↑ Rolih CA, Ober KP (1993). "Pituitary apoplexy". Endocrinol. Metab. Clin. North Am. 22 (2): 291–302. PMID 8325288.
- ↑ Vidal E, Cevallos R, Vidal J, Ravon R, Moreau JJ, Rogues AM, Loustaud V, Liozon F (1992). "Twelve cases of pituitary apoplexy". Arch. Intern. Med. 152 (9): 1893–9. PMID 1520058.
- ↑ Lazaro CM, Guo WY, Sami M, Hindmarsh T, Ericson K, Hulting AL, Wersäll J (1994). "Haemorrhagic pituitary tumours". Neuroradiology. 36 (2): 111–4. PMID 8183446.
- ↑ Asaoka K (1977). "[A study on the incidence of post-partum hypopituitarism, (Sheehan's syndrome)]". Nihon Naibunpi Gakkai Zasshi (in Japanese). 53 (7): 895–909. PMID 303183.
- ↑ Abs R, Bengtsson BA, Hernberg-Stâhl E, Monson JP, Tauber JP, Wilton P, Wüster C (1999). "GH replacement in 1034 growth hormone deficient hypopituitary adults: demographic and clinical characteristics, dosing and safety". Clin. Endocrinol. (Oxf). 50 (6): 703–13. PMID 10468941.
- ↑ Feinberg EC, Molitch ME, Endres LK, Peaceman AM (2005). "The incidence of Sheehan's syndrome after obstetric hemorrhage". Fertil. Steril. 84 (4): 975–9. doi:10.1016/j.fertnstert.2005.04.034. PMID 16213852.
- ↑ 9.0 9.1 Krysiak R, Okopień B (2015). "[Sheehan's syndrome--a forgotten disease with 100 years' history]". Prz. Lek. (in Polish). 72 (6): 313–20. PMID 26817341.
- ↑ Cunningham SK, Moore A, McKenna TJ (1983). "Normal cortisol response to corticotropin in patients with secondary adrenal failure". Arch. Intern. Med. 143 (12): 2276–9. PMID 6316866.
- ↑ Grinspoon SK, Biller BM (1994). "Clinical review 62: Laboratory assessment of adrenal insufficiency". J. Clin. Endocrinol. Metab. 79 (4): 923–31. doi:10.1210/jcem.79.4.7962298. PMID 7962298.
- ↑ Komuro J, Kaneko M, Ueda K, Nitta S, Kasao M, Shirai T (2016). "Adrenal insufficiency causes life-threatening arrhythmia with prolongation of QT interval". Heart Vessels. 31 (6): 1003–5. doi:10.1007/s00380-015-0660-6. PMC 4893060. PMID 25771803.
- ↑ Anselm DD, Baranchuk A (2015). "Confirmed Brugada phenocopy in the setting of hypopituitarism". Herz. 40 (4): 639–40. doi:10.1007/s00059-014-4075-4. PMID 24718975.
- ↑ Martin-Grace J, Ahmed M, Mulvihill N, Feeney ER, Crowley RK (2017). "Getting to the heart of hypopituitarism". Clin Med (Lond). 17 (2): 140–142. doi:10.7861/clinmedicine.17-2-140. PMID 28365624.
- ↑ Doshi S, Roy A, Ramamoorthy A, Kothari SS, Bahl VK (2013). "Dilated cardiomyopathy: a ghost from the past". Circ Heart Fail. 6 (2): e19–21. doi:10.1161/CIRCHEARTFAILURE.112.000062. PMID 23513050.
- ↑ 16.0 16.1 Barkan AL (1989). "Pituitary atrophy in patients with Sheehan's syndrome". Am. J. Med. Sci. 298 (1): 38–40. PMID 2750772.
- ↑ 17.0 17.1 Sherif IH, Vanderley CM, Beshyah S, Bosairi S (1989). "Sella size and contents in Sheehan's syndrome". Clin. Endocrinol. (Oxf). 30 (6): 613–8. PMID 2591059.
- ↑ 18.0 18.1 Bakiri F, Bendib SE, Maoui R, Bendib A, Benmiloud M (1991). "The sella turcica in Sheehan's syndrome: computerized tomographic study in 54 patients". J. Endocrinol. Invest. 14 (3): 193–6. doi:10.1007/BF03346787. PMID 1906495.
- ↑ Frustaci A, Perrone GA, Gentiloni N, Russo MA (1992). "Reversible dilated cardiomyopathy due to growth hormone deficiency". Am. J. Clin. Pathol. 97 (4): 503–11. PMID 1553916.
- ↑ Hsieh CY, Liu BY, Yang YN, Yin WH, Young MS (2011). "Massive pericardial effusion with diastolic right ventricular compression secondary to hypothyroidism in a 73-year-old woman". Emerg Med Australas. 23 (3): 372–5. doi:10.1111/j.1742-6723.2011.01425.x. PMID 21668725.