Literature review: from definition to treatment of Sheehan's Syndrome
Keywords: Sheehan's syndrome, hypopituitarism, postpartum pituitary failure.
Introduction: Sheehan's Syndrome (SS) presents epidemiology, pathophysiology and uncertain and variable clinical manifestations. This makes diagnosis and preventive measures difficult, contributing to underdiagnosis. Therefore, this work proposes to evaluate the panorama of the current bibliography on this condition, in order to emphasize the importance of adequate management, aiming at reducing its morbidity and mortality. Methodology: from 2015 to 2020, the most pertinent articles contained in PubMed were selected for an integrative review. Keywords used were: "Sheehan's syndrome" OR "postpartum hypopituitarism" OR "postpartum pituitary necrosis". The publications underwent identification, screening, eligibility and inclusion. Results: SS is an obstetric neuroendocrine complication resulting from ischemic pituitary necrosis, affecting mainly adenohypophysis, causing partial or total hypopituitarism and, therefore, impaired hormonal function. It is mostly caused after moderate to severe postpartum hemorrhage (PPH). Epidemiological data are uncertain due to the delay and failure of diagnosis, especially in underdeveloped countries. The pathological basis of SS is the interruption or restriction of pituitary blood flow, leading to varied necrosis and, consequently, hormonal hypofunction in the affected regions. It can occur by 1) hypotension and vasospasm in the pituitary arteries triggered by PPH; 2) physiological increase of the pituitary in pregnancy without corresponding elevation of the blood flow provided and/or small sella turcica, causing vascular compression; 3) thrombosis by hypercoagulation and/or 4) anti-hypophysis antibodies causing progression of hypopituitarism. There is a variation of clinical manifestations due to hormonal deficits. Hypofunction of the anterior region may occur with growth hormone deficiency, lactation insufficiency, hypogonadism, secondary hypothyroidism and adrenal insufficiency; from the posterior region it manifests with diabetes insipidus (DI). Acute presentation is rare and severe; it has findings such as unconsciousness, headache, adrenal crisis and DI. Chronic phenomena are common, progressive and nonspecific; they occur with sleepiness, anorexia, nausea, anemia, physical and mental fatigue, premature aging, hypopigmentation, dry, cold and thick skin, madarosis, scattered pubic and axillary hair, breast atrophy, amenorrhea, slow reflexes, increased sensitivity to cold, bradycardia and coma. It may be associated not only with decreased bone mineral density (BMD) and corneal central thickness and increased cardiovascular risk, but also with psychiatric conditions. The diagnosis seeks findings suggestive of pituitary hypofunction and obstetric past conditions through detailed clinical history, physical examination and complementary tests such as stimulation tests and analysis of hormone levels, in addition to the characteristics of the pituitary in the Magnetic Resonance Imaging, which may reveal partially or completely empty Sella turcica. Lymphocytic hypophysis is the main differential diagnosis, followed by pituitary tumors. Continuous hormone replacement therapy is currently the only alternative to repair the hypophysis, but it does not correct lost pituitary function or interfere with the necrotic process. Conclusion: The SS is an entity with failure and delay in diagnosis due not only to the uncertain pathophysiology and non-specific manifestations, but also to medical unpreparedness and negligence with symptoms suggestive of the syndrome. Thus, treatment is postponed and morbidity and mortality increase, mainly due to the absence of follow-up of patients with PPH. Moreover, home births without professional assistance increase the risk of hemorrhage and make it difficult to accurately collect the obstetric history and, therefore, should be discouraged. Finally, the management of PPH should be early and effective, with sufficient blood transfusion and fluid replacement.
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