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Sheehan Syndrome

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Pituitary Disorders throughout the Life Cycle

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Abstract

Sheehan syndrome (SS) is the leading cause of hypopituitarism in adult women in underdeveloped and developing countries. SS is defined as postpartum pituitary necrosis due to severe hypotension or shock secondary to massive uterine bleeding. Although SS has become a rare disease as a result of improved obstetrical care in the modern age, it still exists in developed countries probably due to overlooking of the patients because of the unawareness of medical staff. SS is characterized by variable degrees of anterior pituitary dysfunction including isolated, partial, or complete hypopituitarism. Hypothalamus may also be involved, but diabetes insipidus is very rare. Although underlying mechanisms responsible for the development of postpartum pituitary necrosis are not clearly defined, recent studies have given new insight into the pathogenesis. Pituitary gland becomes physiologically enlarged during pregnancy, and it may result in compressed blood supply in a smaller sella turcica which has been reported in SS. Consequently, blood flow to the pituitary may be severely diminished due to vasospasm following untreated severe hypotension or shock. An autoimmune process involving both the hypothalamus and the pituitary gland may be responsible for the worsening of the pituitary function over the years and may contribute to late pituitary dysfunction reported in SS. The most common presenting symptoms are the failure of postpartum lactation (agalactia) and failure to resume menses after delivery. GH is the most frequently lost hormone in SS, presumably because of localization of the somatotroph cells at the sublateral part of the pituitary gland which is the most vulnerable region to ischemia. Severe GH deficiency is common. Empty sella, either partial or complete, on MRI is one of the most characteristic findings of SS. SS is associated with increased morbidity, and because of its nonspecific signs and symptoms, most patients remain undiagnosed and untreated for a long time. Treatment includes appropriate replacement of deficient hormones.

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Authors and Affiliations

  1. Erciyes University, Medical School, Department of Endocrinology, Kayseri, Turkey

    Zuleyha Karaca

  2. Yeditepe University, Medical School, Department of Endocrinology, İstanbul, Turkey

    Fahrettin Kelestimur

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  1. Zuleyha Karaca
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Correspondence to Fahrettin Kelestimur .

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  1. Mayo Clinic, Jacksonville, FL, USA

    Susan L. Samson

  2. Emory University School of Medicine, Atlanta, GA, USA

    Adriana G. Ioachimescu

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Karaca, Z., Kelestimur, F. (2022). Sheehan Syndrome. In: Samson, S.L., Ioachimescu, A.G. (eds) Pituitary Disorders throughout the Life Cycle. Springer, Cham. https://doi.org/10.1007/978-3-030-99918-6_18

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  • DOI: https://doi.org/10.1007/978-3-030-99918-6_18

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