The diagnosis and management of Cushing’s syndrome in pregnancy

Ross Hamblin, Amy Coulden, Athanasios Fountas, Niki Karavitaki

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Endogenous Cushing’s syndrome (CS) is rarely encountered during pregnancy. Clinical and biochemical changes in healthy pregnancy overlap with those seen in pregnancy complicated by CS; diagnosis is, therefore, challenging, and can be delayed. During normal gestation, adrenocorticotropic hormone (ACTH), corticotrophin releasing hormone (CRH), cortisol, and urinary free cortisol levels rise. Dexamethasone administration fails to fully suppress cortisol in pregnant women without CS. Localisation may be hindered by non-suppressed ACTH levels in a large proportion of those with adrenal CS; smaller corticotroph adenomas may go undetected due to lack of contrast administration or presence of pituitary hyperplasia; and inferior petrosal sinus sampling is not recommended given risk of radiation and thrombosis. Yet, diagnosis is essential; active disease is associated with multiple insults to both maternal and foetal health, and those cured may normalise risk of maternal-foetal complications. Published literature consists mostly of case reports or small case series affected by publication bias, heterogeneous definitions of maternal or foetal outcomes, or lack of detail on severity of hypercortisolism. Consequently, conclusive recommendations, or a standardised management approach for all, cannot be made. Management is highly individualised – the decision for surgery, medical control of hypercortisolism, or adoption of a conservative approach, is dependent on the timing of diagnosis (respective to stage of gestation), the ability to localise the tumour, severity of CS, pre-existing maternal comorbidity, and ultimately, patient’s choice. Close communication is a necessity, with the patient placed at the centre of all decisions, with risks, benefits and uncertainties around any investigation and management carefully discussed. Care should be delivered by an experienced, multidisciplinary team, with the resources and expertise available to manage such a rare and challenging condition during pregnancy.
Original languageEnglish
JournalJournal of Neuroendocrinology
Early online date14 Mar 2022
Publication statusE-pub ahead of print - 14 Mar 2022


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