Pituitary tumor apoplexy is an uncommon syndrome resulting often spontaneously from hemorrhage or infarction of a pre-existing pituitary adenoma. As the primary event involves the adenoma, the syndrome should be referred to as pituitary tumor apoplexy and not as pituitary apoplexy. The sudden increase in sellar contents compresses surrounding structures and portal vessels, resulting in sudden, severe headache, visual disturbances, and impairment in pituitary function. Initial management of patients with pituitary tumor apoplexy includes supportive therapy (intravenous fluids and corticosteroids), following which many patients exhibit clinical improvement. Because those patients can be effectively managed with supportive measures, many who remain clinically and neurologically unstable might benefit from urgent surgical decompression by an experienced neurosurgeon. All patients presenting with this syndrome require long-term follow-up to treat any residual tumor and/or pituitary dysfunction. Close interaction between members of the management team is necessary for optimal patients' outcome.
This article provides the first thorough review and characterization of patients with hemorrhage within an RCC. Based on the clinical manifestations of this presentation, we recommend using the term "Rathke cleft cyst apoplexy" to describe the syndrome.
Despite profound hypocortisolemia after adenomectomy, a simultaneously measured plasma ACTH level of >20 ng/L in the perioperative period is highly predictive of future recurrence of ACTH-secreting adenomas.
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