Abstract
Management of endogenous Cushing syndrome is based on its aetiology. Increased Adrenocorticotropic Hormone (ACTH) levels are the most common cause of this disorder and, therefore, it is critical to determine the source of ACTH before further management. Dynamic post contrast MRI is currently the most common investigation implied to diagnose pituitary adenoma; however, it comes with the drawback of low specificity and high false positive results. Inferior petrosal sinus sampling (IPSS) is an established invasive procedure performed to differentiate central versus peripheral source of ACTH which, in turn, results in hypercortesolaemia. This is a series of 14 patients who underwent IPSS at the Department of Radiology, Aga Khan University Hospital, Karachi, from January 2006 to December 2018. The case series emphasises the role of IPSS in the management of ACTH-dependent Cushing syndrome and combined efficacy of Dynamic post-contrast MRI and the procedure under focus.
Highlights
Adrenocorticotropic hormone (ACTH) acts on the middle layer of adrenal cortex, known as zona fasciculata, resulting in production of cortisol
ACTH is produced by pituitary gland and a functional pituitary adenoma can result in hyper secretion of ACTH, referred to as Cushing disease.[2,3]
Inferior petrosal sinus sampling (IPSS) is an invasive procedure performed by an interventional radiologist to determine the source of elevated serum ACTH.[11]
Summary
Adrenocorticotropic hormone (ACTH) acts on the middle layer of adrenal cortex, known as zona fasciculata, resulting in production of cortisol. A 68-year-old female, who was a known case of diabetes mellitus and hypertension, presented with a history of weight gain by 8 kg in the last 24 months, swelling on the feet and face, central obesity, easy bruisability, and muscle weakness Her serum cortisol levels were 70 microg/dl (Normal reference range 3.4 – 22.4 microgram/ dl) and ACTH levels were 205 pg/ml (Normal reference range less than 46 pg/ml). The patient subsequently underwent inferior petrosal sinus sampling, results of which were suggestive of peripheral source of ACTH. The patient underwent Dynamic contrast enhanced MRI brain examination, (Figure 2) which revealed normal enhancement of the pituitary gland with its normal contour. The patient underwent bilateral inferior petrosal sinus sampling, the results of which were suggestive of a central source of ACTH with left preponderance. Surgery of the pituitary gland was performed and histopathology confirmed the diagnosis of pituitary adenoma
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