Intermittent Blurry Vision: An Unexpected Presentation of Cushing’s Syndrome Due to Primary Bilateral Macronodular Adrenal Hyperplasia (PBMAH)

Abstract

Cushing’s syndrome (CS) is an uncommon endocrine disorder resulting from prolonged exposure to elevated glucocorticoids, with 10-15 million annual cases per the American Association of Neurological Surgeons. Exogenous and endogenous causes can further be divided into adrenocorticotropic hormone (ACTH) dependent (i.e Cushing’s Disease) or ACTH independent. ACTH-independent CS can be caused by primary bilateral macronodular adrenal hyperplasia (PBMAH) representing less than 1% cases of CS. We report a case of a woman presenting with chronic resistant hypertension, episodic blurry vision, weight gain and wasting of extremities. She was diagnosed with Cushing’s syndrome due to PBMAH. Our patient’s presentation was unusual as she presented at 40 years old, 10 years earlier than expected for PBMAH; and primarily with complaints of episodic blurry vision. Her symptoms also progressed rapidly as signs and symptoms largely presented over the course of 12 months, however responded well to surgical resection.

Cushing’s syndrome diagnosed incidentally on CT imaging

“…Uncommon presentations of Cushing’s have also been observed in the last year including mania and psychoses in patients for which they experienced symptom resolution with treatment 15 16. Other unlikely presentations of PBMAH include intermittent blurry vision in a woman in her 40s which largely resolved with bilateral adrenalectomy 17. The case report underscores the importance of reporting incidental adrenal enlargement or nodularity when seen, in order to alert the clinician to the possible presence of a systemic endocrine hyperfunction disorder.…”