Nelson syndrome (N S) is the set of symptoms related to a pituitary macroadenoma secreting ACTH developed following a bilateral adrenalectomy (BA). Its frequency is thought to account for up to 40% of adult cushing diseases (CD) who have undergone BA. We report the case of a patient with macroinvasive NS revealed by pituitary apoplexy. Mr M.L aged 35 years, followed for CD with negative imaging evolving for ten years, treated with BA. The evaluation at 03 months post-surgery noted a disappearance of signs of cushing but accentuation of melanoderma with an ACTH at 135pg/ml and a hemorrhagic adenoma of 08 mm on pituitary MRI. The patient was lost to follow-up for 05 years until he consulted again on a pituitary apoplexy chart with severe ophthalmoplegia revealing a pituitary macro-adenoma 33 mm high with multidirectional invasion and ACTH > 2000pg/ml. He was rushed for transphenoidal surgery, followed a few months later by additional radiation therapy. Nelsons syndrome is a rare entity in practice, but relatively common following bilateral adrenalectomy for cushings disease. Predictors of the onset of this syndrome exist but are controversial. Pituitary MRI monitoring should be routine in all CDs who have undergone BA. Treatment is mostly surgical, supplemented by radiotherapy in invasive cases. Drug treatments are being studied with promising results for dopaminergic agonists and somatostatin analogues.
08 Nov 2021 - 10 Nov 2021