Occurs in around 3 % of cases after removal of both adrenal glands for Cushing's syndrome. It consists in a set of symptoms following surgical removal of both adrenal glands in the treatment of Cushing's syndrome. The typical triad includes:

-         diffuse hyperpigmentation of the skin and mucous membranes, 

-         very high serum levels of adrenocorticotropin (ACTH) 

-         a corticotropic macroadenoma, which can lead to the following mass effects: headaches, visual field abnormalities, facial nerve paralysis, pituitary apoplexy, diabetes insipidus, panhypopituitarism.

Removal of both adrenal glands suppresses cortisol production, which may allow a pre-existing pituitary adenoma to grow uncontrolled by negative feedback. This growth can lead to a local mass effect, with increased production of both corticotropic hormone (ACTH) and melanotropic hormone, which are both derived from the same precursor peptide, proopiomelanocortin.

Treatment: surgery and/or radiotherapy.

Anesthetic implications: 

check endocrine status, hydrocortisone supplements perioperatively

References : 

-        Mehta M, Rath GP, Singh GP.
Anesthesia for Nelson’s syndrome.
MEJA 2009 ; 20 :313-4.

-        Torres-Ríos J, Romero-Luna G, Meraz Soto J, et al.
Nelson's syndrome: a narrative review.
Cureus 2023 ; 15: e39114. DOI 10.7759/cureus.39114

Updated: September 2024