Adrenocorticotropin-independent macronodular adrenal hyperplasia (AIMAH): initial clinical experience in Ukraine

Aim. To analyze modern medical literature and own clinical data related to pathogenesis, diagnosis and treatment of AIMAH patients. Material and methods. Published and electronic resources were analyzed. Own data were received from institutional computed database, collected data of more than 900 patients after adrenalectomies, performed during the last 15 years, including 34 (3,8%) adequately examined patients with AIMAH. Results and discussion. AIMAH is not a rare condition among endogenous adrenal CS. Average age of patients was (53+_7) years (42?71 years), males comprised 23,3% (7 persons). Primary hyperaldosteronism was simultaneously diagnosed with CS in one half of patients with AIMAH. Clinical presentation of disease comprised with predominantly subclinical CS accompanied by arterial hypertension, diabetes mellitus, osteoporosis and obesity. All patients after confirmation of CS, ACTH-independancy and positive visualization were treated by unilateral laparoscopic adrenalectomy. Follow-up was carried out for 30 patients up to 1 year and for 25 patients ? until 5,5 years after surgery. Clinical improvement of symptoms has been established for 93% and 76% correspondingly. All patients demonstrated normalization of serum level of cortisol, not a single case of laboratory relapse of CS was confirmed. Conclusion. Bilateral hyperplastic lesions of adrenals with low radiologic density should be considered as possible AIMAH and should undergo investigation on occult CS. Laparoscopic adrenalectomy on the side of more prominent hyperplasia leads to long term remission in most of patients and does not lead to adrenal insufficiency.