Surgical effect of adrenocorticotropic hormone independent macronodular adrenal hyperplasia / 中华泌尿外科杂志

ABSTRACT

Objective To discuss the treatment experience of adrenocorticotropic hormone independent macronodular adrenal hyperplasia (AIMAH).Methods The clinical data of 24 cases of AIMAH from August 1972 to December 2016 were retrospectively analyzed.The cases included 10 males and 14 females with a mean age of 43 (range 26 to 59) years.16 patients presented with typical Cushing syndrome (CS) and 8 patients had weight gain, hypertension or diabetes mellitus without any sign of CS.The circadian rhythm of serum cortisol was abnormal.Low and high dose dexamethasone suppression tests failed to suppress cortisol secretion.The preoperative CT scan showed bilateral enlargement of the adrenal glands with multiple macmnodules.14 patients underwent open surgery, including 5 cases of unilateral adrenalectomy, 6 cases of adrenalectomy combined with contralateral subtotal adrenalectomy and 3 cases of bilateral adrenalectomy simuhaneously.6 patients underwent conventional laparoscopic adrenalectomy,including 2 cases of unilateral adrenalectomy, 2 cases of adrenalectomy combined with contralateral subtotal adrenalectomy and 2 cases of bilateral adrenalectomy.4 patients underwent robot-assisted laparoscopic bilateral adrenalectomy.Results The postoperative pathological examination showed that all patients had bilateral adrenal macronodular or adenomatoid hyperplasia.During the mean follow-up of 68 months (range 3 to 192 months), the clinical symptoms of CS disappeared after surgery in all cases.7 patients who received unilateral adrenalectomy had urinary free cortisol and serum cortisol within normal ranges and no further enlargement of the contralateral gland was noticed.Among the 3 patients who received bilateral adrenalectomy simultaneously, 1 case died of adrenal crisis on the seventh day post-operation.The remaining 2 cases presented with adrenal insufficiency but returned to normal after glucocorticoid replacement therapy.Nelson's syndrome was not observed in other patients.Conclusions The use of bilateral adrenalectomy to treat AIMAH may involve risk.Unilateral adrenalectomy is recommended for an alternative treatment for AIMAH.Controlateral adrenalectomy with lifelong corticosteroid replacement or contralateral subtotal adrenalectomy may be performed if the symptoms have not improved or recurred after unilateral adrenalectomy.