Subtotal adrenalectomy: one- or two-staged surgical procedure in children?

Bilateral adrenalectomy is a recognized treatment for a wide range of hormonally active and inactive tumors developing in both adrenal glands as well as different forms of hypercortisolism. However, patients after total bilateral adrenalectomy need lifelong adrenocortical hormone supplementation. Despite apparent sufficient exogenous steroid substitution, chronic dependence on adrenal hormones may impair quality of life. To preserve adrenocortical function and make a patient independent of exogenous coticosteroid replacement therapy, subtotal or partial adrenal resection has been proposed. The operation has been useful and efficient especially in patients with bilateral tumor as pheochromocytomas common in patients with multiple endocrine neoplasia type 2 (MEN 2) or von Hippel-Lindau disease (VHL), but also in patients with tumors or bilateral adrenal hyperplasia presenting symptoms of hyperaldosteronism or hypercortisolism. We report four patients treated in the Department of Pediatric Surgery in Bydgoszcz between 2004 and 2007 in whom subtotal adrenalectomy was performed. Two children were operated on because of bilateral neuroblastoma. The next two patients with nodular hyperplasia (one of them had symptoms of hypercortosolism) undergone the first stage of surgery, which was a unilateral adrenalectomy. We discuss diagnostic methods, preparations before surgery, mode of subtotal adrenalectomy and postoperative management.