Laparoscopic Unilateral Adrenalectomy in Children for Isolated Primary Pigmented Nodular Adrenocortical Disease (PPNAD): Case Report and Literature Review

Abstract

Primary pigmented nodular adrenocortical disease (PPNAD) is a pituitary-independent, primary adrenal form of hypercortisolism, characterized by small or normal-sized adrenal glands containing multiple cortical nodules; it is a rare syndrome and its main endocrine manifestation is adrenocorticotropic hormone-independent Cushing's syndrome. ACTH-independent adrenocortical causes of Cushing's syndrome account for up to 15% of all cases of Cushing's syndrome in children older than 7 years.