Synchronous bilateral adrenalectomy for ACTH-independent Cushing's syndrome in children: multidisciplinary management

Adreno CorticoTropic Hormone (ACTH)-independent Cushing's syndrome (CS) in children is very rare but potentially fatal. In bilateral nodular hyperplasia, synchronous bilateral adrenalectomy (SBA) represents the definitive treatment to correct hypercortisolism. We aim to report the multidisciplinary management of this rare condition.

Presentation, management, and outcomes of all children with ACTH-independent CS who underwent SBA in a tertiary reference center between 1992 and 2022 were retrospectively analyzed.

Ten children were included: 6 with McCune-Albright syndrome, 4 with primary pigmented nodular adrenocortical disease (3 with Carney complex). Presentation varied according to age at diagnosis. In infants (n = 7), failure to thrive with anorexia (n = 7), cushingoid facies (n = 7), arterial hypertension (n = 6), hypotonia (n = 5), hyperglycemia (n = 4), liver dysfunction (n = 4), and hypercalcemia with nephrocalcinosis (n = 4) were the main reasons for urgent hospitalization. Prepubertal children (n = 3) presented with linear growth arrest associated with obesity, cushingoid facies, and hirsutism. SBA was indicated in severe neonatal hypercortisolism, or in those that were non-responsive to medical treatment. SBA was performed by robot-assisted laparoscopy (n = 6), laparoscopy (n = 2) or laparotomy (n = 2). One post-operative complication occurred (seroma in the adrenal bed). During follow-up, (median: 4.8 years) no deaths or acute adrenal crisis occurred.

Pediatric CS must be referred to a pediatric center with medical and surgical expertise. Shared decision-making regarding SBA must be prompt to allow rapid cure of CS, even if it implies lifelong steroid replacement. This avoids long-term complications of hypercortisolism, particularly cognitive decline. Robot-assisted SBA is feasible and safe, even in infants when performed by an experienced robotic team.