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

Abstract

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 centre between 1992-2022 were retrospectively analysed. Ten children were included: six with McCune Albright syndrome, four with primary pigmented nodular adrenocortical disease (three 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), hyperglycaemia (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. Paediatric CS must be referred to a paediatric centre 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.