ECEESPE2025 ePoster Presentations Adrenal and Cardiovascular Endocrinology (170 abstracts)
1Alfaisal University, Riyadh, Saudi Arabia; 2AlFaisal University, Riyadh, Saudi Arabia; 3King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
JOINT1528
Introduction: Cushing Syndrome (CS) is an endocrine disorder caused by prolonged exposure to elevated cortisol. Distinctive clinical features of the disease termed cushingoid features include facial plethora, obesity, and growth failure in children. In pediatric patients, diagnosis and treatment is scarcely documented, particularly for ACTH-independent forms where bilateral adrenalectomy (BLA) is currently the only available curative treatment.
Objectives: To present two cases of neonatal ACTH-independent Cushing Syndrome managed with BLA. These cases are analyzed for surgical outcomes and pre- and post-operative cortisol trends, in aims to reach a conclusion for management of CS in pediatric patients aged 0-14. Additionally, this review examines existing literature on BLA in pediatric ACTH-independent CS to provide a comprehensive understanding of its efficacy and safety.
Methods: This case series details clinical presentation, laboratory findings, imaging results, and surgical management of two pediatric patients with ACTH-independent CS. A review of literature on pediatric BLA was conducted to contextualize these findings within broader clinical practice.
Results: A total of 7 cases of pediatric CS treated with BLA were analyzed, including 2 from our center and 5 previously reported in the literature. Of these cases, 6 (85.7%) favored BLA after reporting resolution of symptoms. 5 cases (57%) described a decrease in cortisol levels, and 1 case (14%) succumbed to chronic sequelae of CS.
Conclusion: These cases support the role of BLA as a viable treatment for pediatric ACTH-independent CS to target refractory disease activity. While BLA effectively normalizes cortisol and improves clinical symptoms, postoperative management of potential complications and lifelong glucocorticoid replacement therapy remains essential. Our review highlights the need for further research on pediatric BLA outcomes to enhance decision-making and optimize care for pediatric patients with CS.
Keywords: Cushing Syndrome, pediatric, adrenalectomy, ACTH-independent hypercortisolism.