Clinical and surgical outcomes of pediatric Cushing's disease following endoscopic transsphenoidal surgery.

Abstract

Background: Cushing's disease (CD) is characterized by hypercortisolism due to excessive adrenocorticotropic hormone (ACTH) secretion from a pituitary adenoma. Though more common in adults, pediatric cases constitute approximately 5% of those seen in adults. Early diagnosis and treatment are critical due to the severe impacts on growth and development in children. Endoscopic transsphenoidal surgery (ETSS) is the preferred first-line treatment for both children and adults.

Methods: From 2011 to 2024, 3280 patients with pituitary adenoma underwent surgery using the ETSS technique at Loghman Hospital of Tehran, including 213 CD cases, of which 22 were pediatric (under 18 years old). This retrospective study analyzed clinical data such as age, gender, presenting symptoms, family and medical history, imaging characteristics, surgical findings, pathology, follow-up, and postoperative outcomes. Diagnostic criteria included biochemical tests, MRI imaging, and inferior petrosal sinus sampling.

Results: Out of 22 pediatric patients, 16 were females and 6 males, with an average age of 14.73 years. Obesity was the initial clinical manifestation in 59% of the patients. According to the Hardy-Wilson classification, 18.2% of patients were stage E, 9.09% stage C, and the rest (excluding MRI-negative patients) stage A. ETSS was performed on all patients, with total hypophysectomy in 18.2% and hemi-hypophysectomy in 9.09%. Postoperative complications included cerebrospinal fluid leakage in 13.63% of patients. Remission was achieved in 95.45% of patients, with a recurrence rate of 4.54%. Hormone imbalances post-surgery included hypocortisolism (50%), hypothyroidism (45.45%), and hypogonadism (13.63%).

Conclusion: ETSS is an effective and safe treatment for pediatric CD, achieving high remission rates and minimal complications. This study highlights the importance of specialized surgical care in managing pediatric CD and underscores the need for early diagnosis and intervention. Further studies are warranted to evaluate long-term outcomes and optimize treatment protocols.