Successful Radiofrequency Ablation of Bilateral Macronodular Adrenal Cortical Disease for Cushing's Syndrome in a 6-Year- Old Girl: Preservation of Adrenal Function.

Authors

  • Dau-Ming Niu Department of Pediatrics, Taipei Veterans General Hospital, Taiwan/institute of Clinical Medicine, National Yang-Ming University, Taiwan
  • Shu-Huei Shen Department of Radiology, Taipei Veterans General Hospital, Taiwan/ School of Medicine, National Yang Ming Chiao Tung University, Taiwan
  • Wan-Hsin Liu Department of Pediatrics, Taipei Veterans General Hospital, Taiwan
  • Hsin-Lin Tsai Department of Surgery, Taipei Veterans General Hospital, Taiwan
  • Yung-Hsiu Lu Department of Pediatrics, Taipei Veterans General Hospital, Taiwan

DOI:

https://doi.org/10.3941/jrcr.5581

Abstract

Background: Bilateral macronodular adrenal cortical disease (BMACD) has not been reported in children. In adults with overt CS due to BMACD, bilateral adrenalectomy is a common intervention but typically leads to lifelong dependency on glucocorticoid and mineralocorticoid replacement, increasing the risk of adrenal crises. This highlights the need for adrenal-sparing treatment alternatives.

Case Presentation: A 6-year-old girl presented with growth delay, moon facies, hirsutism, hypertension, short stature, and obesity. Biochemical tests confirmed corticotropin-independent CS, with elevated midnight cortisol, high 24-hour urinary cortisol, and undetectable ACTH. An abdominal CT revealed multiple adrenal nodules, consistent with BMACD.

Results: CT-guided radiofrequency ablation (RFA) of bilateral adrenal adenomas led to complete resolution of CS symptoms and normal adrenal function at one-year follow-up.

Conclusion: CT-guided RFA is an effective adrenal-sparing alternative to bilateral adrenalectomy for corticotropin-independent CS, eliminating the need for lifelong glucocorticoid replacement while minimizing adrenal crisis risk in pediatric patients.

Contrast enhanced CT (A-D, sequential axial scans from cranial to caudal) shows multiple nodular lesions in bilateral adrenal glands (at least 5 on each side, red arrows), with the most prominent ones 1.4 cm on right side and 1.3 cm on left side, compatible with bilateral macronodular adrenal cortical disease.

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Published

2025-05-31

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Section

Pediatric Radiology