Comprehensive Diagnosis and Surgical Management of Cushing Disease: Two-Dimensional Angiographic and Operative Video

World Neurosurg. 2021 Apr:148:188. doi: 10.1016/j.wneu.2021.01.036. Epub 2021 Jan 21.

Abstract

Cushing disease (CD), or hypercortisolemia secondary to an adrenocorticotropic hormone-secreting (ACTH-secreting) pituitary adenoma, is the most common etiology of noniatrogenic Cushing syndrome.1 The diagnostic algorithm employed in the patient with suspected CD is complex and includes consideration for inferior petrosal sinus sampling (IPSS).2,3 When workup is consistent with CD, extracapsular resection of the ACTH-secreting pituitary adenoma through the endonasal corridor is the preferred operative strategy.4 In this publication, we discuss the case of a 26-year-old woman who presented with 9 months of weight gain (Video 1). Initial labs, including low- and high-dose dexamethasone suppression tests, were consistent with CD. Findings on dynamic magnetic resonance imaging were suggestive of a left 2-mm microadenoma. IPSS confirmed central origin of hypercortisolemia and was indicative of a left-sided focus. The patient was taken to the operating room for endoscopic endonasal approach for extracapsular resection of left-sided, ACTH-secreting microadenoma. Following surgery, the patient required glucocorticoid supplementation after her cortisol levels decreased to 2 ug/dL the evening of surgery. Subsequent laboratory analysis has been consistent with hormonal cure.5,6 The accompanying video manuscript describes 1) preoperative diagnostic evaluation of the patient with suspected CD, 2) indications for and techniques of IPSS, 3) nuances of endoscopic transsphenoidal surgical management, and 4) relevant considerations in postoperative care. Of note, full patient consent for photography and/or recording of other forms of video/imaging was obtained in the preoperative period.7-10.

Keywords: Cushing disease; Hypercortisolemia; Inferior petrosal sinus sampling; Pituitary adenoma; Transsphenoidal.

Publication types

  • Case Reports
  • Video-Audio Media

MeSH terms

  • ACTH-Secreting Pituitary Adenoma / surgery
  • Adenoma / diagnosis*
  • Adenoma / surgery*
  • Adult
  • Dexamethasone / pharmacology
  • Female
  • Humans
  • Magnetic Resonance Angiography / methods*
  • Petrosal Sinus Sampling
  • Pituitary ACTH Hypersecretion / diagnosis*
  • Pituitary ACTH Hypersecretion / surgery*
  • Pituitary Gland / diagnostic imaging
  • Pituitary Gland / surgery
  • Pituitary Neoplasms / surgery

Substances

  • Dexamethasone