title: Effect of primary empty sella syndrome on pituitary surgery for Cushing's disease.
abstract: OBJECTIVE
  Primary empty sella syndrome (ESS) results from herniation of arachnoid mater into the pituitary fossa. It has been suggested to have a negative effect on pituitary surgery; however, outcomes in this cohort have not been defined. This study was performed to determine the effect of ESS on immediate and long-term biochemical outcome after pituitary surgery for Cushing's disease (CD).
  
  
  METHODS
  Using a matched cohort study design, the authors followed patients treated with pituitary surgery for CD with and without ESS. Complete ESS was defined as pituitary gland height ≤ 2 mm, whereas partial ESS was defined as pituitary gland height > 2 mm but less than three-quarters of the total sellar depth. The primary end points were immediate and long-term biochemical outcome. Cerebrospinal fluid leaks were recorded as a secondary end point.
  
  
  RESULTS
  Seventy-eight patients with CD and primary ESS were identified and matched with 78 patients with CD without ESS. After surgical management, immediate biochemical remission was achieved in 69 patients (88%) with ESS and 75 controls (96%, p = 0.10). Long-term remission was achieved in most patients in both groups (5-year cure: 85% vs 92%, p = 0.10). Among patients with ESS, the presence of complete ESS predicted a worse long-term outcome (p = 0.04). Intraoperative CSF leaks were significantly more frequent with ESS (54% vs 24%, p < 0.001), and despite sellar floor repair, the rate of postoperative CSF leaks was also increased (6% vs 3%, p = 0.27).
  
  
  CONCLUSIONS
  Biochemical outcome after pituitary surgery for CD was worse in patients with complete ESS, and the risk of a CSF leak was increased with both partial and complete ESS. However, as outcome remains superior to those following alternative therapies and the biology of these tumors is unchanged in the setting of ESS, pituitary surgery should remain the initial treatment of choice.
authors.affiliation: Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland; and.
authors.forename: Gautam U
authors.initials: GU
authors.lastname: Mehta
authors.forename: Kamran D
authors.initials: KD
authors.lastname: Bakhtian
authors.forename: Edward H
authors.initials: EH
authors.lastname: Oldfield
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D065634:Cerebrospinal Fluid Leak; D002559:Cerebrospinal Fluid Rhinorrhea / Q000453:epidemiology; D002648:Child; D015897:Comorbidity; D004652:Empty Sella Syndrome / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery*; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D015994:Incidence; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D019635:Neurosurgical Procedures / Q000009:adverse effects / Q000379:methods*; D047748:Pituitary ACTH Hypersecretion / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery*; D010902:Pituitary Gland / Q000473:pathology / Q000601:surgery*; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D006854:Hydrocortisone
keywords: ACTH = adrenocorticotropin; BMI = body mass index; CD = Cushing's disease; CRH = corticotropin-releasing hormone; Cushing's disease; DI = diabetes insipidus; ESS = empty sella syndrome; GH = growth hormone; SIADH = syndrome of inappropriate antidiuretic hormone; UFC = urinary free cortisol; empty sella syndrome; pituitary adenoma; pituitary surgery; transsphenoidal surgery