Effect of primary empty sella syndrome on pituitary surgery for Cushing's disease

Abstract

Object. 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. (http://thejns.org/doi/abs/10.3171/2014.3.JNS132012) Key wOrDS • Cushing's disease • empty sella syndrome • pituitary adenoma • transsphenoidal surgery • pituitary surgeryAbbreviations used in this paper: ACTH = adrenocorticotropin; BMI = body mass index; CD = Cushing's disease; CRH = corticotropin-releasing hormone; DI = diabetes insipidus; ESS = empty sella syndrome; GH = growth hormone; SIADH = syndrome of inappropriate antidiuretic hormone; UFC = urinary free cortisol.

Delayed diagnosis of Cushing's disease in a pediatric patient due to apparent remission from spontaneous apoplexy

“…Additionally, there have been a number of reports where CD spontaneously resolved because of pituitary apoplexy in adult patients, a consideration that could fit our patient’s early presentation [8–11]. Furthermore, her MRI in 2008 showed an empty sella, which is consistent with pituitary apoplexy (Figure 1) [15, 16]. The timeline for spontaneous regression of pituitary tumors following apoplexy has not been clearly defined, thus empty sella may not be evident on imaging until after the apoplexy has occurred.…”

Cushing's disease and empty sella turcica in a diabetic patient. A case report

“…Elle peut être secondaire à une pathologie tumorale hypophysaire ; dans le cas contraire elle est considérée comme primaire. La selle turcique vide est une affection neuroanatomique qui est plus fréquente chez les femmes multipares obèses d'âge moyen souffrant d'hypertension [3].…”

Thymic Carcinoid with Adrenocorticotropic Hormone-Producing Ectopic Cushing Syndrome and Empty Sella

“…An empty sella may theoretically have occurred due to pituitary hypoplasia as a result of strong negative glucocorticoid feedback from EAS. However, the presence of an empty sella does not preclude the diagnosis of pituitary Cushing disease and appropriate testing should be pursued if the suspicion for a pituitary ACTH source is high (10,11).…”