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

2014 ◽  
Vol 121 (3) ◽  
pp. 518-526 ◽  
Author(s):  
Gautam U. Mehta ◽  
Kamran D. Bakhtian ◽  
Edward H. Oldfield
Keyword(s):  
Pituitary Gland ◽  
Cushing’S Disease ◽  
Pituitary Surgery ◽  
Empty Sella ◽  
Cushing's Disease ◽  
Csf Leak ◽  
Empty Sella Syndrome ◽  
Csf Leaks ◽  
Primary Empty Sella

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.

scholarly journals Does pituitary compression/empty sella syndrome contribute to MRI-negative Cushing’s disease? A single-institution experience

2020 ◽  
Vol 48 (6) ◽  
pp. E3
Author(s):  
Benjamin T. Himes ◽  
Adip G. Bhargav ◽  
Desmond A. Brown ◽  
Timothy J. Kaufmann ◽  
Irina Bancos ◽  
...  
Keyword(s):  
Pituitary Gland ◽  
Cushing’S Disease ◽  
Adrenocorticotropic Hormone ◽  
Empty Sella ◽  
Cushing's Disease ◽  
Relative Reduction ◽  
Single Institution ◽  
Empty Sella Syndrome ◽  
Tissue Diagnosis ◽  
Acth Secreting

OBJECTIVECushing’s disease arises from functioning adrenocorticotropic hormone (ACTH)–secreting pituitary adenomas. These tumors can be very small and evade detection by MRI. Empty sella syndrome is a phenomenon by which an arachnoid outpouching of CSF into the sella leads to compression of the pituitary, likely due to intracranial hypertension (a common issue in Cushing’s disease), further leading to difficulty in visualizing the pituitary gland that may contribute to difficulty in finding a tumor on MRI, so-called MRI-negative Cushing’s disease. The authors sought to examine the association between empty sella syndrome and MRI-negative Cushing’s disease.METHODSA single-institution database of Cushing’s disease cases from 2000 to 2017 was reviewed, and 197 cases were included in the analysis. One hundred eighty patients had a tissue diagnosis of Cushing’s disease and 17 had remission with surgery, but no definitive tissue diagnosis was obtained. Macroadenomas (tumors > 1 cm) were excluded. The degree of empty sella syndrome was graded on the degree of CSF visualized in the sella on midline sagittal T1-weighted MRI.RESULTSOf the 197 cases identified, 40 (20%) presented with MRI-negative disease, and empty sella syndrome was present in 49 cases (25%). MRI-negative disease was found in 18 (37%) of 49 empty sella cases versus 22 (15%) of 148 cases without empty sella syndrome present. Empty sella syndrome was significantly associated with MRI-negative disease (OR 3.32, 95% CI 1.61–6.74, p = 0.0018). Decreased thickness of the pituitary gland was also associated with MRI-negative disease (mean thickness 5.6 vs 6.8 mm, p = 0.0002).CONCLUSIONSEmpty sella syndrome is associated with an increased rate of MRI-negative Cushing’s disease. Pituitary compression causing a relative reduction in the volume of the pituitary for imaging is a plausible cause for not detecting the tumor mass with MRI.

A Case of Cushing's Disease due to Pituitary Microadenoma Combined with Primary Empty Sella Syndrome

2006 ◽  
Vol 21 (6) ◽  
pp. 567
Author(s):  
Yun Jung Lee ◽  
Sangyoul Rhee ◽  
Suk Chon ◽  
Seungjoon Oh ◽  
Jeong-Taek Woo ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Empty Sella ◽  
Cushing's Disease ◽  
Empty Sella Syndrome ◽  
Pituitary Microadenoma ◽  
Primary Empty Sella

Cushing's disease in patient with primary empty sella

2014 ◽  
Author(s):  
Maria Kurowska ◽  
Joanna Malicka ◽  
Grzegorz Zielinski ◽  
Jerzy S Tarach ◽  
Maria Maksymowicz ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Empty Sella ◽  
Cushing's Disease ◽  
Primary Empty Sella

scholarly journals Endogenous ACTH Concentration-Cortisol Secretion Dose Analysis Unmasks Decreased ACTH Potency in Cushing's Disease with Restoration after Successful Pituitary Adenomectomy

2011 ◽  
Vol 96 (12) ◽  
pp. 3768-3774 ◽  
Author(s):  
Ferdinand Roelfsema ◽  
Daniel M. Keenan ◽  
Johannes D. Veldhuis
Keyword(s):  
Surgical Treatment ◽  
Cushing’S Disease ◽  
Pituitary Surgery ◽  
Pulse Frequency ◽  
Cushing's Disease ◽  
P Value ◽  
Cortisol Secretion ◽  
Acth Concentration ◽  
Active Disease

Background: The relationship between circulating ACTH levels and cortisol secretion in Cushing's disease is not precisely known. Hypothesis: Chronic ACTH hyperstimulation leads to decreased adrenal potency and is restored after normalization of ACTH secretion. Subjects: Subjects included 20 patients with Cushing's disease, eight patients in long-term remission, and 36 healthy controls. Outcomes: ACTH and cortisol secretion rates and analytical dose-response estimates of endogenous ACTH efficacy (maximal cortisol secretion), dynamic ACTH potency, and adrenal sensitivity (slope term) from 24-h ACTH-cortisol profiles were evaluated. Results: Both basal and pulsatile secretion of ACTH and cortisol were increased in patients with active disease but normal in cured patients. ACTH, but not cortisol pulse frequency, was amplified in patients and restored after successful surgical treatment. ACTH EC50, an inverse measure of potency, was higher during pulse onset in Cushing's disease (59 ± 7.4 ng/liter) than in controls (20 ± 3.7 ng/liter) (P &lt; 0.0001) and remitted patients after surgery [15 ± 3.2 ng/liter, P value not significant (NS) vs. controls] and during pulse recovery phases [128 ± 18 (P &lt;0.0001), 70 ± 8.4, and 67 ± 17 ng/liter (NS vs. controls), respectively]. Efficacy was increased in active disease and normalized after surgical treatment [patients, 38 ± 8.3 nmol/liter · min, vs. controls, 21 ± 2.3 nmol/liter · min (P &lt;0.0001), and cured patients, 15 ± 3.2 nmol/liter · min (NS vs. controls)]. Sensitivity to endogenous ACTH did not differ among the three groups. Conclusion: The adrenal gland in Cushing's disease exhibits decreased responsiveness to submaximal ACTH drive and amplified efficacy, with unchanged sensitivity. These target-gland abnormalities are reversible in long-term remission after pituitary surgery.

Long-term remission rates after pituitary surgery for Cushing's disease: the need for long-term surveillance

2005 ◽  
Vol 63 (5) ◽  
pp. 549-559 ◽  
Author(s):  
A. Brew Atkinson ◽  
Adele Kennedy ◽  
M. Ivan Wiggam ◽  
David R. McCance ◽  
Brian Sheridan
Keyword(s):  
Cushing’S Disease ◽  
Pituitary Surgery ◽  
Cushing's Disease ◽  
Remission Rates

scholarly journals Primary Empty Sella Syndrome: A Case Report

1970 ◽  
Vol 1 (1) ◽  
pp. 23-25
Author(s):  
Md Sanwar Hossain ◽  
Mahbuba Ashrafi Mumu ◽  
Md Moyenuddin PK
Keyword(s):  
Case Report ◽  
Pituitary Gland ◽  
Subarachnoid Space ◽  
Empty Sella ◽  
Multiple Pregnancies ◽  
Empty Sella Syndrome ◽  
Ocular Abnormalities ◽  
Medical College ◽  
Persistent Headache ◽  
Primary Empty Sella

Primary Empty Sella Syndrome (PES) is a relatively rare or uncommon developmental disease that develops spontaneously. It is characterized by herniation of subarachnoid space within the sella which is often associated with some degree of flattening of pituitary gland. Usually manifested by endocrine abnormalities, ocular abnormalities, rhinitis and intractable persistent headache. It is higher in obese females having multiple pregnancies. Here we report a case of empty sella syndrome in a 27 year old lady to aware the physicians to bear in mind a differential in patients having persistent intractable headache. Key words: Empty Sella Syndrome; Woman; Obesity; Intractable Headache DOI: 10.3329/akmmcj.v1i1.7456 Anwer Khan Modern Medical College Journal 2010; 1(1): 23-25

scholarly journals Extrapituitary Parasellar Microadenoma in Cushing’s Disease

1999 ◽  
Vol 84 (8) ◽  
pp. 2912-2923 ◽  
Author(s):  
Ryszard M. Pluta ◽  
Lynnette Nieman ◽  
John L. Doppman ◽  
Joseph C. Watson ◽  
Nancy Tresser ◽  
...  
Keyword(s):  
Pituitary Gland ◽  
Cushing’S Disease ◽  
Anterior Lobe ◽  
Empty Sella ◽  
Cushing's Disease ◽  
Pars Tuberalis ◽  
Serial Sections ◽  
Resonance Imaging ◽  
High Incidence ◽  
Acth Secreting

Negative sellar exploration (despite the results of endocrine evaluation indicating Cushing’s disease), the high incidence of failure of total hypophysectomy, and remission of Cushing’s syndrome after unsuccessful hypophysectomy and sellar irradiation suggest that the etiology of refractory Cushing’s disease, in some patients, lies near the sella but not in the pituitary gland. We present 5 patients, out of 626 who received surgery for Cushing’s disease, in whom an ACTH-secreting extrapituitary parasellar adenoma was identified: 2 after unsuccessful total hypophysectomy for the treatment of refractory Cushing’s disease, 2 after unsuccessful hemihypophysectomy (the first, 2 yr before treatment at the NIH for Nelson’s syndrome; and the second, with recurrent Cushing’s disease 5 yr after negative transsphenoidal exploration), and 1 with a preoperative diagnosis of an intraclival microadenoma, which was cured by resection of the tumor. In all cases, an extrapituitary parasellar microadenoma was confirmed unequivocally as the cause of the disease, by negative pathology of the resected pituitary gland (patients 1, 2, 3, and 5), and/or the remission of the disease after selective resection of the extrasellar adenoma (patients 3, 4, and 5). Three of 5 patients had a partial empty sella. These patients support the thesis that ACTH-secreting tumors can arise exclusively from remnants of Rathke’s pouch, rather than from the adenohypophysis (anterior lobe or pars tuberalis of the pituitary gland) and can be a cause of Cushing’s disease. In the sixth presented case, an extrapituitary tumor was suspected at surgery after negative pituitary exploration, but serial sections of the hemihypophysectomy specimen revealed a microscopic focus of tumor at the margin of the resected gland. This case demonstrates the importance of negative pituitary histology to establish the presence of an extrapituitary parasellar tumor as an exclusive source of ACTH, and it supports the value of clinical outcome to establish the diagnosis with selective adenomectomy of an extrapituitary parasellar tumor. In patients with negative pituitary magnetic resonance imaging, especially in the presence of a partial empty sella, the diagnostic and surgical approach in Cushing’s disease should consider the identification and resection of extrapituitary parasellar adenoma, which can avoid total hypophysectomy, as was possible in 3 of our 5 patients.

scholarly journals Significant GH deficiency after long-term cure by surgery in adult patients with Cushing’s disease

2007 ◽  
Vol 156 (2) ◽  
pp. 233-239 ◽  
Author(s):  
Francesca Pecori Giraldi ◽  
Massimiliano Andrioli ◽  
Laura De Marinis ◽  
Antonio Bianchi ◽  
Antonella Giampietro ◽  
...  
Keyword(s):  
Replacement Therapy ◽  
Cushing’S Disease ◽  
Gh Deficiency ◽  
Pituitary Surgery ◽  
Male Gender ◽  
Cushing's Disease ◽  
Gh Secretion ◽  
Wide Variability ◽  
Acth Secreting

Objective: Impaired GH secretion usually accompanies Cushing’s syndrome and a variable proportion of patients reportedly fail to recover normal GH secretion after successful treatment. This wide variability is most probably due to differences in the treatment (i.e. surgery and/or radiotherapy), timing of patient re-evaluation after surgery and dynamic tests employed to challenge GH secretion, and hinders a precise assessment of risk of GH deficiency after cure. The aim of the present study is to evaluate GH secretory status after long-term cure of Cushing’s disease achieved by surgery alone. Design and methods: We studied 34 patients (27 females and 7 males, age range 21–68 years) formerly affected by Cushing’s disease. Patients were studied 2–20 years (median 3.3 years) following remission of hypercortisolism; all patients underwent transsphenoidal surgery with the removal of an ACTH-secreting adenoma; repeat pituitary surgery for relapse was performed in two patients while bilateral adrenalectomy was necessary in two patients. In all subjects, the GH response to GHRH+arginine stimulation was evaluated. At the time of testing, 13 patients were still on steroid replacement therapy. Results: In long-term surgical remission, 22 patients (65.0%) presented subnormal GH secretion; partial GH deficiency (GH peak <16.5 μg/l) was found in 11 patients and severe GH deficiency (GH peak <9 μg/l) in another 11. Male gender and length of hypercortisolism were risk factors for postsurgical GH deficiency. Conclusions: This study demonstrates the presence of GH deficiency in a high percentage of patients with Cushing’s disease after long-term remission of hypercortisolism obtained by surgery alone. Male gender and length of hypercortisolism are the most significant predictors of postsurgical GH deficiency. This finding is significant as it highlights that even the most favourable therapeutical course, i.e. remission achieved by surgery alone, is accompanied by impaired GH secretion. Assessment of GH secretion is therefore recommended for all patients cured from Cushing’s disease, even if not submitted to radiotherapy. Studies on the clinical impact of GH deficiency and the use of GH replacement therapy seem warranted in patients cured from Cushing’s disease.

scholarly journals Low immediate postoperative serum-cortisol nadir predicts the short-term, but not long-term, remission after pituitary surgery for Cushing’s disease

2015 ◽  
Vol 15 (1) ◽  
Author(s):  
Jon Ramm-Pettersen ◽  
Helene Halvorsen ◽  
Johan Arild Evang ◽  
Pål Rønning ◽  
Per Kristian Hol ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Pituitary Surgery ◽  
Serum Cortisol ◽  
Cushing's Disease ◽  
Short Term ◽  
Postoperative Serum
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