Prevalence of Signs and Symptoms of Pseudotumor Cerebri Syndrome Before and After Transsphenoidal Surgery for Cushing’s Disease – A Prospective Consecutive Case Series

2020 ◽  
Author(s):  
Torge Huckhagel ◽  
Jörg Flitsch ◽  
Roman Rotermund ◽  
Volker Knospe
Keyword(s):  
Visual Acuity ◽  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Signs And Symptoms ◽  
Pseudotumor Cerebri ◽  
Cushing's Disease ◽  
Pseudotumor Cerebri Syndrome ◽  
Before And After ◽  
Biochemical Remission

Abstract Introduction Pseudotumor cerebri syndrome (PTCS) has anecdotally been described after successful treatment of Cushing’s disease (CD), but the prevalence following transsphenoidal surgery has not been determined so far in a prospective study. Patients and Methods 41 consecutive adult CD patients were prospectively screened for signs and symptoms of PTCS, headache-related impairment, and ophthalmological features associated with intracranial pressure elevation before surgery and at follow-up (mean 4 months). Results Biochemical remission was achieved in 36 of 41 (87.8%) patients after surgery. The most frequent preoperative complaints were visual acuity impairment (19 cases; 46.3%), headache (13 cases; 31.7%), and ear noise (9 cases; 22.0%). A significant reduction of visual disturbances was seen at follow-up. Overall, CD patients presented with fairly low headache-related emotional and functional restrictions before and after surgery. One of 34 (2.9%) patients with sufficient ophthalmological follow-up showed new-onset papilledema combined with temporary worsening of visual acuity and scotoma. Conclusion Our results suggest a very low frequency of signs and symptoms of PTCS after surgical treatment for CD in adults. This estimate is in line with previous outcomes from retrospective pediatric CD series. Further large-scale studies are needed to determine the actual prevalence of this condition following biochemical remission of CD.

scholarly journals Outcomes of endoscopic transsphenoidal surgery for Cushing's disease

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Zarina Brady ◽  
Aoife Garrahy ◽  
Claire Carthy ◽  
Michael W. O’Reilly ◽  
Christopher J. Thompson ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Remission Rate ◽  
Cushing's Disease ◽  
Endoscopic Transsphenoidal Surgery ◽  
Endocrine Society ◽  
Csf Leakage ◽  
Remission Rates

Abstract Background Transsphenoidal surgery (TSS) to resect an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is the first-line treatment for Cushing’s disease (CD), with increasing usage of endoscopic transsphenoidal (ETSS) technique. The aim of this study was to assess remission rates and postoperative complications following ETSS for CD. Methods A retrospective analysis of a prospective single-surgeon database of consecutive patients with CD who underwent ETSS between January 2012–February 2020. Post-operative remission was defined, according to Endocrine Society Guidelines, as a morning serum cortisol < 138 nmol/L within 7 days of surgery, with improvement in clinical features of hypercortisolism. A strict cut-off of < 50 nmol/L at day 3 post-op was also applied, to allow early identification of remission. Results A single surgeon (MJ) performed 43 ETSS in 39 patients. Pre-operative MRI localised an adenoma in 22 (56%) patients; 18 microadenoma and 4 macroadenoma (2 with cavernous sinus invasion). IPSS was carried out in 33 (85%) patients. The remission rates for initial surgery were 87% using standard criteria, 58% using the strict criteria (day 3 cortisol < 50 nmol/L). Three patients had an early repeat ETSS for persistent disease (day 3 cortisol 306-555 nmol/L). When the outcome of repeat early ETSS was included, the remission rate was 92% (36/39) overall. Remission rate was 94% (33/35) when patients with macroadenomas were excluded. There were no cases of CSF leakage, meningitis, vascular injury or visual deterioration. Transient and permanent diabetes insipidus occurred in 33 and 23% following first ETSS, respectively. There was one case of recurrence of CD during the follow-up period of 24 (4–79) months. Conclusion Endoscopic transsphenoidal surgery produces satisfactory remission rates for the primary treatment of CD, with higher remission rates for microadenomas. A longer follow-up period is required to assess recurrence rates. Patients should be counselled regarding risk of postoperative diabetes insipidus.

Long term follow-up of transsphenoidal surgery for the treatment of Cushing's disease in childhood.

1995 ◽  
Vol 80 (8) ◽  
pp. 2475-2479 ◽  
Author(s):  
M C Leinung ◽  
L A Kane ◽  
B W Scheithauer ◽  
P C Carpenter ◽  
E R Laws ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Cushing's Disease ◽  
Long Term Follow Up

Results of repeated transsphenoidal surgery in Cushing's disease. Long-term follow-up

2014 ◽  
Vol 61 (4) ◽  
pp. 176-183 ◽  
Author(s):  
Pablo Valderrábano ◽  
Javier Aller ◽  
Leopoldo García-Valdecasas ◽  
José García-Uría ◽  
Laura Martín ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Cushing's Disease ◽  
Long Term Follow Up

Radiosurgery for Cushing's disease after failed transsphenoidal surgery

2000 ◽  
Vol 93 (5) ◽  
pp. 738-742 ◽  
Author(s):  
Jonas M. Sheehan ◽  
Mary L. Vance ◽  
Jason P. Sheehan ◽  
Dilantha B. Ellegala ◽  
Edward R. Laws
Keyword(s):  
Gamma Knife ◽  
Cushing’S Disease ◽  
Tumor Size ◽  
Transsphenoidal Surgery ◽  
Gamma Knife Radiosurgery ◽  
Magnetic Resonance Images ◽  
Cushing's Disease ◽  
Content Type ◽  
Fine Print

Object. Although transsphenoidal surgery has become the standard of care for Cushing's disease, it is often unsuccessful in normalizing cortisol production. In this study the authors investigate the safety and efficacy of gamma knife radiosurgery (GKRS) for Cushing's disease after failed transsphenoidal surgery.Methods. The records of all patients who underwent GKRS at the authors' institution after unsuccessful transsphenoidal surgery for Cushing's disease were retrospectively reviewed. Successful treatment was considered a normal or below-normal 24-hour urinary free cortisol (UFC) level. Records were also evaluated for relapse, new-onset endocrine deficiencies, interval change in tumor size, and visual complications.Forty-three patients underwent 44 gamma knife procedures with follow up ranging from 18 to 113 months (mean 39.1, median 44 months). Normal 24-hour UFC levels were achieved in 27 patients (63%) at an average time from treatment of 12.1 months (range 3–48 months). Three patients had a recurrence of Cushing's disease at 19, 37, and 38 months, respectively, after radiosurgery. New endocrine deficiencies were noted in seven patients (16%). Follow-up magnetic resonance images obtained in 33 patients revealed a decrease in tumor size in 24, no change in nine, and an increase in size in none of the patients. One patient developed a quadrantanopsia 14 months after radiosurgery despite having received a dose of only 0.7 Gy to the optic tract.Conclusions. Gamma knife radiosurgery appears to be safe and effective for the treatment of Cushing's disease refractory to pituitary surgery. Delayed recurrences and new hormone deficiencies may occur, indicating the necessity for regular long-term follow up.

scholarly journals The Complete Normalization of the Adrenocortical Function as the Criterion of Cure after Transsphenoidal Surgery for Cushing’s Disease

2001 ◽  
Vol 86 (12) ◽  
pp. 5695-5699 ◽  
Author(s):  
Javier Estrada ◽  
José García-Uría ◽  
Cristina Lamas ◽  
José Alfaro ◽  
Tomás Lucas ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Plasma Cortisol ◽  
Cushing's Disease ◽  
Adrenocortical Function ◽  
Urinary Cortisol ◽  
Group Iii ◽  
Group I ◽  
Group Ii

Transsphenoidal microsurgery is the standard treatment for patients with Cushing’s disease. However, there is general lack of agreement regarding the definition of cure. We studied 58 patients with corrected hypercortisolism after transsphenoidal surgery for Cushing’s disease. Plasma and urinary cortisol levels were measured after surgery. After the postsurgical hypocortisolism stage (or periodically in patients without hypocortisolism), urinary free cortisol, plasma cortisol at 0800 h and 2300 h, morning cortisol after 1 mg dexamethasone, and cortisol response to insulin-induced hypoglycemia were performed. Patients were classified in 3 groups: group I, patients with transient hypocortisolism and normal hypothalamus-pituitary-adrenal axis afterwards; group II, patients with transient hypocortisolism and abnormalities in the circadian rhythm or the stress response afterwards; and group III, patients without postoperative hypocortisolism. Thirty-three patients were included in group I, 8 in group II, and 17 in group III. Groups I and II were similar in postsurgical plasma cortisol (46.9 ± 30.3 vs. 60.7 ± 38.6 nm) and mean follow-up (69.8 vs. 68.8 months) but were significantly different in their recurrence rate (3.4% vs. 50%, P &lt; 0.001). Patients in group III had normal postsurgical plasma and urinary cortisol but persistent abnormalities in circadian rhythm and stress response. After a mean follow-up of 39.1 months, their recurrence rate was similar to that of group II (64.7% vs. 50%). The complete normalization of the adrenocortical function, which is always preceded by postsurgical hypocortisolism, is associated with a very low recurrence risk and should be considered, in our opinion, the main criterion of surgical cure in Cushing’s disease.

scholarly journals Delayed Remission after Transsphenoidal Surgery in Patients with Cushing’s Disease

2010 ◽  
Vol 95 (2) ◽  
pp. 601-610 ◽  
Author(s):  
Elena Valassi ◽  
Beverly M. K. Biller ◽  
Brooke Swearingen ◽  
Francesca Pecori Giraldi ◽  
Marco Losa ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Tertiary Care ◽  
Case Series ◽  
Expectant Management ◽  
Pituitary Surgery ◽  
Cushing's Disease ◽  
Optimal Timing ◽  
Retrospective Case

Abstract Background: Transsphenoidal surgery (TSS) is the treatment of choice for Cushing’s disease (CD). Postoperative hypercortisolemia mandates further therapy. Objective: The aim of the study was to characterize patients without immediate postoperative remission who have a delayed decrease to normal or low cortisol levels without further therapy. Design and Setting: A retrospective case series was conducted at three tertiary care centers. Patients and Intervention: We reviewed the records of 620 patients (512 females, 108 males; mean age, 38 ± 13 yr) who underwent transsphenoidal pituitary surgery for CD between 1982 and 2007. Results: Outcomes were classified into the following three groups based upon the postoperative pattern of cortisol testing: group IC (immediate control) included 437 of the 620 patients (70.5%) with hypocortisolism and/or cortisol normalization throughout the postoperative follow-up; group NC (no control) included 148 of 620 patients (23.9%) with persistent hypercortisolism; and group DC (delayed control) included 35 of 620 patients (5.6%) who had early elevated or normal UFC levels and developed a delayed and persistent cortisol decrease after an average of 38 ± 50 postoperative days. The total rate of recurrence was 13% at a median follow-up time of 66 months after TSS; the cumulative rate of recurrence at 4.5 yr was significantly higher in group DC vs. group IC (43 vs. 14%; P = 0.02). Conclusions: Hormonal assessment in the immediate postoperative period after TSS for CD may be misleading because delayed remission can occur in a subset of patients. Expectant management and retesting may spare some patients from unnecessary further treatment. Optimal timing to determine the need for further therapy after TSS remains to be determined.

Transsphenoidal Surgery for Cushing's Disease: Endocrinological Follow-up Monitoring of 82 Patients

Neurosurgery ◽  
2002 ◽  
Vol 51 (1) ◽  
pp. 57-62 ◽  
Author(s):  
Ilan Shimon ◽  
Zvi Ram ◽  
Zvi R. Cohen ◽  
Moshe Hadani
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Adrenocorticotropic Hormone ◽  
Remission Rate ◽  
The United States ◽  
Cushing's Disease ◽  
Free Cortisol ◽  
Remission Rates ◽  
Surgical Failure

Abstract OBJECTIVE Transsphenoidal surgery is the preferred treatment modality for adrenocorticotropic hormone-secreting pituitary adenomas. In the past 2 decades, several institutions in the United States and Europe have reported remission rates of 70 to 85% after transsphenoidal surgery for treatment of Cushing's disease. We analyzed our postoperative results for a large cohort of patients with Cushing's disease. METHODS Eighty-two patients with adrenocorticotropic hormone-secreting adenomas (79 microadenomas and 3 macroadenomas) underwent transsphenoidal surgery between 1990 and 2000. Seventy-seven patients were surgically treated for the first time, and 13 patients underwent reoperations (5 had undergone the first operation elsewhere) because of previous surgical failure (10 patients) or recurrence (3 patients). The mean postoperative follow-up period was 4.2 ± 2.8 years. Biochemical remission was defined as postoperative normalization of elevated 24-hour urinary free cortisol secretion and suppression of morning cortisol levels with 1 mg of dexamethasone. RESULTS Remission was achieved for 78% of all patients after one operation and for 62% of patients who underwent a second operation. The recurrence rate was 5%. Ten patients did not exhibit a visible tumor on magnetic resonance imaging scans, and the other patients were divided according to adenoma size (2–5 or 6–10 mm). Remission rates were similar for the three groups of patients (78–80%). Pituitary tumor stained for adrenocorticotropic hormone was detected in 78% of resected pituitary tissue specimens obtained from patients who achieved remission, compared with 53% from patients who experienced surgical failure (P = 0.06). CONCLUSION Our series demonstrates the efficacy of transsphenoidal surgery for Cushing's disease resulting from pituitary microadenomas. Microadenoma size had no effect on the remission rate. Reoperations are indicated after initial surgical failures.

scholarly journals Ketoconazole revisited: a preoperative or postoperative treatment in Cushing's disease

2008 ◽  
Vol 158 (1) ◽  
pp. 91-99 ◽  
Author(s):  
F Castinetti ◽  
I Morange ◽  
P Jaquet ◽  
B Conte-Devolx ◽  
T Brue
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Urinary Free Cortisol ◽  
Postoperative Treatment ◽  
Cushing's Disease ◽  
Intention To Treat ◽  
Free Cortisol ◽  
In Series

ContextAlthough transsphenoidal surgery remains the first-line treatment in Cushing's disease (CD), recurrence is observed in about 20% of cases. Adjunctive treatments each have specific drawbacks. Despite its inhibitory effects on steroidogenesis, the antifungal drug ketoconazole was only evaluated in series with few patients and/or short-term follow-up.ObjectiveAnalysis of long-term hormonal effects and tolerance of ketoconazole in CD.DesignA total of 38 patients were retrospectively studied with a mean follow-up of 23 months (6–72).SettingAll patients were treated at the same Department of Endocrinology in Marseille, France.PatientsThe 38 patients with CD, of whom 17 had previous transsphenoidal surgery.InterventionKetoconazole was begun at 200–400 mg/day and titrated up to 1200 mg/day until biochemical remission.Main outcome measuresPatients were considered controlled if 24-h urinary free cortisol was normalized.ResultsFive patients stopped ketoconazole during the first week because of clinical or biological intolerance. On an intention to treat basis, 45% of the patients were controlled as were 51% of those treated long term. Initial hormonal levels were not statistically different between patients controlled or uncontrolled. Ketoconazole was similarly efficacious as a primary or postoperative treatment. Among 15 patients without visible adenoma at initial evaluation, subsequent follow-up allowed identification of the lesion in five cases. No adrenal insufficiency was observed. Adverse effects were rare in patients treated long term.ConclusionsKetoconazole is a safe and efficacious treatment in CD, particularly in patients for whom surgery is contraindicated, or delayed because of the absence of image of adenoma on magnetic resonance imaging.

scholarly journals Usefulness of desmopressin testing to predict relapse during long-term follow-up in patients in remission from Cushing’s disease

2017 ◽  
Vol 6 (8) ◽  
pp. 791-799 ◽  
Author(s):  
Alberto Giacinto Ambrogio ◽  
Massimiliano Andrioli ◽  
Martina De Martin ◽  
Francesco Cavagnini ◽  
Francesca Pecori Giraldi
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Response Pattern ◽  
Disease Recurrence ◽  
Cushing's Disease ◽  
Close Monitoring ◽  
Long Term Follow Up ◽  
Over Time

Recurrence of Cushing’s disease after successful transsphenoidal surgery occurs in some 30% of the patients and the response to desmopressin shortly after surgery has been proposed as a marker for disease recurrence. The aim of the present study was to evaluate the response to desmopressin over time after surgery. We tested 56 patients with Cushing’s disease in remission after transsphenoidal surgery with desmopressin for up to 20 years after surgery. The ACTH and cortisol response to desmopressin over time was evaluated in patients on long-term remission or undergoing relapse; an increase by at least 27 pg/mL in ACTH levels identified responders. The vast majority of patients who underwent successful adenomectomy failed to respond to desmopressin after surgery and this response pattern was maintained over time in patients on long-term remission. Conversely, a response to desmopressin reappeared in patients who subsequently developed a recurrence of Cushing’s disease, even years prior to frank hypercortisolism. It appears therefore that a change in the response pattern to desmopressin proves predictive of recurrence of Cushing’s disease and may indicate which patients require close monitoring.

Ghrelin and adiponectin in patients with Cushing's disease before and after successful transsphenoidal surgery

2005 ◽  
Vol 62 (1) ◽  
pp. 30-36 ◽  
Author(s):  
Rossella Libe ◽  
Paola Silvia Morpurgo ◽  
Vincenzo Cappiello ◽  
Antonia Maffini ◽  
Sara Bondioni ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Cushing's Disease ◽  
Before And After
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