Secondary Empty Sella Syndrome Research Articles

Differences in checkpoint-inhibitor-induced hypophysitis: mono- versus combination therapy induced hypophysitis.

Immune checkpoint inhibitors (ICIs) are revolutionary in oncology but may cause immune-related (IR) side effects, such as hypophysitis. Treatment with anti-PD-(L)1, anti-CTLA-4 or anti-CLTA-4/PD-1 may induce hypophysitis, but little is known about the differences in clinical presentation or need for different treatment. We analyzed the differences of anti-PD-(L)1, anti-CTLA-4 and anti-CTLA-4/PD-1 induced hypophysitis. retrospective analysis of 67 patients (27 anti-PD-(L)1, 6 anti-CLTA-4 and 34 anti-CTLA-4/PD-1 induced hypophysitis). The median time between starting ICIs and IR-hypophysitis was longer after anti-PD(L)-1) therapy (22 weeks versus 11 and 14 weeks after anti-CTLA-4 and anti-CTLA-4/PD-1 therapy, respectively). The majority of patients (>90%), presented with atypical complaints such as fatigue, nausea, and muscle complaints. Headache, TSH or LH/FSH deficiency were more common in anti-CTLA-4 and anti-CLTA-4/PD-1 versus anti-PD-(L)1 induced hypophysitis (83% and 58% versus 8%, 67% and 41% versus 11%, and 83% and 48% versus 7%, respectively). Pituitary abnormalities on MRI (hypophysitis or secondary empty sella syndrome) were only seen in patients receiving anti-CTLA-4 or anti-CTLA-4/PD-1 therapy. Recovery from TSH, LH/FSH and ACTH deficiency was described in 92%, 70% and 0% of patients after a mean period of 14 and 104 days, respectively, and did not differ between patients who did or did not receive high-dose steroids. The clinical presentation of IR-hypophysitis varies depending on the type of ICIs. MRI abnormalities were only seen in anti-CTLA-4 or anti-CTLA-4/PD-1 induced hypophysitis. Endocrine recovery is seen for LH/FSH and TSH deficiency but not for ACTH deficiency, irrespective of the corticosteroid dose.

A rare case of partially empty sella with epileptiform discharges presenting with first episode of mania.

Sir, Busch described the condition empty sella syndrome (ESS) in 1951 where the sella turcica is filled with cerebrospinal fluid (CSF) causing displacement or flattening of the normal pituitary gland.[1] It is reported to occur in 5.5%–23.5% of the population[2] which is pathophysiologically characterized by either anatomic abnormalities in the diaphragma sellae (primary ESS) or damage to the pituitary by irradiation/surgery, or autoimmunity leading to the availability of “empty” space in the sella (secondary ESS).[1] The common signs and symptoms includes headache, papilledema due to raised intracranial pressure, leakage of CSF through nose and weight gain.[1] It can also present as decreased libido, galactorrhea, amenorrhea and secondary infertility.[3] Psychiatric manifestations of ESS are rare but can occur in the form of behavioral disturbances and psychotic symptoms.[4,5] A 25-year-old male presented to us with violent behavior towards family members, reduced need for sleep, elevated self-esteem and grandiose delusions claiming himself to be the prime minister of India. It was associated with one episode of tonic–clonic movements two nights back, with urinary incontinence. Patient also complained of jerky movements in his left arm occasionally about two to three times a day following this. All the symptoms started abruptly for past 10 days, this being his first such episode in his lifetime. There was no history of any febrile illness, head injury, substance abuse; blood parameters that included serum electrolytes, serum prolactin, vitamin B12 levels, and serum osmolality were all within normal limit. He was diagnosed to be in mania as per Diagnostic and Statistical Manual of Mental Disorders (DSM 5). Young’s Mania Rating Scale (YMRS) score was 24. The patient was subjected to magnetic resonance imaging (MRI) scan which revealed empty sella [Figure 1]. 10–20 electroencephalogram findings revealed generalized periodic epileptiform discharges (GPED). The patient was placed on sodium valproate 1000mg, haloperidol 10 mg and trihexyphenidyl 4 mg in divided dosages. The symptoms reduced in severity and YMRS score came 12 on day 5 of admission and EEG was normal. This was his first episode of mania that coexisted with first lifetime episode of seizure. GPEDs results in kindling of neuronal pathways, leading to psychiatric manifestations such as mania. Lateral hypothalamic kindling can induce manic-like behavior in a rat model.[5]Figure 1: MRI scan showing empty sellaThere are reports of atypical presentations of ESS in the form of acute psychosis, sinus bradycardia, herniation of anterior cerebral artery.[5–7] The presentation of empty sella as seizure episode has been reported rarely.[8] Moreover, it is even more rare to be presenting with both seizure episode and mania.[9] In one case report mania was precipitated by hyponatremia in a patient with ESS[10] whereas in our case sodium and other electrolytes level were in normal range. The possible mechanism of such is still a gray area.[9] Even there is also a dilemma of seizure precipitating mania or vice-versa. More sample based studies is needed to establish the neurochemical events leading to the same for better diagnosis and management. Ethical concerns Proper consent was taken from the patient regarding publication of the case without revealing patient’s identity Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Partially empty sella with generalized epileptiform discharges presented as manic episode in an elderly female: A case with diagnostic dilemma - Causation or coincidence?

Partially empty sella with generalized epileptiform discharges presented as manic episode in an elderly female: A case with diagnostic dilemma - Causation or coincidence?

Chiasmapexy for secondary empty sella syndrome: diagnostic and therapeutic considerations.

Secondary empty sella syndrome (SESS) following pituitary surgery remains a diagnostic and therapeutic challenge. The aim of this study was to specify the diagnostic criteria, surgical indications and results of chiasmapexy in the SESS. Three cases from two experienced neurosurgical centers were collected and the available literature was reviewed. The 3 patients were operated for a giant non-functioning pituitary adenoma, a cystic macroprolactinoma, and an arachnoid cyst respectively. Postoperative visual outcome was initially improved, and then worsened progressively. At the time of SESS diagnosis, visual field defect was severe in all cases with optic nerve (ON) atrophy in 2 cases. Patients were operated via an endoscopic endonasal extradural approach. One patient was re-operated because of early fat reabsorption. Visual outcome improved in 1 case and stabilized in 2 cases. Statistical analyses performed on 24 cases from the literature review highlighted that patient age and severity of the preoperative visual defect were respectively significant and nearly significant prognostic factors for visual outcome, unlike the surgical technique. T2-weighted or CISS/FIESTA sequence MRI is mandatory to visualize adhesions, ON kinking and neurovascular conflict. TS approach is the most commonly used approach. The literature review could not conclude on the need for an intra or extradural approach suggesting case by case adapted strategy. Intrasellar packing with non-absorbable material such as bone should be considered. Severity of the visual loss clearly decreases the visual outcome suggesting early chiasmapexy. In case of severe and long standing symptoms before surgery, benefits and surgical risks should be carefully balanced.

SUN-LB072 Symptomatic Refractory Hypoglycemia as the Initial Prentation of Primary Empty Sella

Introduction: Empty Sella syndrome (ESS) is characterized by subarachnoid space herniating into the Sella turcica, causing displacement or flattening of the pituitary gland. Primary ESS has an unknown etiology, secondary ESS may be caused by trauma, infection, adenoma, ischemia, surgery, pharmacological or radiological Rx. It has been noted in 5-23% of the population, usually found incidentally in autopsies or imaging. 25% to 50% of patients have endocrine abnormalities (panhypopituitarism, DI, DM type 2, irregular menses). Headache or ophthalmological symptoms may be seen. Case summary: 55-year-old AAF with Hx of anemia, presented with lethargy and acute encephalopathy after several months of progressively worsening dizziness, muscle weakness, and fatigue. For the past few days, her appetite was poor. She also complained of cold intolerance, brittle hair, dry skin, and occasional constipation. Denied headache, blurry vision, neck pain, palpitation, previous head imaging, trauma or surgery. Remote history included an uncomplicated pregnancy; menopause occurred several years ago. Found to be hypoglycemic with blood glucose 35 mg/dl. Six D50 ampules initially improved her hypoglycemia but blood glucose quickly dropped thereafter. Placed on D5 drip; with the same results. A&O to place and person only, lacked a goiter. Lab work revealed low morning serum cortisol <1.2 mcg/dl (10-20 mcg/dl),high TSH 37.1 mU/L (0.4-4.5Mu/L), low free T4 0.2 mcg/dl (4.5-11.2 mcg/dl), low insulin 1.6 mcU/ml (4-30 mcU/dl), low C peptide 0.48 ng/ml (1.1-22 ng/ml), elevated prolactin 31 ng/ml (2-29 ng/dl), and low FSH 10 mlU/ml (26-134 mlU/ml). ACTH stimulation test revealed adrenal insufficiency. Started on IV Dexamethasone 2mg BID, hypoglycemia resolved (later weaned to prednisone 5mg BID). In view of hypothyroidism, adrenal insufficiency, and low FSH in post-menopause, hypopituitarism was suspected. MRI brain revealed empty sella without micro- or macroadenoma. Discussion & Conclusion: PES is usually asymptomatic but can present with endocrine, neurologic, or visual symptoms. A fraction of patients present with nonspecific headache, dizziness, or cranial nerve disorders. Others may have ophthalmological symptoms such as blurry or decreased vision, or mild papilledema. Though mild hyperprolactinemia and various levels of hypopituitarism have been noted, it is rare that adult patients are hospitalized for undiagnosed hypopituitarism with refractory hypoglycemia or severe hyponatremia. Panhypopituitarism is noted in only 2% of ESS patients. Our case represents a rare clinical presentation of primary ESS, with no known trauma, surgery, or ischemia. As clinicians, we should include ESS in our differential when assessing patients presenting with hypopituitarism, refractory hypoglycemia, or neurological/visual symptoms. MRI brain should be obtained if ESS is suspected. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.

Этиопатогенетическая роль вторичного синдрома «пустого турецкого седла» в генезе эректильной дисфункции

Этиопатогенетическая роль вторичного синдрома «пустого турецкого седла» в генезе эректильной дисфункции

Treatment of empty sella associated with visual impairment: a systematic review of chiasmapexy techniques.

Chiasmapexy is a poorly described surgical procedure adopted to correct the downward displacement of suprasellar visual system (SVS) into an empty sella (ES) causing visual worsening. The aim of our study is to define the indications for extradural and intradural chiasmapexy. A systematic literature review has been performed on MEDLINE database (US National Library of Medicine), including only articles that depicted cases of surgically treated patients affected by ES and progressive delayed visual worsening. Moreover, we have reported three cases of secondary ES syndrome (SESS) with visual worsening treated in our Department with transsphenoidal (TS) microsurgical intradural approach. Finally, we have compared the results of extradural and intradural chiasmapexy described in literature. The etiology of visual impairment is different in primary and secondary ESS. In primary ESS (PESS) the only predisposing factor is a dehiscence of diaphragma sellae, and the anatomical distortion caused by displacement of optic chiasm or traction of pituitary stalk and infundibulum may determine a direct injury of neural fibers and ischemic damage of SVS. In PESS the mechanical elevation of SVS performed through extradural approach is sufficient to resolve the main pathologic mechanism. In SESS, arachnoidal adhesions play an important role in addition to downward herniation of SVS. Consequently, the surgical technique should provide elevation of SVS combined to intradural release of scar tissue and arachnoidal adhesions. In treatment of SESS, the intradural approaches result to be more effective, guaranteeing the best visual outcomes with the lowest complications rates. The intradural chiasmapexy is indicated in treatment of SESS, instead the extradural approaches are suggested for surgical management of PESS.

Pseudotumor cerebri resulting in empty sella syndrome and multiple pituitary hormone deficiencies

Background: Pseudotumor cerebri is frequently associated with an empty sella turcica in neurologic literature. This radiologic abnormality is commonly asymptomatic, but it may present with non-specific neurological symptoms or more rarely with pituitary dysfunction. This case highlights a patient with pseudotumor cerebri and secondary empty sella syndrome whose symptoms of hypopituitarism were attributed to the pseudotumor cerebri, chronic pain, and medication side effects and resulted …

Reconstruction of the Intrasellar Tumor Resection Cavity Is Not Necessary to Prevent Optic Chiasm Prolapse Following Transsphenoidal Removal of Pituitary Macroadenomas

Introduction: Secondary empty sella syndrome associated with delayed visual deterioration has been reported following transsphenoidal resection of pituitary adenomas. To prevent postoperative herniation of the optic apparatus, reconstruction of the intrasellar tumor resection cavity with autologous tissues such as adipose tissue or muscle is commonly employed. Harvesting of such tissues may be complicated by cosmetic deformity, infection, or hematoma formation. In our practice, the resection cavity is not reconstructed, and we sought to determine the incidence of postoperative optic chiasm prolapse following removal of pituitary macroadenomas.

Third ventricle herniation into the sphenoid sinus following endoscopic transnasal transsphenoidal fenestration of rathke's cleft cyst

Rathke cleft cyst (RCC) is an uncommon albeit benign sellar lesion with an incidence rate of between 2 to 33%. RCCs are usually asymptomatic except in the large cases whit suprasellar extension. We herein describe a unique case of RCC, which presented with severe visual loss owing to massive herniation of the optic chiasm and third ventricle down into the sphenoid sinus through a small 8 x 8 mm foramen after transnasal endoscopic surgical fenestration and marsupialization of the cyst. We describe a reconstruction method via endonasal transsphenoidal approach in this case and suggest prophylactic reconstruction of the sellar floor in sellar lesions with equal or more voluminous suprasellar extensions that are susceptible to such massive herniation and secondary empty sella syndrome.

Transsphenoidal Treatment of Secondary Empty Sella Syndrome using Low Field Strength Intraoperative MRI: Case Report

The purpose of this study is to demonstrate the added value of intraoperative MRI in treating secondary empty sella syndrome. We describe the case of a 66-year-old woman who was diagnosed with a prolactinoma stage IIIb. During treatment with cabergoline she presented with a secondary empty sella syndrome resulting in visual symptoms. We performed intraoperative MRI-guided packing of the secondary empty sella. We explain why this is useful in surgical treatment of secondary empty sella syndrome. Intraoperative MRI helps to achieve adequate sellar packing while avoiding insufficient packing as well as overpacking.

Analysis of Secondary Empty Sella Syndrome Due to Brain Tumors by Brain MRI: A Preliminary Report

Analysis of Secondary Empty Sella Syndrome Due to Brain Tumors by Brain MRI: A Preliminary Report

Analysis of Secondary Empty Sella Syndrome Due to Brain Tumors by Brain MRI: A Preliminary Report

We experienced a case of delayed postoperative CSF rhinorrhea after the removal of a huge falx meningioma by the parieto-occipital interhemispheric approach. Retrograde review of the MRI revealed empty sella and subsequent Isovist CT cisternography disclosed the site of leakage, which was managed by a trans-sphenoidal approach. In order to avoid such cases we have made an analysis of brain MRIs to assist in detecting secondary empty sella syndromes. The authors retrospectively reviewed the preoperative MRIs of 53 cases of large brain tumors regardless of pathology, exempting pituitary tumors. Retrospective analysis of empty sella and review of brain MRI was done. The pituitary fossa was enlarged and filled partially or fully with CSF. Empty sella was evident in 60.3% of cases (32/53) and negative in 39.7% of cases (21/53). The incidence of secondary empty sella by pathology was as follows: (1) 20 out of 24 cases (83.3%) in meningiomas excluding 4 cases where the tumor had invaded the pituitary fossa (out of total 28 cases); (2) 55.6% (5/9 cases) in gliomas; (3) 0 cases out of 1 case in ganglioma; (4) 2/3 cases in hemangioblastomas; (5) 1/3 cases in acoustic schwannoma; (6) 2/2 cases in NF2; (7) 1/1 case of hemangiopericytoma; (8) 0/1 case lymphoma; (9) 1/2 cases lymphoma; (10) 0/1 case of PNET; (11) 0/1 case in AT & RT. Increased ICP attributed to long-standing, relatively large or huge benign tumor, causes compression of the CSF pathway, resulting in the herniation of the basal cisternal arachnoid membrane through a weakening in the diaphragm sellae into the pituitary fossa. This is generally regarded as the pathogenesis of secondary empty sella syndrome. The analysis showed that relatively slow-growing meningiomas with the exception of cavernous sinus, tuberculum sella, and clinoidal meningioma that directly invaded the pituitary fossa had a high incidence of secondary empty sella (83.3%), while rapid-growing glial tumors whose pathomechanism is invasion rather than ICP elevation showed lower incidence (55.6%). The cases of other pathologies were too few to analyze their respective incidences. Careful review of brain MRI with periodic follow-up is necessary for the detection of secondary empty sella in patients with brain tumor. In patients with confirmed empty sella, follow-up is mandatory because management of hypopituitarism, CSF rhinorrhea, visual disturbance, and increased ICP is necessary. Further accumulation of cases and analysis is required.

Delayed visual deterioration after pituitary surgery ? a review introducing the concept of vascular compression of the optic pathways

Delayed visual deterioration after pituitary surgery has been attributed to secondary empty sella syndrome and downward herniation of the optic nerves and chiasm, but the pathophysiological basis of this condition is still a matter of debate. According to the literature, prior radiation therapy, previous visual impairment and transcranial surgery constitute risk factors for delayed visual deterioration. Radiation-induced vascular changes and/or strangulation of the optic nerves or chiasm are thought to compromise local blood flow. Downward herniation of the optic pathways was present in the majority of cases, but did not correlate with visual symptoms and signs, while dense scarring of the chiasm was a reproducable finding in all surgically explored cases. Indentations in the upper margin of the optic nerves or chiasm caused by the A1 segments of the anterior cerebral arteries have been reported repeatedly. As perichiasmal scarring constitutes the most consistent finding, the intimate relationship between artery and nerve with consecutive pulsatile pressure may constitute a causative factor in delayed visual dysfunction after pituitary surgery. The authors therefore introduce the concept of vascular compression, which is illustrated with a personal case of a successful decompression procedure with teflon interposition between the A1 segment and the non-herniated optic nerve to treat visual loss eight months following removal of a hemorrhagic pituitary adenoma. Clinicians should be aware that surgical exploration via a transcranial approach is indicated in cases of progressive visual loss late after pituitary surgery, no matter whether downward displacement of the optic pathways is present on imaging studies or not. Special attention should be paid intra-operatively to the dissection of the intimate relationship between the anterior cerebral arteries and the optic nerves and chiasm.

Secondary partial empty sella syndrome in an elite bodybuilder

The pituitary gland is a hormone-responsive gland and is known to vary in size depending on the hormonal status of the patient snd the multifaceted positive and negative feedback hypothalamic-pituitary-gonadal axis. Partial empty sella syndrome with an atrophied pituitary gland is seen in primary neuroendocrinopathies such as growth hormone deficiency, primary hypothyroidism, central diabetes insipidus and hypogonadism. Partial empty sella has also been shown to occur in patients with elevations in intracranial pressure. Secondary partial empty sella syndrome with significant pituitary gland atrophy from negative feedback inhibition of long-term exogenous hormonal use has not been previously reported. We are reporting on a case of partial empty sella syndrome occurring in an elite bodybuilder with a long history of exogenous abuse of growth hormone, testosterone and thyroid hormone. The pathophysiologal mechanisms of secondary partial empty sella syndrome from exogenous hormone use and the possibility for elevations in intracranial pressure contributing to this syndrome will be discussed.

Goldmann perimetry in acromegaly: A survey of 307 cases from 1951 through 1996

Objective Because visual pathway lesions are a common complication of pituitary tumors, visual field examinations in patients with acromegaly were studied. Proportion and outcome of visual field defects in patients with acromegaly were evaluated. Design Large, retrospective case series. Participants We reviewed 307 cases of acromegaly seen from 1951 through 1996 at a single referral center. Methods Kinetic visual field testing had been performed with the Goldmann perimeter, and the frequency of visual field defects and their correlation with other clinical manifestations and characteristics of the adenoma were examined. Main outcome measures Repeat visual field examinations. Results Of the 307 patients included in the analysis, a visual field defect that could be attributed to the pituitary adenoma was observed in 62 (20.2%) during follow-up. Visual field defects were bilateral in 38 (61.3%) of these cases. Patients with visual field abnormalities were significantly younger ( P = 0.04), had larger tumors ( P < 0.001), had more suprasellar extensions ( P < 0.001), and had higher levels of growth hormone in their serum ( P = 0.04) than patients free of visual field defects. At the end of the follow-up period, visual field examination remained abnormal in 32 (10.4%). Return to a normal visual field examination after treatment was more frequently observed in patients who were less than 40 years of age at the time of diagnosis ( P = 0.004). Secondary empty sella syndrome was the main cause of visual field defects after treatment. Abnormal visual field, either at the time of diagnosis or during follow-up, decreased from 27% of patients between 1951 and 1975 to 15.4% of patients between 1976 and 1996, when modern neuroimaging techniques became available. Conclusions Endocrinologic and neuroimaging follow-up of patients with acromegaly should be accompanied by ophthalmic assessment. Factors predictive of visual field defects have been identified.

The management of pituitary tumours and post-operative visual deterioration.

The indications and results of transsphenoidal surgery for pituitary adenomas are analysed. The advantages and disadvantages of Bromocriptine and radiotherapy are considered. Transsphenoidal surgery does not damage pre-existing pituitary function. It produces excellent results in micro and mesoadenomas, curing about 70-80% of patients irrespective whether the tumour produces ACTH, prolactin or growth hormone. Moreover there is a very small relapse rate. Macroadenomas of non-functioning type are also well treated by transphenoidal surgery, with a small recurrence rate as judged by CT scan follow-up. But macroadenomas secreting prolactin or growth hormone are less effectively treated by transsphenoidal surgery, only about 40% being cured. Invasive macro prolactinomas are not helped by surgery and should be treated with Bromocriptine and radiotherapy. Radiotherapy should be used sparingly and has inevitable complications. Post-operative delayed visual deterioration is caused either by recurrent tumour or radiotherapy. There is no good evidence to support the secondary empty sella syndrome as a cause of such delayed visual deterioration.

Endocrinology of Pseudotumor Cerebri

This article will review the endocrine function of obese young women with pseudotumor cerebri and primary empty sella syndrome. The literature can be difficult to interpret. Much of it predates the era of CT scanning. Often, cases of primary and secondary empty sella syndrome are comingled. The author reviews specific endocrinologic disorders that have sometimes been associated with increased intracranial pressure excluding pituitary adenomas.