RARE-13. PRIMARY EMPTY SELLA SYNDROME AND PSEUDO-TUMOR CEREBERI: CORRELATION OF ASSOCIATION AND SURGICAL PLAN

Abstract Introduction Management of primary empty sella syndrome (ESS) is generally remaining a neurosurgical challenge due to lack of a well standardized approach. Pseudo-tumor cereberi (Benign or idiopathic intracranial hypertension) is commonly associated condition. In this study, we have demonstrated the relationship and surgical plan and outcome of such cases. Patients and Methods: We retrospectively studied 24 patients with primary empty sella syndrome (ESS) for two years who were diagnosed radiologically as ESS. Fundus and other ophthalmological examinations were done. Lumbar puncture and cerebrospinal fluid (CSF) manometer were evaluated in those with papilledema. All patients’ data were collected and analyzed. Results Basically, 24 patients (18 females and 6 males) were radiologically diagnosed as EES. 13 females and only one male were having symptoms of BIH. 17 patients (70.83%) had headache as the first presentation. Second most common presentation in our study was visual in 14 patients (58.3%). Two patients (8.3%) had pituitary hypersecretion namely; growth and prolactin hormones. In those (58.3%) confirmed to have BIH Theco-peritoneal shunts were inserted. Incidental cases (29.17%) without symptoms were followed up. Conclusion Although (ESS) is a well-known radiological hallmark for BIH, in our study not all patients had BIH. Interestingly, pituitary hyperfunction may be the first presentation in some rare cases. Generally, natural history of that entity was benign. Frequent follow-up by neurosurgeons and increased awareness of associations are advised. We believe a more prospective large number cohort is important to outline the natural history.

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Empty Sella Syndrome with Typical Facies: A Case Report

JOURNAL FOR CLINICAL AND DIAGNOSTIC RESEARCH ◽

10.7860/jcdr/2021/49167.15072 ◽

2021 ◽

Author(s):

Megha Mukundan ◽

Prativa Sethi ◽

Prasan Kumar Panda

Keyword(s):

Empty Sella ◽

Total Testosterone ◽

Ataxic Gait ◽

Rare Presentation ◽

Empty Sella Syndrome ◽

History Of ◽

Loss Of Libido ◽

Magnetic Resonance Imaging Mri ◽

Work Up

Empty Sella syndrome with typical facies is a rare presentation of panhypopituitarism. Here, authors presented a case of 45-year- old male with a history of altered behaviour such as low mood, passivity, talkativeness with low-hoarse voice and spells of cries for three months. He also had headache, vomiting, abdominal pain, ataxic gait, and loss of libido. He had meningitis 10 years back since then he had multiple similar episodes. He had sparse thinned out hair, male pattern temporal balding, madarosis, brownish pigmentation of face and chest and dry coarse skin. Patient had hypotension but without hypoperfusion. Hormone levels including thyroid hormones, Follicle Stimulating Hormone (FSH), Luteinizing hormone (LH), total testosterone cortisol, and plasma Adrenocorticotropic Hormone (ACTH) were less than normal. Magnetic Resonance Imaging (MRI) brain suggested streak pituitary gland (empty sella). He was supplemented with required hormones. On follow-up, he improved significantly. Trio composite hypothyroidism, hypocortisolism, and hypogonadism have characteristic facies. This unique presentation of patient with blank look facies gives a hint of empty sella, thus leading the clinician to diagnose the disease through timely evaluation and work-up.

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A Case of Endoscopic Repair of Cerebrospinal Fluid Rhinorrhea Associated with Primary Empty Sella Syndrome

Korean Journal of Otorhinolaryngology - Head and Neck Surgery ◽

10.3342/kjorl-hns.2011.54.10.727 ◽

2011 ◽

Vol 54 (10) ◽

pp. 727

Author(s):

Jun Hyuk Chi ◽

Seok-Won Park ◽

Min Seok Han ◽

Eun Kyeong Lee

Keyword(s):

Cerebrospinal Fluid ◽

Empty Sella ◽

Endoscopic Repair ◽

Empty Sella Syndrome ◽

Cerebrospinal Fluid Rhinorrhea ◽

Primary Empty Sella

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Non‐traumatic cerebrospinal‐fluid rhinorrhoea in cases of primary empty‐sella syndrome

The Medical Journal of Australia ◽

10.5694/j.1326-5377.1989.tb136570.x ◽

1989 ◽

Vol 150 (8) ◽

pp. 458-461 ◽

Cited By ~ 7

Author(s):

Russ J. Scott ◽

Michael J. Redmond

Keyword(s):

Cerebrospinal Fluid ◽

Empty Sella ◽

Empty Sella Syndrome ◽

Cerebrospinal Fluid Rhinorrhoea ◽

Primary Empty Sella

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AYURVEDIC MANAGEMENT OF PARTIAL EMPTY SELLA SYNDROME: A CASE REPORT

International Journal of Research in Ayurveda and Pharmacy ◽

10.7897/2277-4343.120498 ◽

2021 ◽

Vol 12 (4) ◽

pp. 15-18

Author(s):

Eswara M P Sarma ◽

Arathi P K

Keyword(s):

Cerebrospinal Fluid ◽

Case Report ◽

Pituitary Gland ◽

Sella Turcica ◽

Marked Change ◽

Empty Sella ◽

Empty Sella Syndrome ◽

Lukewarm Water

Empty Sella Syndrome is a disorder that involves the presence of Cerebro-spinal fluid in Sella turcica in an excess amount. In empty Sella syndrome the Sella turcica is either partially filled with cerebrospinal fluid and an atrophied pituitary gland lying in the floor of the Sella (Partial Empty Sella Syndrome) or filled with cerebrospinal fluid which pushes the pituitary gland into a side of Sella turcica resulting in non-visualization of the gland. (Completely empty Sella). A 37-year-old female diagnosed with partial empty Sella syndrome was managed with Pathyakshadhatryadi kashayam 15 ml twice daily in empty stomach with 45 ml lukewarm water, Rasnadi choornam tailam application, mahatraiphala ghritam 10 ml at night after food, Kshirabala 7 Avarti tailam pratimarsha nasyam. After completion of the treatment, there was considerable relief in the primary complaint of the patient which was a recurrent headache and the follow up MRI showed a marked change in the condition. This case report highlights the role of Ayurveda in the management of partial empty Sella syndrome and sheds light for further study on the same.

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The primary empty sella syndrome. Analysis of the clinical characteristics, radiographic features, pituitary function and cerebrospinal fluid adenohypophysial hormone concentrations

The American Journal of Medicine ◽

10.1016/0002-9343(77)90441-7 ◽

1977 ◽

Vol 62 (4) ◽

pp. A100 ◽

Cited By ~ 1

Keyword(s):

Cerebrospinal Fluid ◽

Clinical Characteristics ◽

Empty Sella ◽

Pituitary Function ◽

Empty Sella Syndrome ◽

Radiographic Features ◽

Syndrome Analysis ◽

Primary Empty Sella

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Primary empty sella syndrome in a series of 142 patients

Journal of Neurosurgery ◽

10.3171/jns.2005.103.5.0831 ◽

2005 ◽

Vol 103 (5) ◽

pp. 831-836 ◽

Cited By ~ 36

Author(s):

Giulio Maira ◽

Carmelo Anile ◽

Annunziato Mangiola

Keyword(s):

Cerebrospinal Fluid ◽

Intracranial Hypertension ◽

Empty Sella ◽

Csf Leak ◽

Csf Shunt ◽

Csf Dynamics ◽

Empty Sella Syndrome ◽

Content Type ◽

Fine Print ◽

Primary Empty Sella

Object. The primary empty sella syndrome (ESS) represents a heterogeneous clinical picture characterized by endocrine disturbances and signs of intracranial hypertension. An increase in intracranial pressure (ICP) is proposed to be one of the involved pathogenetic factors. Methods. The series included 142 patients who were observed during a period of 20 years. All patients underwent an ICP and cerebrospinal fluid (CSF) dynamics evaluation through the use of a lumbar constant-rate infusion test. Impairment of ICP and CSF dynamics was observed in 109 patients (76.8%). In 35 of the 36 patients affected by severe intracranial hypertension without rhinorrhea, improvement in adverse neurological symptoms was achieved after implanting a CSF shunt. Visual function, already seriously compromised before surgery, remained severely altered in one patient. In the group of 34 patients affected by rhinorrhea, CSF leakage was controlled using different surgical treatments: CSF shunt placement in 16 cases, surgical repair of the sellar floor in three, and both procedures in the remaining 13. Two patients refused any surgical treatment. Conclusions. The role of increased ICP in the pathogenesis and perpetuation of primary ESS has been confirmed. Adverse neurological signs and a CSF leak are correlated with an actual increase in ICP and are relieved after CSF shunt insertion. Cerebrospinal fluid rhinorrhea is more common than generally thought. Its resolution can be achieved using a careful diagnostic protocol and sometimes may require different surgical procedures.

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Acromegaly in Primary Empty Sella

Journal of Medicine ◽

10.3329/jom.v10i2.2827 ◽

1970 ◽

Vol 10 (2) ◽

pp. 119-120

Author(s):

Farzana Shumy ◽

M Azizul Kahhar

Keyword(s):

Growth Hormone ◽

Cerebrospinal Fluid ◽

Fluid Pressure ◽

Empty Sella ◽

Cerebrospinal Fluid Pressure ◽

The Other ◽

Empty Sella Syndrome ◽

Active Acromegaly ◽

Pituitary Irradiation ◽

Primary Empty Sella

Acromegaly has occasionally been found in patients with an empty sella secondary to prior pituitary irradiation and/or surgery. Active acromegaly, on the other hand, has only rarely been found in patients with a primary empty sella. The two most commonly held hypotheses concern-ing the aetiology of the primary empty sella are hydrodynamic transmission of cerebrospinal fluid pressure through a congenital defect in the diaphragm sella, and infarc-tion of the pituitary contents. Pituitary masses may undergo clinically silent infarction with development of a partial or totally empty sella by cerebrospinal fluid filling the dural herniation. Here we describe a patient who has empty sella without any prior pituitary procedures. Keywords: Acromegaly, empty sella syndrome, growth hormone. doi: 10.3329/jom.v10i2.2827 J MEDICINE 2009; 10 : 119-120

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Follow-up MR of Intracranial Cavernomas the Relationship between Haemorrhagic Events and Morphology

Interventional Neuroradiology ◽

10.1177/159101999600200205 ◽

1996 ◽

Vol 2 (2) ◽

pp. 127-135 ◽

Cited By ~ 22

Author(s):

R. Willinsky ◽

W. Harper ◽

M.C. Wallace ◽

W. Kucharczyk ◽

W. Montanera ◽

...

Keyword(s):

Natural History ◽

Posterior Fossa ◽

Classification System ◽

Classification Scheme ◽

Mass Effect ◽

Blood Products ◽

History Of ◽

The Relationship ◽

Regressive Changes

The purpose of this review was to determine whether a classification scheme for cavernomas based on the MR appearance is predictive of the natural history of the lesions with particular reference to the risk of haemorrhage. We reviewed the clinical and MR findings in 156 patients with intracranial cavernomas. The dominant or symptomatic cavernoma was graded into three groups according to the appearance of the blood products. The presence of haemorrhage, mass effect and oedema was evaluated. Sixty patients had serial MRs with a mean follow-up of 1.9 years. Subacute haemorrhage with mass effect and oedema was evident on the initial MR in 30 patients (19%). In seven patients follow-up MR showed haemorrhage with mass effect and oedema. Six of these bleeds were in the posterior fossa. The haemorrhages seen on follow-up MR occurred in all three morphological types. Regression was found on follow-up MR in five of the seven patients who initially showed a bleed. In 35% of patients, the dominant or symptomatic cavernoma changed on serial MR. Intracranial cavernomas are dynamic lesions with both active and regressive changes seen on follow-up MR. A classification system based on morphology was not found to be useful in predicting future bleeds. Bleeding rates, based on MR follow-up, were 10.9% in the posterior fossa and 1.7% in the supratentorial compartment.

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The primary empty Sella: An endocrine study on 36 cases

World Journal of Advanced Research and Reviews ◽

10.30574/wjarr.2021.11.2.0371 ◽

2021 ◽

Vol 11 (2) ◽

pp. 124-129

Author(s):

Marwa Chiboub ◽

Fatma Mnif ◽

Boubaker Ben Miloud ◽

Dhouha Ben Salah ◽

Nabila Rekik ◽

...

Keyword(s):

Post Partum ◽

Sella Turcica ◽

Empty Sella ◽

Pituitary Insufficiency ◽

Multiparous Women ◽

Anterior Pituitary Function ◽

History Of ◽

Visual Disturbances ◽

Primary Empty Sella

Introduction: The term primary empty sella (PES) makes reference to the herniation of the subarachnoid space within the sella turcica in patients with no history of pituitary tumor, surgery or radiotherapy. Materials and Methods: We studied 36 patients; 27 females and 9 males. The mean age at diagnosis was 50, 4 years [21-80 years]. Most diagnoses were made by magnetic resonance imaging (n = 32). The anterior pituitary function was evaluated by basal hormonal measurements. Then, we had compared two groups of patients: G1(n=17), who had a pituitary disorder, and G2 (n=10), patients without hormonal disease, in order to determine risk factors for endocrine dysregulation in PES. Results: The reasons for ordering pituitary scans were: headaches (65%), visual disturbances (32%) and neurological symptoms in 8% of cases. Sixty-six point five per cent of women were multiparous. Diabetes, obesity and hypertension were found in 19.45 %, 11 % and 19.45 % of the studied population respectively. Hyperprolactinemia was present in 17.24 % of patients. Fifty-five percent of our patients had some degree of hypopituitarism. We didn’t find any correlation in our study, between pituitary insufficiency and age, gender, weight, parity nor post-partum hemorrhage. Failure of lactation was significantly associated with a hormonal disorder. On the other hand, headache was negatively correlated with hormonal effects in PES. Conclusion: PES was most commonly found in middle-aged multiparous women. In most patients, PES is a heterogeneous condition that ranges from hypopituitarism to various degrees of isolated GH deficiency, and which needs careful endocrine assessment, treatment and follow-up.

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