Search

Background: Chiari I malformations (CM-I) and Primary Empty Sella syndrome (PESS) are rare disorders of structural defects within a skull. Headache is the most common presenting symptom in both conditions. However, CM-I and ESS comorbidity in patients with severe headaches is yet to be reported. Case description: The patient was a 38-year-old woman with a one-year history of headaches at various localisations (occipital and bilateral frontal) preceded by mood agitation. Physical examination was significant for obesity. Magnetic resonance imaging (MRI) revealed a 6.4 mm cerebellar tonsil descent through the foramen magnum and hypointense (CSF-filled) flattened sella. The lipid panel showed elevated cholesterols (total) and low-density lipoprotein (LDL) levels. She was medically managed, with no report of symptoms relapses at one-month and two-month followups. Conclusion: This is the first report on comorbid CM-I and PESS in adults presenting with cephalalgia. PESS and CM-I co-occurrence may present with mixed headache localisation; careful history-taking and imaging are mandatory for diagnosis confirmation. Future extensive studies are warranted to analyse the pathophysiological interplay between these two rare disorders. [ABSTRACT FROM AUTHOR]

In the article we present a case report of a young man who was undergoing investigation for infertility. Spermogram results showed azoospermia. Only a complex sexological, urological and genetic examination found the cause of the couple's infertility, namely a structural aberration of the Y chromosome isochromosome i(Y)(p10), duplication of the short arm of the Y chromosome and deletion of the entire long arm of the Y chromosome (hence the entire AZF gene region). This finding correlated with the clinical findings of azoospermia. Isochromosome Yp (ORPHA:98797) is a rare gonosomal aberration characterised by variable clinical manifestations including: normal healthy fertile men, men with infertility and men with ambiguous genitalia and incomplete masculinisation. After the discovery of this chromosomal aberration, the couple decided to undergo assisted reproduction using donor sperm. [ABSTRACT FROM AUTHOR]

We reported a case of a 68-year-old man who presented with recurrent fever and multiorgan dysfunction. His significantly elevated procalcitonin and C-reactive protein levels indicated recurrent sepsis. However, no focus of infection and no pathogens were identified through a variety of examinations and tests. Although the increase of creatine kinase was less than five times the upper limit of normal value, the diagnosis of rhabdomyolysis secondary to adrenal insufficiency resulting from primary empty sella syndrome was finally made, as supported by serum myoglobin elevation, serum cortisol, and adrenocorticotropic hormone deficiency, bilateral adrenal atrophy on computed tomography, and empty sella on magnetic resonance imaging. After the glucocorticoid replacement treatment, the patient's myoglobin gradually returned to normal range, and his condition continued to improve. Rhabdomyolysis resulting from a rare cause may be misdiagnosed as sepsis in patients who present with increased procalcitonin levels.

Background: Empty sella syndrome is a rare disorder characterized by enlargement or malformation of a structure in the skull known as the sella turcica. Most individuals with empty sella syndrome do not have any associated symptoms, but the finding raises concerns about hormone deficiencies. Empty sella syndrome may occur as a primary disorder (idiopathic) or as a secondary disorder in which it occurs due to an underlying condition or disorder such as a treated pituitary tumor, head trauma, or a condition known as idiopathic intracranial hypertension (also called pseudotumor cerebri) during which elevated intracranial pressure causes empty sella syndrome. Here we present a case of 25 year married female having symptoms and signs of raised ICP and decreased secondary sexual characters with blood investigations showing hypothyroidism. Her MRI Brain showed features of raised intracranial pressure (ICP) suggestive of Idiopathic Intracranial Hypertension with partially empty sella. Patient was diagnosed as Idiopathic Intracranial Hypertension with partial empty Sella with hypothyroidism and hypogonadotrophic normogonadism secondary to raised ICP. Patient was treated with IV Mannitol, thyroxine was started according to weight and other supportive treatment. Patient conciousness improved and got better. Patient was discharged with follow up on oral acetazolamide, thyroxine and hormone replacement therapy. [ABSTRACT FROM AUTHOR]

Abstract Background Empty sella syndrome is characterized by a constellation of symptoms that encompass various systems, and includes endocrine, neurologic, ophthalmologic, and psychiatric presentations. We here report a case of a young man presenting with severe hyponatremia due to empty sella syndrome and focus on changes in electrolytes during corticosteroid supplementation. Case report A 36-year-old man presented with general weakness, poor appetite, and dizziness for 4 days. Physical assessment revealed lower limbs nonpitting oedema. Pertinent laboratory data showed severe hyponatremia (sodium 108 mmol/L). Endocrine work-up revealed low cortisol levels at 1.17 µg/dL (reference: 4.82–19.5 µg/dL) and inappropriately normal adrenocorticotropic hormone levels at 12.4 pg/mL (reference: 0.1–46.0 pg/mL), indicating secondary adrenal insufficiency. Brain magnetic resonance imaging confirmed the diagnosis of empty sella syndrome. He developed delirium and agitation one day after cortisol supplementation with a sodium correction rate of 10 mmol/L/day, while hypokalaemia (potassium 3.4 mmol/L) also developed. The symptoms improved after lowering the serum sodium level. This patient was eventually discharged after 12 days of hospitalization when the serum sodium and potassium levels were 139 mmol/L and 3.5 mmol/L, respectively. Conclusion Herein, we address the importance of timely diagnosis of empty sella syndrome in patients with hyponatremia and highlight the close monitoring of the changes in electrolytes during corticosteroid replacement.

• Rhabdomyolysis can present with a sepsis-like manifestation. • Rhabdomyolysis may be a result of a rare cause - empty sella syndrome. • Elevated procalcitonin levels may occur under noninfectious inflammatory conditions. We reported a case of a 68-year-old man who presented with recurrent fever and multiorgan dysfunction. His significantly elevated procalcitonin and C-reactive protein levels indicated recurrent sepsis. However, no focus of infection and no pathogens were identified through a variety of examinations and tests. Although the increase of creatine kinase was less than five times the upper limit of normal value, the diagnosis of rhabdomyolysis secondary to adrenal insufficiency resulting from primary empty sella syndrome was finally made, as supported by serum myoglobin elevation, serum cortisol, and adrenocorticotropic hormone deficiency, bilateral adrenal atrophy on computed tomography, and empty sella on magnetic resonance imaging. After the glucocorticoid replacement treatment, the patient's myoglobin gradually returned to normal range, and his condition continued to improve. Rhabdomyolysis resulting from a rare cause may be misdiagnosed as sepsis in patients who present with increased procalcitonin levels. [ABSTRACT FROM AUTHOR]

A 64-year-old woman was transported suffering from persistent lower abdominal pain, vomiting, and low-grade fever. Magnetic resonance imaging revealed an empty sella (ES) and hormone tests revealed a disappearance of diurnal variation of cortisol, low cortisol and adrenocorticotropic hormone (ACTH) secretion especially in the morning, and poor ACTHcortisol axis reaction, as well as normal hypothalamus-pituitary gland-thyroid or adrenal gland axis hormone reaction. The cause of ES remained unclear; however, based on a diagnosis as adrenal insufficiency due to inappropriate ACTH secretion caused by total primary ES syndrome, we started hydrocortisone (15 mg/day). Afterwards, she immediately became symptom-free and was discharged. [ABSTRACT FROM AUTHOR]

Introduction: The term empty sella refers to a shrunken or displaced (by a subarachnoid diverticulum) pituitary gland. It can be primary (genetically determined) or secondary (due to trauma/surgery/radiation). It has been reported that 50% of patients are asymptomatic, and others experience symptoms, such as headache, hypertension, or visual field defects. Few cases have an empty sella syndrome, i.e., lacking functional pituitary hormones. Diagnosis is made through NMR or CT. If asymptomatic, this condition requires no treatment; otherwise, empty sella syndrome needs hormonal replacement therapy. We examined the case of asymptomatic empty sella syndrome., Case Report: A 67-year-old female patient was admitted for dilatative cardiomyopathy. She had a past medical history of arterial hypertension and right ICA endovascular repair. Blood tests demonstrated hypothyroidism, hypoadrenalism, and GH deficiency, without any signs or symptoms. NRM confirmed an empty sella, hence replacement therapy with levothyroxine and cortisone acetate was started. During a follow-up evaluation, we discovered that this biochemical profile of the patient had been known for more than a decade and never treated. Despite being exposed to stress conditions, vascular surgery and angiography, she never developed an adrenal crisis, nor has she ever been symptomatic for severe hypothyroidism. Hormonal replacement therapy was performed., Conclusion: The described clinical scenario is rare, as usually, empty sella syndrome presents with signs of hormone deficiency, even if asymptomatic cases have been described. Some authors suggest considering it as a hypothalamic dysfunction requiring treatment; others identify it as a paraphysiological variant. However, more cases are needed to establish a correct therapeutic strategy for these patients., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

The symptoms associated with empty sella syndrome (ESS) include headache, giddiness, vomiting, visual field deficits, and endocrine problems, as well as the radiological appearance of an enlarged sella turcica. This case report highlights a 45-year-old female who had a COVID-19 infection 2 months back and presented with chronic headache, giddiness, and lethargy having persistent hyponatremia later diagnosed as empty sella syndrome on brain magnetic resonance imaging. In this case, we tried to correlate all of these clinical and radiological features as COVID-19 sequelae due to post-Covid hypothalamic-pituitary axis dysfunction. [ABSTRACT FROM AUTHOR]

Spontaneous resolution of nonfunctioning pituitary adenoma after hemorrhagic apoplexy is a rare clinical entity of unknown etiology and is defined as disappearance of a tumor without any specific treatment. Here we present a 54-year-old male patient who presented with acute onset of severe headache, vomiting, photophobia, and sonophobia. He was referred to brain computed tomography, which showed a 16x12x16 mm tumor mass located in the sellar region with signs of hemorrhage. Endocrinologic evaluation was consistent with under-function of pituitary gonadotropic cells. Magnetic resonance imaging (MRI) performed ten days later was consistent with hemorrhagic apoplexy of the pituitary adenoma. The patient’s symptoms resolved after conservative treatment with dexamethasone, but he was scheduled for elective pituitary surgery. Preoperative MRI was performed one month after the first one and disclosed normal pituitary gland without any signs of adenoma. Our case is remarkable due to the fact that spontaneous remission of pituitary adenoma occurred within the first month, which is the shortest interval reported to date. Our case highlights the importance of conservative therapy as the first-line treatment for pituitary apoplexy in the absence of neurological impairment, since spontaneous remission may occur in a short time interval.

Empty sella is the neuroradiological or pathological finding of an empty sella turcica containing no pituitary tissue. Even though empty sella syndrome (ESS) and schizophrenia are both relatively common in the general population, to the best of our knowledge, there is only one similar case report regarding the co-occurrence of those two conditions

Background: Empty sella syndrome is characterized by a constellation of symptoms that encompass various systems, and includes endocrine, neurologic, ophthalmologic, and psychiatric presentations. We here report a case of a young man presenting with severe hyponatremia due to empty sella syndrome and focus on changes in electrolytes during corticosteroid supplementation.Case Report: A 36-year-old man presented with general weakness, poor appetite, and dizziness for 4 days. Physical assessment revealed lower limbs nonpitting oedema. Pertinent laboratory data showed severe hyponatremia (sodium 108 mmol/L). Endocrine work-up revealed low cortisol levels at 1.17 µg/dL (reference: 4.82-19.5 µg/dL) and inappropriately normal adrenocorticotropic hormone levels at 12.4 pg/mL (reference: 0.1-46.0 pg/mL), indicating secondary adrenal insufficiency. Brain magnetic resonance imaging confirmed the diagnosis of empty sella syndrome. He developed delirium and agitation one day after cortisol supplementation with a sodium correction rate of 10 mmol/L/day, while hypokalaemia (potassium 3.4 mmol/L) also developed. The symptoms improved after lowering the serum sodium level. This patient was eventually discharged after 12 days of hospitalization when the serum sodium and potassium levels were 139 mmol/L and 3.5 mmol/L, respectively.Conclusion: Herein, we address the importance of timely diagnosis of empty sella syndrome in patients with hyponatremia and highlight the close monitoring of the changes in electrolytes during corticosteroid replacement. [ABSTRACT FROM AUTHOR]

Background. Acromegaly is an acquired disorder related to excessive production of growth hormone (GH) and insulin-like growth factor-1 (IGF-1). Empty sella (ES) is an anatomical condition of sella turcica that is partially or completely filled with cerebrospinal fluid mainly due to intrasellar herniation of subarachnoid space. Here, we describe a patient who presented with clinical and biochemical features of acromegaly and who had an ES on pituitary magnetic resonance imaging (MRI). Case report. A 73-year-old male patient was consulted in our clinic because of the acromegalic phenotype while planning for colorectal adenocarcinoma surgery. The patient noticed gradual enlarging of his hands, feet and nose for 30 years, but never consulted to any clinician for this reason. Serum GH was 20.6 ng/mL (normal <3 ng/mL) and IGF-1 was 531 ng/mL (normal, 69--200 ng/ml). An oral glucose tolerance test showed no suppression of GH values. T1-weighted MRI revealed an ES. 18F-FDG PET/CT and Ga-DOTATADE PET/CT did not show any finding consistent with ectopic GH secretion. Growth hormone releasing hormone (GHRH) was within the normal range (<100mg/dL). He was treated with long-acting octreotide 20 mg per 28 days. At the 6th month of treatment, serum GH and IGF-1 levels were decreased to 5.45 ng/mL and 274 ng/mL, respectively. Conclusion. The mechanism underlying the association of acromegaly and ES remains unclear. Apoplexy on existing pituitary adenoma and then formation of necrosis can proceed to ES. Since our patient did not have a history of pituitary apoplexy and we could not find any reason for secondary ES, we considered primary ES. [ABSTRACT FROM AUTHOR]

The sella turcica is the normal neuroanatomical location of the pituitary gland. Empty sella syndrome (ESS) is a rare condition in which the sella turcica is partially or completely filled with cerebrospinal fluid. It is a radiological diagnosis that is often made incidentally following imaging for another reason (eg, to exclude intracranial hemorrhage following head injury) or as part of the work-up when investigating a patient's neurological symptoms. ESS can be classified as primary or secondary, depending on the identification of underlying etiologies. We report the highly unusual case of a 74-year-old patient who presented with progressive neurological disturbance many years after receiving intrathecal chemotherapy. Clinical assessment (including cross-sectional imaging) led to a diagnosis of secondary ESS.

ObjectiveTo describe the work-up and treatment of rare ectopic acromegaly caused by a biopsy-proven somatotroph pituitary adenoma located within the bony intersphenoid septum of a patient with empty sella syndrome (ESS).MethodsWe report the presentation, clinical course, diagnostic work-up, and lesion localization and treatment challenges encountered in a 55-year-old patient, with a brief review of relevant literature.ResultsA 55-year-old African-American man presented with acromegaly and ESS. Attempts to definitively localize the causative tumor were unsuccessful, though petrosal sinus sampling supported central growth hormone production and imaging suggested bone-enclosed subsellar pituitary tissue. Endoscopic endonasal transphenoidal exploration was undertaken with resection of a somatotroph pituitary microadenoma, and subsequent clinical improvement and biochemical remission. Retrospective review revealed the patient's pituitary to have been located ectopically within a unique bony intersphenoid septum.ConclusionThis report describes the first known case of an ectopic pituitary adenoma located within the midline bony intersphenoid septum, which we postulate to have resulted from anomalous embryological pituitary migration. Intra-intersphenoid septal tumors should be considered in cases of apparent central acromegaly with ESS or absence of tumor tissue within the paranasal sinuses or other peripheral locations.IndexingAcromegaly, ESS, pituitary adenoma, sphenoid sinus septum.

Abstract Background Primary Empty Sella (PES) syndrome is an increasingly common disorder, mostly diagnosed as an incidental finding during brain imaging scans. We intended to review the clinical management and hormonal profile of patients with PES. Methods The study included ten-year retrospective analysis of registry containing PES cases in the period 2007 to 2017, from a single tertiary care center. The keyword ‘primary empty sella’ was used to retrieve patient details from the radiology unit. The clinical and biochemical profile of PES patients was analyzed. Case management of PES patients and their rate of referral to endocrinologists was explored. Results The registry had 765 cases with a male: female ratio of 1:3.8 suggesting female predominance by almost four times. Although not significant, the onset of disease was earlier for males [Mean ± standard deviation (SD) (46.7 years ±17.3 vs 48.8 years±14.1), p = 0.110]. Almost 79% of the cases were found as an incidental finding during Magnetic Resonance Imaging. Of the total PES cases, only 20% were referred to the endocrinologists and the rest were handled by general physicians. Only 1–2.5% of the cases were evaluated for gonadal, growth and adrenal hormones by the general physicians. The hormonal evaluation by the endocrinologists was also found to be sub-optimal. Headache and visual disturbances were the most common presenting complaints followed by menstrual abnormalities. Endocrine abnormalities like thyroid dysfunction, hyperprolactinemia, hypogonadism and hypocortisolism were highly prevalent among those assessed. Conclusion There is a gross under-evaluation of hormonal assessment and minimal case-referral to Endocrinologists. PES is associated with varying degrees of hormonal dysfunction, and hence early assessment and management is needed. Establishing a standard protocol for diagnosis and case management is essential with the involvement of a multidisciplinary team consisting of endocrinologists, neurologists, primary care phys icians and ophthalmologists.

Background: The empty sella syndrome (ESS) is a neurological or pathologic finding in which sella turcica is devoid of pituitary tissue and the subarachnoid space extends into sella turcica, which is either primary or secondary as well as partial and complete. The widespread use of CT scans and MRIs today has made the ESS a common finding in imaging. The aim of this study was to evaluate the prevalence of the empty sella syndrome. Methods: This is a retrospective descriptive-analytic study in which all patients referred to Imam Ali Hospital (Zahedan) for electromagnetic brain imaging (n=1856) were recruited by cross-sectional sampling during the first 6 months from 21 March 2018 to 23 September 2018. Inclusion criteria included the absence of another known problem in the central nervous system and the absence of concurrent underlying disease. The data gathering tool was a questionnaire consisting of demographic and related variable to empty sella disorder. Results: The results of this study showed that the prevalence of empty sella was 8.2% with a mean age of 37.02±12.51 years. 66.4% of the patients were female. The prevalence of primary empty sella was 78.9% with a mean age of 34.51±11.26 years. 71.7% of the patients had partial empty sella. There was a significant difference between the mean age and sex of patients with empty sella and non-empty sella subjects (P=0.008) and (P

Hypothyroidism is a common pathological condition of thyroid hormone deficiency that varies widely in relation to both the acuity with which the deficiency develops and the clinical manifestations of the disease. While hypothyroidism is commonly mild or even subclinical, one rare complication of hypothyroidism that can cause multiorgan dysfunction is myxedema coma. Myxedema coma is precipitated by an acute stressor such as infection, surgery, or trauma, which disrupts the otherwise compensatory mechanisms present in severe hypothyroidism. Swift diagnosis and treatment of the condition is vital to improving patient outcomes as mortality from myxedema coma ranges from 40 to 60%. The mainstay of treatment includes IV levothyroxine (LT4) with or without the use of liothyronine (LT3). Here we present a case of a patient who presented with presyncope in the setting of generalized fatigue, poor appetite, anhedonia, and slowed speech and movement. Full workup revealed hypotension, hyponatremia, hypoglycemia, respiratory acidosis, undetectable cortisol, free T4, total T4 and an inappropriately normal TSH level. A diagnosis of myxedema coma and adrenal crisis due to pituitary dysfunction was made. A combination of 300 mcg IV LT4 and 5 mcg IV LT3 every 8 hours was used with successful resolution of the patient's symptoms. The patient is currently well maintained on a combination of oral LT4 and hydrocortisone replacement with no further endocrinological complications. This case underscores the need for physicians to maintain a high index of suspicion for secondary adrenal insufficiency and central hypothyroidism in patients with compatible symptoms or risk factors. Furthermore, it also highlights the successful treatment of adrenal crisis and myxedema coma with severe cardiogenic shock with a combination of hydrocortisone, levothyroxine, and liothyronine therapy.

Pembrolizumab, an anti-programmed death-1 specific monoclonal antibody is a second-line treatment for metastatic urothelial carcinoma. Physicians should be aware of adverse immune-related events associated with the use of immune checkpoint inhibitors, particularly adrenocortical insufficiency, which poses a risk of death.We report a case of secondary adrenocortical insufficiency due to isolated adrenocorticotropic hormone deficiency with empty sella syndrome after pembrolizumab treatment in a patient with metastatic renal pelvic cancer. Fortunately, a therapeutic effect was observed 4 months after discontinuation of pembrolizumab, and a durable antitumor response has persisted for 5 months.

Objective: Incidental radiographic findings of an empty sella are prevalent in up to 35% of the general population. While empty sella was initially considered clinically insignificant, a subset of patients exhibits endocrine or neuro-ophthalmologic manifestations which are diagnostic of empty sella syndrome (ESS). Recent studies suggest that more patients are affected by ESS than previously recognized, necessitating a deeper understanding of this condition. This comprehensive review describes a practical approach to evaluating and managing ESS., Methods: Literature review was conducted on etiologies and risk factors associated with primary and secondary empty sella, the radiologic features that differentiate empty sella from other sellar lesions, and the role of clinical history and hormone testing in identifying patients with ESS, as well as treatment modalities., Results: Pituitary function testing for somatotroph, lactotroph, gonadotroph, corticotroph, and thyrotroph abnormalities is necessary when suspecting ESS. While an isolated empty sella finding does not require treatment, ESS may require pharmacologic or surgical interventions to address hormone deficits or intracranial hypertension. Targeted hormone replacement as directed by the endocrinologist should align with guidelines and patient-specific needs. Treatment may involve a multidisciplinary collaboration with neurology, neurosurgery, or ophthalmology to address patient symptoms., Conclusion: This review underscores the evolving understanding of ESS, stressing the significance of accurate diagnosis and tailored management to mitigate potential neurologic and endocrine complications in affected individuals., Competing Interests: Disclosure The authors declare the following financial interests/personal relationships which may be considered as potential competing interests., (Copyright © 2024 AACE. Published by Elsevier Inc. All rights reserved.)

Background: This two-patient case series describes a rare sequela of postoperative empty sella syndrome (ESS) following transsphenoidal resection of pituitary macroadenomas. This is characterized by progressive hormone dysfunction, diabetes insipidus (DI), and associated MRI evidence of pituitary stalk disruption. Case Description: This phenomenon was retrospectively evaluated in a review of 2000 pituitary tumor resections performed by a single neurosurgeon (KOL). Chart review was retrospectively conducted to gather data on demographics, pituitary hormone status, tumor characteristics, and management. We identified 2 (0.1%) cases of progressive pituitary endocrine dysfunction occurring in the postoperative period associated with MRI evidence of pituitary stalk disruption within 6 weeks of discharge from the hospital. This was felt to be caused by the rapid descent of the residual normal pituitary gland down to the floor of the postoperative empty sella, causing relatively swift stalk stretching. Both patients developed DI, and one patient demonstrated increased pituitary hormone dysfunction. Conclusion: This phenomenon is a rare manifestation of postoperative ESS, secondary to surgical resection of a pituitary macroadenoma. We discuss the associated potential risk factors and strategies for avoidance in these two cases. Routine instillation of intrasellar fat in patients at risk is felt to be protective. [ABSTRACT FROM AUTHOR]

This case is about a 68 years old patient presented with worsening generalized weakness, dizziness, low mood and reduced appetite with the background history of gout and vitamin D deficiency. On general examination, the patient appeared very lethargic. However, the systemic examination was unremarkable. The vitals check showed a low blood pressure of 104/72 mm Hg. The investigations showed low levels of serum Sodium, Thyroid stimulating hormone, Cortisol. The short Synacthen test was abnormal. The anterior pituitary hormones level showed low levels of Follicle stimulating hormone, Luteinizing hormone and prolactin. CT head with contrast showed enlarged fluid filled pituitary fossa with fat. An MRI Pituitary gland showed an empty sella syndrome. The patient was hydrated initially and electrolytes were monitored regularly. Treated with hydrocortisone and thyroxine to which the patient responded and improved significantly. A follow-up appointment was arranged at endocrine clinic

This case is about a 68 years old patient who presented with worsening generalized weakness, dizziness, low mood, and reduced appetite with a background history of gout and vitamin D deficiency. On general examination, the patient appeared very lethargic. However, the systemic examination was unremarkable. The vitals check showed a low blood pressure of 104/72 mm Hg. The investigations showed low levels of serum Sodium, Thyroid-stimulating hormone, Cortisol. The short Synacthen test was abnormal. The anterior pituitary hormone level showed low levels of Follicle-stimulating hormone, Luteinizing hormone, and prolactin. CT head with contrast showed enlarged fluid-filled pituitary fossa with fat. An MRI Pituitary gland showed an empty sella syndrome. The patient was hydrated initially and electrolytes were monitored regularly. Treated with hydrocortisone and thyroxine to which the patient responded and improved significantly. A follow-up appointment was arranged at the endocrine clinic. [ABSTRACT FROM AUTHOR]

SummarySpontaneous lateral sphenoid cephaloceles arise from bony defects in the lateral sphenoid, in the absence of predisposing factors such as trauma, surgery, mass, or congenital skull base malformation. We reviewed CT and MR imaging findings and clinical data of 26 patients with spontaneous lateral sphenoid cephaloceles to better understand anatomic contributions to pathogenesis, varying clinical and imaging manifestations, and descriptive terminology. Two types of spontaneous lateral sphenoid cephaloceles were identified. In 15 of 26 patients, a type 1 spontaneous lateral sphenoid cephalocele was noted, herniating into a pneumatized lateral recess of the sphenoid sinus, and typically presenting with CSF leak and/or headache. In 11 of 26 patients, a type 2 spontaneous lateral sphenoid cephalocele was noted, isolated to the greater sphenoid wing without extension into the sphenoid sinus, presenting with seizures, headaches, meningitis, cranial neuropathy, or detected incidentally. All patients had sphenoid arachnoid pits, and 61% of patients had an empty or partially empty sella, suggesting that altered CSF dynamics may play a role in their genesis.

Background: Empty sella is an imaging finding previously thought to be of no clinical consequence in our environment. It has been classified into primary empty sella which is related to increased intracranial pressure and secondary empty sella which is considered a sequel of previous pituitary lesions. Studies have documented various clinical manifestations which we are beginning to see in our environment. Methods: This is a retrospective study done over a three-year period between May 2016- May 2019 in a private radiodiagnostic center. The subjects presented for brain magnetic resonance imaging for diverse, chronic clinical symptoms such as chronic headaches, visual disturbances and galactorrhoea and empty sella was found on imaging. The essence of the imaging was to demonstrate or to rule out a structural cause for the clinical symptoms of the subjects. Results: Eight female subjects between the ages of 34-56 years with a mean age of 45.0 ± 11.0 years presented with chronic symptoms. The most common symptoms were headaches (n = 8, 100%), visual disturbances (n = 3, 37.5%), chronic fatigue (n = 3, 37.5%), galactorrhea (n = 2, 25%), secondary infertility (n = 2, 25%), weight gain (n = 2, 25%) and psychotic symptoms (n = 2, 25%). Imaging finding showed empty sella in all subjects. One subject had features of chronic venous infarct. Conclusion: Empty sella syndrome presents in complex ways. Most of the symptoms can be explained by increased pressure on structures around the sella which control various organs in the body. In a resource poor setting like ours, empty sella should be considered in obese female subjects with chronic headaches.

Background: The empty sella syndrome (ESS) is a neurological or pathologic finding in which sella turcica is devoid of pituitary tissue and the subarachnoid space extends into sella turcica, which is either primary or secondary as well as partial and complete. The widespread use of CT scans and MRIs today has made the ESS a common finding in imaging. The aim of this study was to evaluate the prevalence of the empty sella syndrome. Methods: This is a retrospective descriptive-analytic study in which all patients referred to Imam Ali Hospital (Zahedan) for electromagnetic brain imaging (n=1856) were recruited by cross-sectional sampling during the first 6 months from 21 March 2018 to 23 September 2018. Inclusion criteria included the absence of another known problem in the central nervous system and the absence of concurrent underlying disease. The data gathering tool was a questionnaire consisting of demographic and related variable to empty sella disorder. Results: The results of this study showed that the prevalence of empty sella was 8.2% with a mean age of 37.02±12.51 years. 66.4% of the patients were female. The prevalence of primary empty sella was 78.9% with a mean age of 34.51±11.26 years. 71.7% of the patients had partial empty sella. There was a significant difference between the mean age and sex of patients with empty sella and non-empty sella subjects (P=0.008) and (P<0.0001). There was a statistically significant difference between the mean age of affected patients with type of empty sella (P<0.0001). There was no statistically significant difference between mean age of patients with empty sella and severity of empty sella (P=0.056). There was no significant difference between the frequency of empty sella type and the severity with gender (P=0.224) and (P=0.091). Conclusion: The findings of this study indicated that the overall prevalence of empty sella in the referring patients was relatively low. Most of them were females with primary type and minor severity. [ABSTRACT FROM AUTHOR]

Background: Empty sella is an imaging finding previously thought to be of no clinical consequence in our environment. It has been classified into primary empty sella which is related to increased intracranial pressure and secondary empty sella which is considered a sequel of previous pituitary lesions. Studies have documented various clinical manifestations which we are beginning to see in our environment. Methods: This is a retrospective study done over a three-year period between May 2016- May 2019 in a private radiodiagnostic center. The subjects presented for brain magnetic resonance imaging for diverse, chronic clinical symptoms such as chronic headaches, visual disturbances and galactorrhoea and empty sella was found on imaging. The essence of the imaging was to demonstrate or to rule out a structural cause for the clinical symptoms of the subjects. Results: Eight female subjects between the ages of 34-56 years with a mean age of 45.0 ± 11.0 years presented with chronic symptoms. The most common symptoms were headaches (n = 8, 100%), visual disturbances (n = 3, 37.5%), chronic fatigue (n = 3, 37.5%), galactorrhea (n = 2, 25%), secondary infertility (n = 2, 25%), weight gain (n = 2, 25%) and psychotic symptoms (n = 2, 25%). Imaging finding showed empty sella in all subjects. One subject had features of chronic venous infarct. Conclusion: Empty sella syndrome presents in complex ways. Most of the symptoms can be explained by increased pressure on structures around the sella which control various organs in the body. In a resource poor setting like ours, empty sella should be considered in obese female subjects with chronic headaches. [ABSTRACT FROM AUTHOR]

Objective: Data regarding the etiology, clinical and biochemical patterns in hypopituitarism is scant for Pakistan. We describe the characteristics of patients with hypopituitarism other than sellar and parasellar tumors or traumatic brain injury from a tertiary care center in Pakistan. Methods: We conducted a retrospective descriptive study in the Aga Khan University Hospital, Karachi, Pakistan. We studied all patients presenting with hypopituitarism, between January 2004 and December 2013. Clinical, hormonal and imaging data pertinent to the study was collected according to inclusion criteria. Results: Forty-two patients presented to the endocrinology clinics at the Aga Khan University Hospital during the study period. Thirty-seven patients (88.1%) were females. Mean age ± standard deviation of the participants was 53.8 ± 14.7 years. Sixteen patients had secondary infertility and all were females; a majority of patients in this group had Sheehan’s syndrome (n=8) followed by empty sella syndrome (n=3), partial empty sella syndrome (n=2), idiopathic cause (n=2) and tuberculoma (n=1). Eighteen females (48.6%) reported inability to lactate. Conclusions: Non-traumatic hypopituitarism was more common in women, with Sheehan syndrome being the most common cause of hypopituitarism in our study (35.7%). Secondary hypothyroidism was the most common hormonal deficiency. The most commonly reported symptom was weakness. [ABSTRACT FROM AUTHOR]

Empty sella is an anatomic finding that is usually asymptomatic. However, when patients with empty sella finding present with visual deficits, surgical treatment may be necessary. The main goal of surgery is to elevate sellar content through a transsphenoidal approach. The aim of this study was to demonstrate a new technique for precise reconstruction of the sellar floor using a heterologous bone block to restore the anatomic elements of the sella turcica. Three patients with primary empty sella who presented with visual field defects were prospectively included. Surgery was performed through a microsurgical transsphenoidal approach and involved elevating the sellar content by using a tricortical heterologous bone graft, the dimensions of which were obtained considering the exact dimensions of each patient's sella turcica. The graft was premodeled outside the surgical bed and carefully introduced into the sella turcica to achieve the required elevation (chiasmapexy). Vision of all 3 patients improved, and the improvement persisted throughout the follow-up period. 2 patients, this improvement was noted immediately after surgery, and in the third patient, this change in vision occurred 1 week later. Long-term imaging studies showed the persistence of the bone graft in the sella turcica and the exact elevation of the sellar content at the end of the follow-up period. There were no complications in the present series. The technique presented here is simple and reproducible and allows an almost exact and persistent elevation of the sellar content. [ABSTRACT FROM AUTHOR]

Empty sella syndrome is a rare presentation and is usually diagnosed by imaging specifically done for assessing pituitary gland. It occurs due to herniation of subarachnoid space in to sellar space through a defect in diaphragm sellae. Some develop features of hypopituitarism gradually over a period of time. We report a case where an elderly presented with recurrent vomiting and hyponatremia, evaluation suggestive of hyponatremia, hypocortisolism, hypothyroidism and diagnosed to have Empty sella syndrome. [ABSTRACT FROM AUTHOR]

Background Visual field deterioration caused by secondary empty sella after cabergoline therapy for prolactinoma is a rare event. Chiasmapexy is performed to treat empty sella syndrome. Although various materials have been used for the elevation of the optic chasm, the most appropriate material remains to be established. Here, we describe the efficiency of chiasmapexy for empty sella syndrome following dopamine agonist treatment and the utility of septal cartilage and sphenoidal sinus bone as materials for chiasmapexy. Case Description A 35-year-old male with a history of cabergoline therapy for prolactinoma presented with visual deterioration. His magnetic resonance imaging revealed optic chiasm herniation into the empty sella. Endoscopic endonasal transsphenoidal chiasmapexy was performed using septal cartilage and sphenoidal sinus bone as materials for elevating the chiasm. Visual function improved immediately after operation. Conclusions Chiasmapexy is an effective surgical method for treating visual deterioration caused by empty sella after cabergoline treatment. Endoscopic endonasal chiasmapexy with septal cartilage and sphenoidal sinus bone is a considerable option because it is minimally invasive and involves decreased risk of infection. Highlights • Visual deterioration caused by empty sella after cabergoline therapy is rare. • Chiaspamexy is an effective surgical method for treating the condition. • The most appropriate material for chiasmal elevation remains to be established. • Septal cartilage and sphenoidal bone are considerable materials for chiasmapexy. [ABSTRACT FROM AUTHOR]

A 25-year-old Saudi female with a known case of autoimmune thyroiditis presented to the Emergency Room in stuporous condition. A blood test revealed a blood sugar level of 1.7 mmols/l (30.6 mg/dl). The patient was resuscitated with intravenous glucose. Further evaluations of the patient revealed celiac disease and idiopathic thrombocytopenia with preexisting autoimmune thyroiditis (polyglandular autoimmune syndrome III [PAS III]). The severe hypoglycemia, coupled with 6 years of infertility evaluation, revealed a rare association of empty sella syndrome with hypopituitarism {PAS II}.

Background: Empty sella syndrome (ESS) is a condition in which there is loss of volume of the pituitary gland, which is the normal constituent of the sella turcica. There may be visual and endocrine deficits associated with this condition, and radiologically, there may be downward prolapse of the optic chiasm. It occurs in a primary ESS, poorly understood form, as well as a secondary ESS form that follows medical or surgical treatment of a pituitary macroadenoma, or else spontaneous hemorrhage into such a tumor. Case Description: A 56-year-old man presenting with deficits of both visual acuity and visual fields in the setting of radiological ESS without associated optic chiasm prolapse is discussed. He underwent endoscopic endonasal chiasmapexy with gradual improvement of his visual function over the following 6 months. Conclusion: ESS is a potentially potent cause of visual deterioration that lends itself to reversal through a relatively simple neurosurgical technique. This case illustrates that actual prolapse of the chiasm is neither a prerequisite for visual deterioration nor its reversal the mechanism of visual improvement after chiasmapexy, raising the question of the mechanisms at play in cases such as this. It confirms the role of chiasmapexy in the management of selected cases of ESS. [ABSTRACT FROM AUTHOR]

Macroprolactinomas usually need long-term cabergoline therapy, often lifelong, as complete resolution of these tumors is rare. We present a vanishing pituitary macroprolactinoma which evolved into a complete empty sella following 2 years of cabergoline therapy. A 17-year-old girl with primary amenorrhea, chronic headache, and galactorrhea for 2 months was diagnosed to have pituitary macroprolactinoma (20 mm × 17 mm × 16 mm; prolactin 451 ng/ml). T2-weighted imaging revealed fluid level in adenoma suggestive of bleed in the tumor suggestive of pituitary apoplexy. Cabergoline was initiated at 0.5 mg/week and was uptitrated to 3 mg/week over 6 months to ensure prolactin was suppressed. Magnetic resonance imaging pituitary after 2 years of cabergoline therapy revealed empty sella with pituitary stalk traced till the floor of sella turcica, without any evidence of adenoma. Hormone evaluation revealed low total T4 levels (5.1 mcg/dl); low thyroid-stimulating hormone (0.9 mIU/L); normal morning cortisol (18.1 mcg/dl); and normal prolactin (4 ng/ml), leading to the diagnosis of secondary hypothyroidism. Cabergoline dose was tapered gradually. Levothyroxine replacement was started at 50 mcg/day. Pituitary apoplexy and associated infarction 2 years back lead to ischemic necrosis in adenoma, leading to its spontaneous resolution in our patient. The adenoma had damaged adjacent normal pituitary cells due to limited space in sella, resulting in empty sella. Pituitary apoplexy is nature's way of curing pituitary adenoma. Complete disappearance of adenoma and the resulting empty sella syndrome are the novelty of this report. [ABSTRACT FROM AUTHOR]

An 8-year-old male child presented to our pediatric outpatient department with complaint of his height being short compared to peers of his age group. Systemic examination was normal. On further investigation, he was found to have a rare cause of pathological short stature. We want to present this case because of its rarity and to throw light on availability of treatment for the same. [ABSTRACT FROM AUTHOR]