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BACKGROUND AND PURPOSE: Empty sella has been reported in patients with idiopathic intracranial hypertension and is thought to be a sign of elevation of intracranial pressure. However, it can also be found in patients with lateral sinus stenosis presenting with isolated pulsatile tinnitus without signs of intracranial hypertension. We hypothesized that the volume of the sella turcica would be similar in both groups of patients undergoing stent placement for lateral sinus stenosis. MATERIALS AND METHODS: Consecutive patients with idiopathic intracranial hypertension or isolated venous pulsatile tinnitus and undergoing lateral sinus stent placement from January 2012 to December 2017 were included. The primary outcome was the estimated volume of the sella turcica based on preoperative CTA measurements. The ratio of the pituitary gland height/sellar height was calculated on preoperative MR imaging. Sellar volumes were compared among the 3 groups: pulsatile tinnitus, idiopathic intracranial hypertension, and a control group, matched by age and sex. RESULTS: Eighty-eight patients underwent lateral sinus stent placement. The median age was 37 years, and 94% were women. No difference in age, sex, or body mass index was found among the groups. Patients undergoing venous stent placement had significantly higher sellar volumes than the control group (P < 0.001). There was no difference in the sellar volumes (P = .63) or gland/sellar height ratios (P = .25) between the pulsatile tinnitus and idiopathic intracranial hypertension groups. CONCLUSIONS: Empty sella is found in 2 differing groups of patients undergoing lateral sinus stent placement, suggesting that it is a radiologic sign of symptomatic hemodynamic lateral sinus stenosis rather than elevated intracranial pressure.

A 54 year old lady presented to our institute with a history of low grade fever for one week associated with occasional loose stools, vomiting and severe malaise. Initial evaluation revealed low serum sodium. An initial diagnosis of acute gastroenteritis with secondary hyponatremia was made. Work up for infective causes of gastroenteritis was however negative. ENT evaluation and review of drug history did not contribute towards a diagnosis. The patient's symptoms persisted and did not respond to symptomatic treatment. Ultrasound of abdomen revealed cholelithiasis with no evidence of cholecystitis. Further evaluation revealed hypotonic hyponatremia with normal levels of urinary sodium excretion. With other causes of hyponatremia ruled out, an endocrinopathy was suspected as the likely culprit. Follow up hormonal studies revealed hypopituitarism and MRI of brain revealed a partially empty sella. On reviewing the case, a past history of amenorrhea immediately after the birth of her third child (almost 20 years ago!) was elicited. Intractable vomiting is quite an unusual presentation for Sheehan's syndrome, but a thorough case history coupled with a high index of suspicion can contribute towards identifying the cause among a series of confounding clinical and radiological findings, as in our case.

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. doi: https://doi.org/10.12669/pjms.35.4.174 How to cite this:Malik S, Kiran Z, Rashid MO, Mawani M, Gulab A, Masood MQ, et al. Hypopituitarism other than sellar and parasellar tumors or traumatic brain injury assessed in a tertiary hospital. Pak J Med Sci. 2019;35(4):1149-1154. doi: https://doi.org/10.12669/pjms.35.4.174 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Актуальність. На даний час залишається актуальним пошук діагностично значущих маркерів агресивності неактивних аденом гіпофіза (НАГ) для визначення подальшого прогнозу в постопераційному періоді. Аденоми гіпофіза, що не мають клінічних проявів гіперсекреції гіпофізарних гормонів, є формами доброякісних пухлин аденогіпофіза. У загальній популяції частота НАГ становить 50 випадків на 1 млн населення. Мета дослідження: визначити клініко­діагностичні маркери агресивності пухлини у хворих із НАГ і дефіцитом гормона росту і їх роль у формуванні тяжкості нейроендокринної симптоматики захворювання. Матеріали та методи. Під наглядом перебувало 87 хворих (із них чоловіків — 44, жінок — 43) із верифікованим діагнозом НАГ після трансназальної гіпофізектомії; період спостереження — від одного до трьох років. Результати. При розподілі хворих на групи залежно від гістологічної характеристики НАГ переважали пацієнти з крупноклітинною хромофобною аденомою гіпофіза (77,5 %). НАГ у жінок супроводжувалася ожирінням, первинним і вторинним гіпотиреозом, вторинним гіпогонадизмом, синдромом персистуючої галактореї — аменореї (симптоматична й ідіопатична форми), нецукровим діабетом, синдромом порожнього турецького сідла та ін. У чоловіків НАГ супроводжувалася ожирінням, первинним і вторинним гіпотиреозом, вторинним гіпогонадизмом, нецукровим діабетом, гінекомастією. Розроблена шкала маркерів агресивності НАГ дозволила ідентифікувати фактори за трьома ступенями і розробити комплекс профілактичних заходів, покликаних унеможливлювати рецидив зростання пухлини. Висновки. Маркерами агресивності перебігу НАГ є: молодий вік пацієнта, перші симптоми маніфестації хвороби, великі розміри пухлини, асиметричність і деформація гіпофіза, ознаки інвазії пухлини в сусідні тканини/артерії/кавернозний синус, наявність дрібноклітинних і/або темноклітинних хромофобних аденом, пангіпопітуїтаризм., Актуальность. В настоящее время остается актуальным поиск диагностически значимых маркеров агрессивности неактивных аденом гипофиза (НАГ) для определения дальнейшего прогноза в постоперационном периоде. Аденомы гипофиза, не имеющие клинических проявлений гиперсекреции гипофизарных гормонов, являются формами доброкачественных опухолей аденогипофиза. В общей популяции частота НАГ составляет 50 случаев на 1 млн населения. Цель исследования: определить клинико-диагностические маркеры агрессивности опухоли у больных с НАГ и дефицитом гормона роста и их роль в формировании тяжести нейроэндокринной симптоматики заболевания. Материалы и методы. Под наблюдением находилось 87 больных (из них мужчин — 44, женщин — 43) с верифицированным диагнозом НАГ после трансназальной гипофизэктомии, период наблюдения — от одного до трех лет. Результаты. При распределении больных по группам в зависимости от гистологической характеристики НАГ преобладали пациенты с крупноклеточной хромофобной аденомой гипофиза (77,5 %). НАГ у женщин сопровождалась ожирением, первичным и вторичным гипотиреозом, вторичным гипогонадизмом, синдромом персистирующей галактореи — аменореи (симптоматическая и идиопатическая формы), несахарным диабетом, синдромом пустого турецкого седла и др. У мужчин НАГ сопровождалась ожирением, первичным и вторичным гипотиреозом, вторичным гипогонадизмом, несахарным диабетом, гинекомастией. Разработанная шкала маркеров агрессивности НАГ позволила идентифицировать факторы по трем степеням и разработать комплекс профилактических мер, предупреждающих рецидив роста опухоли. Выводы. Маркерами агрессивности течения НАГ являются: молодой возраст пациента, первые симптомы манифестации болезни, большие размеры опухоли, асимметричность и деформация гипофиза, признаки инвазии опухоли в соседние ткани/артерии/кавернозный синус, наличие мелкоклеточных и/или темноклеточных хромофобных аденом, пангипопитуитаризм., Background. Nowadays, a search for diagnostically significant markers of aggressiveness of non­functioning pituitary adenomas remains relevant for further prognosis in the postoperative period. Non­functioning pituitary adenomas without pituitary hypersecretion are the forms of adenohypophysis tumors. In the general population, frequency of non­functioning pituitary adenomas is 50 cases per 1 million people. The purpose of the study is to determine the clinical diagnostic markers of tumor aggressiveness in patients with non­functio­ning pituitary adenomas and growth hormone deficiency, as well as their role in the severity of the neuroendocrine symptoms of the disease. Materials and methods. Under our supervision, there were 87 patients (44 men, 43 women), of them 31 with a verified diagnosis of non­functioning pituitary adenomas after transnasal hypophysectomy. Follow­up was from 1 to 3 years. Results. When patients were distributed in groups depending on histological description of non­functioning pituitary adenomas, those with chromophobe pituitary adenoma prevailed (77.5 %). Non­functioning pituitary ade­nomas in women were associated with obesity, primary and secondary hypothyroidism, secondary hypogonadism, syndrome of persistent galactorrhea­amenorrhea (symptomatic and idiopathic forms), diabetes insipidus, empty sella syndrome and others. In men, non­functioning pituitary adenomas was accompanied by obesity, primary and secondary hypothyroidism, secondary hypogonadism, diabetes insipidus, gynecomastia. The scale developed to determine the markers of aggressiveness of non­functioning pituitary adenomas allowed identifying factors by 3 degrees and developing a set of measures to prevent the recurrence of tumor growth. Conclusions. Markers of aggressiveness of non­functioning pituitary adenomas are: young age of the patient, first symptoms of the disease, large tumor sizes, asymmetry and deformation of the pituitary gland, signs of tumor invasion into adjacent tissues/arteries/cavernous sinus, small cell and/or dark cell chromophobe adenomas, panhypopituitarism.

Acromegaly is a condition defined by excess growth hormone (GH). The clinical features of acromegaly including overgrowth of many tissues including cartilage, bone, skin and visceral organs. It often times presents with coarsening of facial features and increased hat or ring size. Cardiovascular disease including hypertension with left ventricular hypertrophy and metabolic deficiencies including insulin resistance are also commonly seen as a result of the GH excess. The most common cause of acromegaly has been found to be GH secreting pituitary adenomas. MRI is generally sufficient to identify these lesions, but rarely a patient with symptoms of acromegaly will be found to have empty sella syndrome. Case: A 37 year old male, with a PMH of uncontrolled HTN presented to the emergency department after his wife found him on the floor, unconscious, foaming at the mouth, and shaking. He had an episode of urinary incontinence and was postictal for 20-30 minutes after the episode. In ED, he was hypertensive at 246/162, CT head showed right frontal intraparenchymal 1.2 cm hemorrhage and MRI brain showed various foci of hyperintensity compatible with hypertensive encephalopathy. He was also hypokalemic (2.9) with evidence of an AKI (BUN 27, Cr 1.73). He was admitted for further evaluation of suspected seizure. Upon further evaluation, the patient admitted to coarsening of facial features as noted on comparison of prior photographs. He also reported difficulties with his wedding ring fitting which he attributed to hand swelling. ROS was other negative with no vision changes or headaches. This sparked concern for a secondary cause of hypertension in the setting of clinical acromegaly. MRI brain was complicated by movement but revealed pituitary characteristics of empty sella syndrome. Insulin like growth factor 1 - 745. ECHO showed severe concentric left ventricular hypertrophy with aortic valve sclerosis and left atrial dilation. Renal Artery ultrasound showed no evidence of renal artery stenosis. The patient was subsequently scheduled for repeat MRI brain pituitary as outpatient under anesthesia to evaluate for possible surgical treatment for acromegaly. Discussion: This case demonstrates that empty sella syndrome does not warrant exclusion of acromegaly. GH secreting tumors can be present despite empty sella syndrome and acromegaly can even cause enlargement of the sella (?) . In these cases, the pituitary tissue lining the sella can be the source of excess GH. Upon case review, the mechanism remains unclear but empty sella syndrome may result from necrosis by infarction or from hemorrhage. Since no resectable mass is present in these cases, medical management with somatostatin analogues remains the only treatment that can be offered to these patients. It is important to keep acromegaly on the differential despite empty sella.

Introduction; Empty Sella syndrome (ESS) is an uncommon finding on brain imaging whose functional significance remains controversial. While sometimes associated with pituitary hypofunction it is far less commonly associated with pituitary hyperfunction syndromes. We present two cases of this; one with acromegaly and one of hyperprolactinemia presumably due to prolactinoma both associated with ESS on brain imaging. Clinical Cases; A 48 yr old man was evaluated on account of reduced libido and chronic fatigue which revealed hypogonadotrophic hypogonadism. Further work up revealed prolactin levels in the 250-300ng/ml range. Brain MRI showed ESS and the rest of the pituitary hormone functional work up was normal. Bone density showed osteopenia and octreotide scan showed no sella enhancement nor other anomalies. When commenced on cabergoline 0.5mg 2 x wkly his prolactin levels normalized over 6 months and the hypogonadism resolved without requiring androgen replacement. A 49 yr old gentleman with long standing hypogonadotrophic hypogonadism on androgen replacement presented with persistent headaches and generalized arthralgias. He had clinical features suggestive of acromegaly including macrocephaly, macroglossia, facial dysmorphia and enlarged hands and feet. Diagnosis was confirmed by elevated IGF-1 (300-400ng/ml range), growth hormone levels and non suppressible growth hormone on Oral glucose tolerance test. No other pituitary functional deficits were found. Brain MRI showed ESS and octreotide scan showed no sella enhancement nor other anomalies. His IGF-1 levels normalized with octreotide LAR 20mg monthly. His androgen repletion also improved with lower testosterone dose requirement along with improvement in headaches and arthralgias though he developed type 2 diabetes. Results and Discussion; Review of our system's clinical records over the prior 5 years revealed 307 cases of ESS. 10 had hypopituitarism, 6 had hypogonadism, 12 had hyperprolactinemia presumed due to prolactinoma, 1 each had central hypothyroidism, acromegaly and diabetes insipidus. 276 (92%) of cases had normal pituitary functional status. Concluding remarks; ESS is an uncommon but important imaging finding on brain imaging. Though most patients with ESS have normal pituitary function it may be associated with pituitary hypofunction or even hyperfunction states. Its finding in pituitary hyperfunction states like hyperprolactinemia and acromegaly make medication therapy the only viable treatment option as surgery and radiotherapy have no visible target to direct therapy.

Background Pituitary apoplexy may occur when a large tumor compresses or outgrows its nutrient supply, resulting in ischemic necrosis and hemorrhage. Although once deemed a neurosurgical emergency, increasing evidence suggests that conservative management of pituitary apoplexy leads to favorable neuro-ophthalmologic and endocrinologic outcomes as well. Spontaneous remission after pituitary apoplexy has been described in functioning pituitary adenomas, but it is a rare occurrence in nonfunctioning tumors. Case Description We report a man that presented with pituitary apoplexy of a nonfunctioning pituitary macroadenoma that was managed conservatively and treated hormonally for hypopituitarism during a 2-year follow-up period, with serial neuroimaging demonstrating significant tumor volume reduction with almost complete resolution resulting in partial empty sella. In addition, a short literature review was performed pertaining to the management of pituitary apoplexy with emphasis on a more conservative approach. Conclusions A subset of patients with pituitary apoplexy without altered consciousness and nonprogressive or mild ophthalmologic deficits may be managed conservatively; however, lifelong periodic assessment, preferably by a specialized multidisciplinary pituitary team, is essential until clinical outcomes become clear.

Le syndrome de la selle vide est une affection dans laquelle la selle turcique est partiellement ou complètement remplie de liquide céphalorachidien (LCR), entraînant un déplacement de l'hypophyse. Nous rapportons le cas d'un patient obèse de 49 ans qui a présenté de façon progressive les céphalées, une asthénie physique et une hypothyroïdie et chez qui le scanner cérébral a été en faveur d'un syndrome de la selle turcique vide.

Background Sheehan syndrome refers to a series of clinical symptoms resulting from avascular necrosis of anterior pituitary due to various reasons. Case We report a case of multiple myeloma patient after one cycle of chemotherapy consisting of bortezomib and dexamethasone diagnosis of Sheehan syndrome with obstinate diarrhea, low blood glucose, and coma symptom, especially, the fluorodeoxyglucose positron emission tomography (FDG-PET) and brain magnetic resonance imaging (MRI) revealed that the manifestations in the saddle area were in accordance with empty sella syndrome. A single administration with lenalidomide was given for both consolidative and maintenance treatment, and satisfactory efficacy was achieved. With the patient now remaining in satisfactory medical condition, the success of this therapy has been shown. Conclusions This is the first report showing a patient with combined multiple myeloma and rarely seen Sheehan syndrome, in which lenalidomide was given for treatment, and satisfactory efficacy was achieved.

Background 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. Methods 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). Results 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. Conclusions The technique presented here is simple and reproducible and allows an almost exact and persistent elevation of the sellar content.

Sellar arachnoidocele is a term used to define the herniation of the subarachnoid space to the sella. 1 This is a rare radiologic finding that, in most cases, does not require treatment. 2 , 3 , 4 , 5 When symptoms appear, the term empty sella syndrome is used. Two varieties exist: primary and secondary empty sella syndrome. 2 The aim of this 3-dimensional operative video ( Video 1 ) is to demonstrate the extradural microsurgical remodeling of the sellar fossa with autologous bone in 2 cases of primary empty sella syndrome. Both patients signed an informed consent for the procedures and agree with the use of their images for research purposes. In both cases, magnetic resonance imaging scans showed herniation of the subarachnoid space into the pituitary fossa and an anchor-like silhouette on coronal view. Patients evolved favorably, improving their visual deficit after the surgery, as can be observed in the postoperative visual field study. If surgery is indicated due to visual loss, the procedure is known as chiasmapexy. Recently, Guinto et al 3 described a technique for chiasmapexy. Our team considers this procedure to be useful, technically simple, and low cost. Being autologous, rejection possibilities are almost null. This 3D video serves as a complement to illustrate the technique.

Purpose: It is not clear if Hashimoto’s thyroiditis leads to Empty sella syndrome. In the present study, we aimed to investigate if Hashimoto’s thyroiditis was associated with Empty sella syndrome, and Hashimoto’s thyroiditis was a secondary cause od some cases of primary Empty sella syndrome. Material and Method: Eighty-one patients who were diagnosed with primary Empty sella syndrome were included in the study. All patients underwent thyroid ultrasonography and biochemical tests for thyroid-stimulating hormone, free triiodothyronine, free thyroxine, anti thyroid peroxidase, anti thyroglobulin, follicle stimulating hormone, luteinizing hormone, 17 β estradiol, growth hormone, insulin-like growth factor 1, adrenocorticotropic hormone and total testosterone for Hashimoto’s thyroiditis and pituitary hormone deficiency. Results: Out of 81 patients, thyroid disease was diagnosed in 34 (42%) patients; 18 of them had Hashimoto’s thyroiditis (22.2%) and 16 (19.8%) had central hypothyroidism. Among Hashimoto’s thyroiditis patients, 11 (13.6%) had hypothyroidism and 7 (8.6%) were euthyroid. Discussion: In conclusion, it is possible that some cases of primary Empty sella syndrome are caused by Hashimoto’s thyroiditis. It is recommended that the presence of Hashimoto’s thyroiditis should be investigated in patients with primary Empty sella syndrome. Further studies investigating anti-pituitary antibody in patients with primary Empty sella syndrome, are needed to further declare this relationship.

Hyponatremia due to isolated adrenocorticotropic hormone (ACTH) deficiency is difficult to diagnose as it is usually indistinguishable from non-endocrine syndrome of inappropriate antidiuretic hormone secretion (SIADH). We present a case secondary to empty sella. Most patients with empty sella remain asymptomatic throughout life and require no treatment; however, in cases involving the development of isolated ACTH deficiency, corticosteroid treatment should be enforced to avoid fatal consequences.

Purpose of the study Familial occurrence of either Turner syndrome or hypopituitarism is very rare. Particularly, their association is an uncommon finding. In this context, we describe for the first time 4 sisters with Turner syndrome, hypopituitarism was reported in three among them. Patients and methods Our cohort consists of four Tunisian adult sisters belonging to a consanguineous family. Biochemical analysis, resonance magnetic imaging and cytogenetic analyses were performed. Results Turner syndrome was diagnosed at the ages of 14, 17, 31 and 43 years in cases 1, 2, 3 and 4 respectively. They suffered from short stature, dysmorphic syndrome and/or delayed puberty. Interestingly, 3 among them showed also hypopituitarism, hypogonadotrophic hypogonadism and central hypothyroidism. Somatotropic insufficiency was proven in one case. Pituitary MRI has shown an empty sella turcica with hypoplastic pituitary gland in three cases. Their karyotypes were compatible with 45X in one case, 45X/46XX in the second and 45X/46XX/47XXY with x label in two cases. Conclusion Hence, the presence of these familial cases of TS must evoke new etiopathogenetic arguments. Coincidence of hypopituitarism in this family, might suggest common genetic background for the two diseases. This particular family would be a precious tool for an extensive molecular analysis. More attention should be given to other family's members mainly in the presence of delayed puberty and sterility in other members.

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}.

Rationale: Bleeding in the anterior pituitary lobe leading to tissue necrosis occurs in the acute stage of severe clinical forms of hemorrhagic fever with renal syndrome (HFRS), while atrophy of the anterior pituitary lobe with diminution of the gland function occurs after the recovery stage. The relationship between Hantaan virus infection and empty Sella syndrome (ESS) has rarely been reported. Patient concerns: This patient was a 54-year-old previously healthy Chinese male. He presented with fever, headache, and backache with dizziness and oliguria. Physical examination was notable for hypotension and the signs of conjunctival suffusion. His platelets decreased, and his urine protein was positive. Hantaan virus IgM and virus RNA were positive. Diagnosis: He was diagnosed as having HFRS. In his diuretic phase, his 24-hour urine volume was maintained at 10,000 mL, and his blood pressure was higher for a week. Then, he was diagnosed as having ESS after a series of examinations. Interventions: Hormone replacement therapy was given to this patient after the diagnosis “ESS” was made. Outcomes: The patient's symptoms improved, and he was discharged from the hospital soon after hormone replacement therapy. Lessons: Pituitary function examination and brain magnetic resonance imaging (MRI) need to be considered to scan for ESS and panhypopituitarism in the patients with HFRS accompanied by diabetes insipidus.

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.

Tectal gliomas (TG) are slow-growing tumors and generally present with features of increased intracranial pressure. We describe an unusual case of a young girl who presented with clinical triad of obesity, primary amenorrhea and central cord syndrome. The radiology unveiled a pentad of hydrocephalus, empty sella, suprapineal recess diverticulum, secondary Chiari malformation and cervical syringomyelia, masking a relatively obscure tectal plate glioma. She was subjected to endoscopic third ventriculostomy (ETV). All of her symptoms improved after ETV and the tumor is being followed up. The possible pathogenesis of such a highly atypical clinico-radiological presentation is described. This unusual syndromic presentation of tectal glioma without clinical features of increased intracranial pressure is probably due to increased compliance of third ventricular walls, and is relieved with ETV.

Empty sella means the absence of the pituitary gland on cranial computed tomography or magnetic resonance imaging. Empty sella syndrome is the pathological variant of the imaging-described empty sella. We present the case of a male Caucasian child, aged four years and two months, for short stature and diagnosed by imaging procedures as empty sella. The cause of short stature was isolated growth hormone (GH) deficiency. Associated he presented left hand postaxial polydactyly. In connection with this particular case, we propose a review of current knowledge in empty sella syndrome. The particularity of reported case consists of association empty sella with GH deficiency and polydactyly. The association of empty sella with polydactyly is not reported yet in the medical literature and is probably coincidental.

Empty sella is the neuroradiological or pathological finding of an apparently empty sella turcica containing no pituitary tissue. The prevalence of primary empty sella, i.e., empty sella without any discernible cause, is not precisely known; estimates range from 2% to 20%. Technical advances in neuroradiology have made empty sella an increasingly common incidental finding. It remains unclear whether, and to what extent, asymptomatic adult patients with an incidentally discovered empty sella should undergo diagnostic testing for hormonal disturbances.To answer this question, the authors carried out a systematic search in the PubMed and Web of Science databases for publications that appeared in the period 1995-2016 and that contained the search term "empty sella" (registration: PROSPERO 2015: CRD42015024550).The search yielded 1282 hits. After the exclusion of duplicates, pediatric reports, case reports, and veterinary studies, 120 publications on primary empty sella syndrome (PES) were identified. 4 of these dealt with the prevalence of pituitary insufficiency in patients with PES as an incidental finding. Among patients with PES, the relative frequency of pituitary insufficiency in the pooled analysis was 52% (95% confidence interval [38; 65]).The data on PES as an incidental finding are too sparse to enable any evidence-based recommendation on the potential indications for hormone testing or its nature and extent. We advise basic neuroendocrinological testing (fasting cortisol, free thyroxine [fT4], estradiol or testosterone, insulin-like growth factor 1 [IGF-1], and prolactin). There is an unexplained discrepancy between the reported high prevalence of pituitary insufficiency among persons with PES and its low prevalence in epidemiologic studies. We suspect that the former may be high because of selection bias in the publications that we reviewed, or else the latter may be erroneously low.

Kallmann syndrome (KS) is a form of hypogonadotropic hypogonadism in combination with a defect in sense of smell, due to abnormal migration of gonadotropin-releasing hormone-producing neurons. We report a case of a 17-year-old Tunisian male who presented with eunuchoid body proportions, absence of facial, axillary and pubic hair, micropenis and surgically corrected cryptorchidism. Associated findings included anosmia. Karyotype was 46XY and hormonal measurement hypogonadotropic hypogonadism. MRI of the brain showed bilateral agenesis of the olfactory bulbs and 3.5 mm pituitary microadenoma. Hormonal assays showed no evidence of pituitary hypersecretion. Learning points: The main clinical characteristics of KS include hypogonadotropic hypogonadism and anosmia or hyposmia. MRI, as a non-irradiating technique, should be the first radiological step for investigating the pituitary gland as well as abnormalities of the ethmoid, olfactory bulbs and tracts in KS. KS may include anterior pituitary hypoplasia or an empty sella syndrome. The originality of our case is that a microadenoma also may be encountered in KS. Hormonal assessment indicated the microadenoma was non-functioning. This emphasizes the importance of visualizing the pituitary region in KS patients to assess for hypoplastic pituitary malformations or adenomas. Background Kallmann syndrome (KS) is a rare disorder first described in 1856 and later studied by Kallmann in 1944 (1). It is now designated as olfactogenital dysplasia with an association between agenesis of the olfactory bulbs and hypogonadism. The prevalence of KS is still unknown. The reported incidence is 1 in 8000 to 1 in 10 000 in men and is rarer in women (2). KS is a genetically heterogeneous disease. Its inheritance may be X-linked, autosomal dominant or autosomal recessive. There are more than 24 genes underlying KS that have been identified (3). Mutations in these genes are thought to interfere with expression of cell markers that guide migrating neurons, leading to failed migration of GnRH neurons and olfactory neurons to the forebrain during fetal development. The main clinical characteristics of KS include hypogonadotropic hypogonadism and anosmia or hyposmia. Less common phenotypes include cardiovascular anomalies, unilateral renal agenesis, cleft palate and cleft lip, cryptorchidism and osteoporosis (4). Magnetic resonance imaging (MRI) can show abnormalities of the olfactory system and other forebrain structures (5). Other abnormalities may be discovered by using MRI because of its high resolution and multiplanar capabilities, such as pituitary abnormalities (6). The association of KS with a pituitary microadenoma has not been well described in the literature. In this case report study, we describe a combination of KS discovered in the setting of a delayed puberty and olfactory impairment, and an MRI finding of a pituitary microadenoma.

Objective To review the results of sphenoid sinus lateral recess (SSLR) cerebrospinal fluid (CSF) leaks treated with the endoscopic endonasal minimal transpterygoid approach (EEMTPA) and to discuss the surgical technique and outcomes. Methods We performed a retrospective analysis of 13 cases who underwent SSLR CSF leak repair through the EEMTPA in our clinic between September 2008 and December 2017. Demographic and etiological features with reconstruction and surgical outcomes were examined. Mean follow-up time was 6.1 years. Results In regard to etiology, the SSLR CSF leaks included 9 patients with spontaneous, 2 patients with traumatic, and 2 with iatrogenic causes. CSF leak was at the left lateral recess in 8 cases and at right lateral recess in 5 cases. Nine patients had empty sella syndrome, and 11 patients had meningoencephaloceles in addition to SSLR CSF leaks. All patients underwent surgery through the EEMTPA, and a multilayer closure with tissue overlay grafts were used for reconstruction. A pedicled nasoseptal flap and/or pedicled middle turbinate flap were applied to the area of the leak in all cases. One patient had a persistent CSF leak and another had recurrence, both of which required revision surgery. Our overall success rate was 100%. Conclusions EEMTPA is a safe and effective method that can be used to treat challenging pathologies at the SSLR, including CSF leaks accompanying meningoencephaloceles. Furthermore, the success rate of EEMTPA for SSLR CSF leaks can be increased by applying endoscopic skull base reconstruction techniques such as the pedicled nasoseptal flap and pedicled middle turbinate flap.

Ectopic pituitary adenoma is a rare entity that is most commonly located in the sphenoid sinus. We report a case of a patient with ectopic pituitary adenoma with no functional expression associated with empty sella turcica, which gives rise to a broad differential diagnosis. Although it is a benign neoplasm, necrosis is encountered in a proportion of cases. Magnetic resonance imaging is the diagnostic method of choice for hypothalamic-pituitary-related endocrine diseases with endoscopic biopsy for histological confirmation. It is important to include pituitary markers in the immunohistochemical diagnostic panel.

Describe clinical and radiographic features of sigmoid sinus wall anomalies (SSWA) associated with pulsatile tinnitus (PT) and determine factors predictive of response to surgery.Preoperative diagnostic imaging and treatment response were reviewed after surgical repair of 40 ears among 38 consecutive patients presenting with PT associated with SSWA who underwent transtemporal sinus wall reconstruction.Twenty-three ears had isolated sigmoid sinus dehiscence, and 17 had diverticulum. The rates of transverse sinus stenosis (TSS) and empty sella, 66% and 32% respectively, were significantly higher than in historical controls (P = 0.02 and 0.001). Thirty-six out of 40 subjects (90%) had complete resolution of their PT following surgery, including all those with a diverticulum. For subjects with dehiscence alone without diverticulum, a favorable response to surgery was strongly associated with the presence of TSS (P = 0.01) and empty sella (P = 0.02).Sigmoid sinus diverticulum and dehiscence are a clinically important cause of PT. Women of childbearing age with an elevated body mass index (BMI) are commonly affected, and there is a high rate of associated TSS and empty sella. Transtemporal sinus wall reconstruction has a high rate of success in appropriately selected patients. Patients with isolated sinus wall dehiscence without diverticulum, TSS, or empty sella are less likely to respond to transtemporal sinus wall reconstruction. These data imply a multifactorial cause of PT in at least some patients with SSWA.4 Laryngoscope, 128:S1-S13, 2018.

Purpose: 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. Methods: 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. Results: 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. Conclusions: The intradural chiasmapexy is indicated in treatment of SESS, instead the extradural approaches are suggested for surgical management of PESS. © 2017 Springer Science+Business Media, LLC

Hamartomas of the tuber cinereum is a rare congenital mass lesion more frequently seen in boys, and is located between the mammillary bodies and pituitary stalk. Hamartoma is not a proper tumor but a congenital non-tumor heterotopia. On histology, a hamartoma consists of accumulations of small and large neurons, astrocytes and oligodendrocytes, which are in correct ratio with tuber cinereum tissue (Matsko and Korshunov 1998a, b). They do not invade the surrounding structures, but may progress slowly, sometimes reaching rather large sizes. The most frequent and early clinical manifestation occurs approximately at the age of 2 years: isosexual precocious puberty. Other signs such as epileptic seizures and behavioral changes occur later when a hamartoma acquires a diameter of about 10 mm (Mittal et al. 2013).

To assess and quantify the prevalence and co-occurence of a sellar floor lowering and empty sella phenomenon in patients with GH (growth hormone)-secreting pituitary adenoma.A total number of 159 acromegalic patients were included in this study, as well as two control groups (150 patients with non-GH-secreting adenomas and 50 patients without pituitary adenomas). Magnetic resonance images of all patients were evaluated for presence of an empty sella, downward and lateral tumor extension, and maximum superoinferior diameter of the mass. Additionally, these values were correlated with growth hormone and IGF-1 levels.The empty sella phenomenon was detected significantly more often in patients with a GH-secreting adenoma with a prevalence of 22% vs. 5.3% in non-GH-secreting adenomas (p 0.001) or 8% in the healthy control group (p = .036). Moreover, GH-secreting adenomas presented with a significant rate of downward tumor extension (74.8% vs. 35.5%; p 0.001), whose extent correlated inversely but weakly with the GH hormone level (r = - 0.17; p = .036). It was also found that a decreased superoinferior diameter and higher ratio of intrasellar to suprasellar extension are predictive quantitative values for the presence of a GH-secreting adenoma (area under curve, 0.712).GH-secreting pituitary adenomas are frequently associated with an empty sella phenomenon. Moreover, GH-secreting adenomas are frequently accompanied by an enhanced, quantitatively measurable impression of the sellar floor. Hypothetically, this is caused by tumor-induced local bone remodeling processes.

Background Prolactinomas are typically treated nonsurgically with a dopamine agonist. Once the tumor shrinks, adjacent eloquent structures, such as the optic apparatus, can become skeletonized and herniate into the dilated parasellar space. Case Description A 48-year-old man with a prolactin-secreting macroadenoma treated with cabergoline presented with progressive bitemporal hemianopsia. Magnetic resonance imaging showed no recurrence of disease and a stretched optic chiasm herniating into an empty sella. Elevation of the optic chiasm via a transnasal transsphenoidal approach with ALLODERM graft and septal cartilage strut was performed. The patient was discharged home the next day with significant improvement in vision; magnetic resonance imaging showed interval elevation of the optic chiasm. Conclusions We review secondary empty sella syndrome and discuss surgical strategies for optic chiasmapexy.

This article analyzes the case of a 74 year old patient who was hospitalized four times with recurrent complaints, which consisted of hypothermia, hypotension, weakness, and a hyponatremia, and were always caused by an underlying acute infection. Laboratory results showed an hypothyroidism, a secondary adrenal insufficiency, a secondary hypogonadism, and a growth hormone deficiency, which led to a diagnosis of pituitary dysfunction. Magnetic resonance imaging of the brain showed an 'empty sella', a non-visualization of the pituitary gland caused by an herniation of a supra-sellar cistern into the pituitary fossa. Considering the lack of an underlying pituitary tumor, a treatment consisting of partial hormonal substitution was started, eventually resulting in the full recovery of the patient.

The psycho-neuroendocrine-immune approach relies on the concept of considering diseases from a holistic point of view: the various components (psyche, nervous system, endocrine system, and immune system) control the diseased organ/apparatus and in turn are influenced by a feedback mechanism. In this article, we will consider the psycho-neuroendocrine-immune approach to coloproctological disorders, by providing clinical cases and discussing them in light of this approach.

Background: Spontaneous CSF leak represents less frequent cause of CSF leak, but cases are more difficult to control, with the highest failure rate and recurrence despite adequate repair. The problems in these cases might be related to an underlying undiagnosed associated intracranial hyper-tension. Recognition and long-term treatment of elevated ICP is therefore critical to the successful management of these patients. Objective: To evaluate the CSF pressure in cases of spontaneous CSF rhinorrhea and to describe our same setting combined protocol to the repair of the leak, measurement and management of CSF pressure. Patients and Methods: All patients presenting to Ain Shams University Hospitals, Cairo, Egypt, with spontaneous CSF leak were included prospectively in the study. Clinical and radiologic data were collected to suspect elevated intracranial pressure. After CSF repair, CSF pressure was measured and if found to be more than 20 cmH2O, a lumboperitoneal shunt was used. Results: Twenty-seven cases, 23 women and 4 men, presented with spontaneous CSF leak. 23 patients had BMI above 30. All patients had empty sella syndrome (100%), and a meningoencephalocele was found in 13 cases (48%). CSF pressure ranged from 5 to 39 cmH2O (mean = 28.7). A pressure above 21 cmH2O was found in19 patients (70%) and subsequently had lumboperitoneal shunt in the same setting. No recurrence occurred in this subset of patients with 6 - 60 months follow-up period. Conclusion: A selective and specific same setting protocol can result in a better diagnosis and control of the accompanying elevated ICP in cases of spontaneous CSF leak. It avoids a second operative intervention, and shortens the hospital stay, with an increase in the success rate. In the same time, the smaller number of patients with normal ICP can avoid further drainage.

An empty sella occurs due to herniation of the arachnoid through an incompetent diaphragma sellae. Over time, cerebrospinal fluid (CSF) pulsations may enlarge the sella and compress the gland against the floor of the sella. Empty sella syndrome is considered as a less common entity and is usually asymptomatic and an incidental finding. However, it can be a manifestation of increased intracranial pressure and can be occasionally severe. Compression of the pituitary gland may affect function, or traction on the optic chiasm and nerves may cause visual symptoms. An empty sella may be classified as primary when this occurs in persons who have not received pituitary radiation or pituitary surgery, while an empty sella discovered following such procedures is classified as secondary empty sella. In this report, we presented a 41-year-old multiparous patient who presented to us with symptoms of headache and left sided hemi-sensory disturbance. Examination was unrevealing except for the fact that she was obese. On evaluation, she was detected to have impaired blood sugars, dyslipidemia and vitamin insufficiency. MRI brain revealed presence of Empty Sella. Further hormonal analysis was normal. She was treated conservatively and regular follow-up was advised.

A 55-year-old male was admitted to emergency department with a hypoglycemic shock of unknown origin. He was presented with tonic seizure activity after admission. Initial diagnostic procedure could exclude diabetes mellitus, drug side effects, and exogenous insulin application. Detailed evaluation of the patient's history revealed that the patient had experienced repeated hypoglycemic episodes for 2 years. He was diagnosed with hypothyroidism six years ago. Initial laboratory investigations revealed hypoglycemia, hyponatremia, and low plasma cortisol level (0.18 pg/dᄂ)· Sellar magnetic resonance imaging showed empty sella. Replacement therapy with hydrocortisone resulted in the improvement of clinical symptoms. Combined pituitary stimulation test with exception of hypoglycemia induced growth hormone and cortisol stimulation test was performed. The response of thyroid stimulating hormone, prolactin, follicle-stimulating hormone, and luteinizing hormone was normal. We report the case of empty sella syndrome associated with hypoglycemic shock due to with multiple anterior pituitary hormone deficiencies.

Primary empty sella syndrome is a phenomenon caused by cerebrospinal fluid filling resulting from the herniation of the subarachnoid space within the sella. The pituitary function of primary empty sella syndrome is usually normal. But sometimes this syndrome causes some degree of pituitary dysfunction associated with hypersecretion or deficiency of pituitary hormone. Central diabetes insipidus with primary empty sella syndrome is rarely reported. Furthermore, most of those cases are accompanied by other pituitary dysfunction. We report here on a 35-year-old female who suffered from polyuria, polydipsia since childhood. She was diagnosed with central diabetes insipidus with primary empty sella syndrome. She had no anterior pituitary dysfunction except mild hyperprolactinemia.