"Qué hay de nuevo""Osseous Remodeling Technique of the Sella Turcica: A New Surgical Option for Primary Empty Sella Syndrome" / "What's new ""Osseous Remodeling Technique of the Sella Turcica: A New Surgical Option for Primary Empty Sella Syndrome"

La silla turca vacía (STV) o como se la denominó más recientemente, aracnoidocele selar, es una entidad clínico-radiológica con una prevalencia que, si bien no se conoce con exactitud, se estima que es entre el 2 al 20%1. Dos tipos de STV han sido descriptas según su etiología: primaria y secundaria. En la primaria, no existe antecedente de cirugía hipofisaria previa y se cree que se produce debido a un diafragma selar deficiente. Por otro lado, cuando se genera luego de la exéresis de un tumor hipofisario se denomina secundaria. Por lo general, la STV primaria es diagnosticada como un hallazgo incidental en resonancia magnética, en donde se evidencia una herniación aracnoidea y de líquido cefalorraquídeo (LCR) a través del diafragma selar, comprimiendo la glándula hipofisiaria contra el piso de la silla turca. Algunos pacientes pueden manifestar síntomas clínicos (síndrome de silla turca vacía), entre ellos, cefalea, alteraciones endocrinológicas, fístula de LCR y alteraciones visuales. La cirugía está indicada en estas dos últimas manifestaciones

Variaciones anatómicas de la silla turca en radiografías laterales de cráneo. Hospital "Faustino Pérez", 2017-2018 / Anatomic variants of sella turcica in lateral crania radiographies. Hospital "Faustino Pérez¨, 2017-2018

RESUMEN Introducción: diversos autores reflejan que la morfología de la silla turca constituye un factor predisponente para algunas enfermedades. Por ejemplo, se considera que existe correlación entre la morfología de esta estructura anatómica y varias patologías; como el síndrome de la silla turca vacía, síndrome de Williams, paladar hendido, entre otras. Objetivo: describir las variaciones anatómicas de la silla turca que se observan en las radiografías laterales de cráneo del Hospital Universitario "Faustino Pérez" de la ciudad de Matanzas, en el período de enero del 2017 a enero del 2018. Materiales y métodos: el universo fue 140 radiografías laterales de cráneo, de estas 85 pertenecieron al sexo femenino y 55 al masculino. Las variables estudiadas fueron edad, sexo y variaciones anatómicas de la silla turca. Se emplearon métodos teóricos y empíricos. Resultados: se observó un predominio de la variación anatómica de la silla turca en forma de U, en ambos sexos. Seguido de la forma de J, predominando la variación en forma de U en el grupo etario de 41 a 50 años y la forma de J en los pacientes mayores de 60 años. Conclusiones: es imprescindible el conocimiento de la anatomía normal de la silla turca y de sus variaciones anatómicas, tanto para las especialidades quirúrgicas como para las no quirúrgicas. Un análisis exhaustivo de la morfología de esta estructura es necesario para establecer parámetros que excluyan determinadas patologías.

ABSTRACT Introduction: several authors declare that sella turcica morphology is a predisposing factor to several diseases. For example, it is considered that there is a correlation between the morphology of this anatomical structure and several pathologies like empty sella turcica syndrome, Williams syndrome, cleft palate and others. Objective: to describe the anatomical variants of sella turcica observed in side cranial radiographies of the University Hospital ¨Faustino Perez¨ of Matanzas, in the period January 2017-January 2018. Materials and methods: the universe was 140 side cranial radiography: 85 belonged to female patients and 55 to male patients. The studied variables were age, sex and sella turcica anatomical variables. Theoretic and empirical methods were used. Results: it was observed a predominance of the U-shaped sella turcica anatomical variant in both sexes, followed by the J-shaped one. The U-shaped form predominated in the 41-50-years-old age-group and the J-shaped form in patients elder than 60 years. Conclusions: it is essential to know sella turcica normal anatomy and its anatomical variables, both for the surgical specialties and for the non-surgical ones. It is necessary the exhaustive analysis of this structure to establish parameters excluding several pathologies.

Acromegalia y silla turca vacía: una asociación infrecuente: presentación de un caso / Acromegaly and empty sella syndrome: an infrequent association: presentation of a case

La acromegalia, originada por un exceso de producción de Hormona de crecimiento (Gh), se caracteriza por crecimiento somático exagerado, alto riesgo cardio-metabólico, así como reducción de la expectativa de vida. Tiene una incidencia de 3-4 casos por millón de habitantes. El diagnóstico se retrasa hasta 10 años aumentando la morbi-mortalidad. Las alternativas terapéuticas incluyen medicamentos y cirugía, que van encaminados a reducir los efectos de masa tumoral, normalizar los parámetros bioquímicos y resolver las manifestaciones clínicas. En casos muy infrecuentes, el tumor hipofisario que la origina se asocia a silla turca vacía.

Acromegaly, caused by an excess production of growth hormone (Gh), it is characterized by exaggerated somatic growth, high cardio-metabolic risk, as well as reduction of life expectancy. It has an incidence of 3-4 cases per million population. The diagnosis is delayed up to 10 years increasing morbidity and mortality. The therapeutic alternatives include medications and surgery, which are aimed at reduce the effects of tumor mass, normalize biochemical parameters and resolve clinical manifestations. In very infrequent cases, the pituitary tumor that originates it is associated with empty sella syndrome. Key words: Acromegaly, Empty sella syndrome, Pituitary tumor.

Unusual presentation of Sheehan's syndrome with severe hyponatremia and recurrent symptomatic hypoglycemia: A case report / Journal of the ASEAN Federation of Endocrine Societies

@#<p style="text-align: justify;">Sheehan's syndrome (SS) is postpartum hypopituitarism caused by necrosis of the pituitary gland. The onset in most cases is several months or even years after the inciting delivery, so it is often unrecognized and not adequately treated. Because SS often evolves slowly, it is usually diagnosed late. We report a 47-year old woman with loss of consciousness. Fourteen years ago, she had postpartum hemorrhage with subsequent amenorrhea and failure to lactate. Laboratory investigation showed low blood sugar and serum sodium levels, amid normal cortisol and thyroid function tests. Magnetic resonance imaging (MRI) of the pituitary revealed an empty sella consistent with SS. The presentation of hypoglycemia and hyponatremia are less known complications of Sheehan's syndrome with only a few documented in case reports.</p>

Related factors analysis of spontaneous cerebrospinal fluid leak recurrence after endoscope operation / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To Summarize and analyze the clinical characteristics and treatment of patients with spontaneous cerebrospinal fluid rhinorrhea, and to explore the related factors of recurrence.@*METHOD@#Retrospective- ly analyze the clinical data of 58 patients of spontaneous cerebrospinal fluid rhinorrhea from July 2002 to July 2012, combined with its clinical characteristics, and statistically analyze the related recurrence factors.@*RESULT@#Fifty-eight cases were accepted the nasal endoscopic repairment of cerebrospinal fluid rhinorrheak, follow-up 3 years, 20 cases (34.5%) recurred, 1 case recurred in half a year after operation. In the first year, there were 10 cases recurred. In the second year there were 9 cases recurred, and 1 case recurred in the third year. Through multiariable analysis it was found that higher BMI, empty sella and skull base bone defect were the independent risk factors influencing the recurrence of spontaneous cerebrospinal fluid rhinorrhea (P < 0.05).@*CONCLUSION@#The recurrence rate of spontaneous cerebrospinal fluid rhinorrhea is high, needing long-term follow-up. Higher BMI, empty sella, skull base bone defect are independent risk factors of recurrence of the disease, and the treatment should be individualized.

Polyglandular Autoimmune Syndrome Type 2 Complicated by Multiple Organ Failure, Empty Sella Syndrome and Aplastic Anemia

Polyglandular autoimmune syndrome (PAS) is a group of syndromes comprised of glandular and extra-glandular disorders characterized by autoimmunity. A 57-year-old woman presented with acute progressive dyspnea and generalized weakness for several months. The patient was assessed to have acute congestive heart failure with cardiomyopathy, chronic renal failure with hyporeninemic hypoaldosteronism, and pancytopenia in addition to primary hypothyroidism and adrenal insufficiency. With the diagnosis of PAS type 2 complicated by multiple organ failure (MOF), medium-dose prednisolone (30 mg/d) was introduced primarily to control the activity of autoimmunity, which triggered MOF over the adrenal insufficiency. Levothyroxine (25 microg/d) was followed for replacement of the thyroid hormone deficiency. However, the symptoms and signs fluctuated, depending on the dosage of prednisolone, and progressively worsened by empty sella syndrome and aplastic anemia. Here, we report on a case of PAS type 2 with MOF and atypical complications, and suggest that recognition, assessment, and control of PAS as a systemic autoimmune disease may be essential.

Adenohipopituitarismo de rápida instalación, sin correlato imagenológico / Acute onset adenohypopituitarism without an image diagnosis

We report two cases of acute onset of adenohypopituitarism without a sellar MRI finding. The first case is a postmenopause woman complaining of fatigue, weakness, nausea, vomiting, diarrhea and mild weight loss. She was extensively studied with upper gastrointestinal endoscopy, colonoscopy and abdominal CT. An incidental possible pituitary enlargement on a Brain CT opened a pituitary function study, revealing adenohypopituitarism. The sellar MRI was perfectly normal, without anatomical explanation. The second case is a postmenopause woman complaining of fatigue and weakness, who had an episode of syncope and concomitant hyponatremia. Her study revealed adenohypopituitarism and a primary empty sella image in the MRI. The clinical problem of adenohypopituitarism without an image diagnosis brings the ethical dilemma to make a “blind” transsphenoidal biopsy or just treat them without a certain diagnosis. Patients with Empty Sella in the MRI show frequently normal pituitary function and it is not considered as a cause of so extensive hypopituitarism. Sometimes the clinical and image evolution can suggest the etiology and require of histological sample, so it is rational to keep an active surveillance and repeat the functional tests and Sellar MRI within the follow up...

A Case with Empty Sella Syndrome Combined with Multiple Anterior Pituitary Hormone Deficiencies Presenting as Hypoglycemic Coma / 고신대학교의과대학학술지

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 microg/dL). 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.

A Case of Primary Empty Sella Syndrome with Central Diabetes Insipidus

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.

Children with isolated growth hormone deficiency: Empty sella versus normal sella.

BACKGROUND: Empty sella (ES) may be associated with variable clinical conditions ranging from the occasional discovery of a clinically asymptomatic pouch within the sella turcica to severe intracranial hypertension and rhinorrhea. The need for replacement hormone therapy in ES, as in other syndromes that may cause hypopituitarism, must be assessed for every single hormone, including growth hormone (GH). AIM: To determine whether or not the presence of ES could allow some changes in the GH responses of the isolated growth hormone deficiency (GHD) patients. MATERIALS AND METHODS: We included a cohort of 59 short stature children and adolescents with isolated GHD. According to computed tomography finding, they were classified into 2 groups: Group 1 included 40 children with normal sella and 19 children with ES in Group 2. All patients received recombinant human growth hormone (rhGH) with a standard dose of 20 IU/m2/week. RESULTS: The baseline results were not significantly different for all variables except weight standard deviation was smaller with statistical significant difference (P = 0.02). We identified no significant differences when comparing both groups, except for height standard deviation (HTSD) after the first year of therapy which revealed significant difference in favor of group 1. When comparing pre- and the two post-treatments HTSD results of the studied cases, all showed significant changes after GH therapy. The results of related variables pre-and post-treatment in both the groups showed significant improvement in all variables of the two groups of the study. CONCLUSION: Our study showed a similar stature outcome in the two treatment groups.

The characteristics and treatment of empty sella combined cerebrospinal fluid leakage of nasal / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To study the feature and treatment method of patients with empty sella merger cerebro-spinal fluid leakage of nasal.@*METHOD@#There were 8 cases with empty sella merger cerebrospinal fluid leakage of nasal, 2 cases were accepted the repairing surgery of cerebrospinal fluid leakage one time, 4 cases were accepted the repairing surgery of cerebrospinal fluid leakage used endoscope 2 times, 1 case was accepted repairing surgery of cerebrospinal fluid leakage used endoscope merge craniotomy and ventricle celiac bypass, 1 case recurrences after repairing surgery of cerebrospinal fluid was recurred after conservative treatment. Some postoperative were stayed in bed for three weeks and lumbar drainage for 1 week.@*RESULT@#One case of cerebral hemorrhage after surgery was cured with craniotomy, followed for 2 years without recurrence. One case was recurred after conservative treatment. Two cases recurrences after surgery 3 years ago were accepted surgery again followed by one year without recurrence. One case who recurrence 1 year later was accepted repairing surgery of cerebrospinal fluid leakage used endoscope merge craniotomy and ventricle celiac bypass followed six months without recurrence. One cash after once surgery was followed half a year without recurrence. One case with recurrence 5 years later was accepted repairing surgery again.@*CONCLUSION@#The patient with empty sella combined cerebrospinal fluid leakage of nasal was rare, the main method was endoscopic sinus surgery treatment, but it recurred usually. The patients with repeatedly recurrence can be considered to accepted the surgery of ventricle celiac bypass. It required long-term postoperative follow-up and review.

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

The empty sella syndrome is defined as the herniation of a subarachnoid space within the sella, with a flat-pressed pituitary gland accompanying hormonal problems or cerebrospinal fluid (CSF) rhinorrhea. CSF rhinorrhea is rare in the primary empty sella syndrome. We report a case of primary empty sella syndrome with CSF rhinorrhea in a 57 year-old woman. The CSF rhinorrhea was treated successfully by endoscopic transnasal transsphenoidal approach.

Sheehan syndrome with reversible dilated cardiomyopathy

Cardiac abnormalities in patients with Sheehan syndrome are uncommon. A case of Sheehan syndrome with dilated cardiomyopathy is presented in whom hormone replacement with levothyroxine and prednisolone resulted in complete recovery of cardiomyopathy. A 25-year-old woman presented with lactation failure, secondary amenorrhea, features of hypothyroidism and a hypocortisol state following severe postpartum hemorrhage after her last child birth. She also had smear positive pulmonary tuberculosis. After starting antitubercular treatment, she developed shock, suggestive of hypocortisol crisis. Hormonal investigations revealed evidence of panhypopitutarism and magnetic resonance imaging revealed partial empty sella. Meanwhile echocardiography revealed evidence of dilated cardiomyopathy [DCM]. The patient was given replacement therapy in the form of glucocorticoids and levothyroxine in addition to antitubercular treatment. She improved and on follow-up over a period of 7 months, the DCM completely reversed. To our knowledge this is the first report of reversible DCM in a patient with Sheehan syndrome

Morphometric Study of the Korean Adult Pituitary Glands and the Diaphragma Sellae

OBJECTIVE: To investigate the morphometric characteristics of the pituitary gland and diaphragma sellae in Korean adults. METHODS: Using the 33 formaline fixed adult cadavers (23 male, 10 female), the measurements were taken at the diaphragma sellae and pituitary gland. The authors investigated the relationship between dura and structures surrounding pituitary gland, morphometric aspects of pituitary gland and stalk, and morphometric aspect of central opening of diaphragma sellae. RESULTS: The boundary between the lateral surface of pituitary gland and the medial wall of cavernous sinus was formed by the thin dural layer and pituitary capsule. The pituitary capsule adherent tightly to the pituitary gland was observed to continue from the diaphragma sellae. Mean width, length, and height of the pituitary gland were 14.3 +/- 2.1, 7.9 +/- 1.3, and 6.0 +/- 0.9 mm in anterior lobes, and 8.7 +/- 1.7, 2.9 +/- 1.1, and 5.8 +/- 1.0 mm in posterior lobes, respectively. Although all dimensions of anterior lobe in female were slightly larger than those in male, statistical significance was noted in only longitudinal dimension. The ratio of posterior lobe to the whole length of pituitary gland was about 27%. The mean thickness of pituitary stalk was 2 mm. The diaphragmal opening was 5 mm or more in 26 (78.8%) of 33 specimen. The opening was round in 60.6% of the specimen, and elliptical oriented in an anterior-posterior or transverse direction in 39.4%. CONCLUSION: These results provide the safe anatomical knowledge during the transsphenoidal surgery and may be helpful to access the possibility of the development of empty sella syndrome.

Pituitary magnetic resonance imaging experience in Jordan

To assess the pituitary findings as demonstrated on MRI and to compare the results with the data published in the literature. One thousand, one hundred and thirty-eight pituitary MRI's with and without intravenous contrast media [gadolinium] were performed over 6 years from 2001 to 2007 in the Department of Diagnostic Radiology, Jordan University Hospital, Amman, Jordan. The patients were referred from various departments and were evaluated for pituitary, other sellar, and juxtasellar abnormalities. The results were compared with those in the published literature. Four hundred and eight-three normal scans were excluded from the study. The remaining 655 were abnormal, pituitary adenoma was detected in 327 [49.9%], microadenoma was present in 213 [32.5%], and macroadenoma in 114 [17.4%]. Partial empty sella was seen in 157 [24%], diffuse pituitary gland enlargement in 98 [14.9%], ectopic pituitary posterior lobe in 13 [2%], and other findings in 31 [4.7%]. The incidence of pituitary adenoma was equal in both genders; however, microadenoma was more common, affected a younger age group, and was predominately seen in females. The other parameters showed agreement with the published literature

Analysis of Empty Sella Secondary to the Brain Tumors

OBJECTIVE: The definition of empty sella syndrome is 'an anatomical entity in which the pituitary fossa is partially or completely filled with cerebrospinal fluid, while the pituitary gland is compressed against the posterior rim of the fossa'. Reports of this entities relating to the brain tumors not situated in the pituitary fossa, have rarely been reported. METHODS: In order to analyze the incidence and relationship of empty sella in patients having brain tumors, the authors reviewed preoperative magnetic resonance imaging (MRI) of 72 patients with brain tumor regardless of pathology except the pituitary tumors. The patients were operated in single institute by one surgeon. There were 25 males and 47 females and mean patient age was 53 years old (range from 5 years to 84 years). Tumor volume was ranged from 2 cc to 238 cc. RESULTS: The overall incidence of empty sella was positive in 57/72 cases (79.2%). Sorted by the pathology, empty sella was highest in meningioma (88.9%, p = 0.042). The empty sella was correlated with patient's increasing age (p = 0.003) and increasing tumor volume (p = 0.016). CONCLUSION: Careful review of brain MRI with periodic follow up is necessary for the detection of secondary empty sella in patients with brain tumors. In patients with confirmed empty sella, follow up is mandatory for the management of hypopituitarism, cerebrospinal fluid (CSF) rhinorrhea, visual disturbance and increased intracranial pressure.

A case of acromegaly with empty sella syndrome associated with colonic neoplasm / 대한내과학회지

Acromegaly is a disorder caused by hypersecretion of growth hormone (GH) and insulin-like growth factor-1 (IGF-1). The most common cause of acromegaly is a pituitary GH-producing adenoma. Complete or partial disappearance of the adenoma, probably as a result of hemorrhage or infarction, may lead to empty sella. A case of acromegaly with empty sella syndrome has rarely been reported in Korea. It has been suggested that acromegaly might be associated with the incidence of colon neoplasm. Here, we describe a case of acromegaly with empty sella syndrome in a patient who was diagnosed with colon cancer.

A Case of Severe Hyponatremia Associated with Hypopituitarism due to Hemorrhagic Fever with Renal Syndrome / 대한신장학회지

Most of the patients with hemorrhagic fever with renal syndrome (HFRS) by Hantaviruses recover completely. However, the prevalence of hypopituitarism as sequel of HFRS may be approximately 10%. However, it is rare for hyponatremia to present as a manifestation of hypopituitarism due to HFRS. A 42-year-old Asian woman presented with anorexia, nausea, and vomiting. She had a history of HFRS 7 years ago. During her first hospital admission, her serum sodium was 103 mmol/L. The rapid adrenocorticotropic hormone (ACTH) stimulation test showed no abnormal findings suggesting the possibility of adrenal insufficiency. After she underwent fluid therapy for 5 days, her serum sodium level was slightly increased to 131 mmol/L, and her clinical symptoms were improving. The next day after she was discharged, she suffered from the same symptoms and was admitted to our hospital again. On the day of her second admission, her serum sodium was 117 mmol/L. A combined pituitary function test showed diminished function of the anterior pituitary gland, which led to secondary adrenal insufficiency. Magnetic resonance imaging findings of her sella were compatible with empty sella turcica. After the replacement of prednisolone, her serum sodium levels returned to normal. Pituitary dysfunction should be included in the differential diagnosis of severe hyponatremia, especially in patients who suffered HFRS. This case also suggests that the rapid ACTH stimulation test has limitations with regard to the differential diagnosis of primary or secondary adrenal insufficiency.

A Case of Parathyroid Adenoma Presenting as Acute Pancreatitis Accompanied with Empty Sella / 영남의대학술지

The incidence of coexisting hyperparathyroidism and empty sella syndrome is rare and the etiology and incidence of their coexistence is not known. The association of hyperparathyroidism and the empty sella syndrome may be related to multiple endocrine neoplasia (MEN) syndrome due to a genetic disorder. We experienced a rare case of hyperparathyroidism presenting as acute pancreatitis combined with empty sella. We report here a 37-year old female who manifested epigastric pain because of acute pancreatitis. She had hypercalcemia due to parathyroid adenoma. A pituitary gland was not visible in the sella turcica on MRI scans. On genetic analysis, she did not show a mutation of the MENIN gene. Empty sella is thought to be a coincidental finding with hyperparathyroidism.