Diabetes insípida, panhipofisitis y silla turca vacía. Presentación de un caso / Diabetes insipidus, panhypophysitis, and empty sella turcica. Presentation of a case

RESUMEN La diabetes insípida central se produce por déficit de síntesis o secreción de hormona antidiurética. Es una entidad de muy baja prevalencia que se puede ver asociada a hipofisitis linfocítica y silla turca vacía. Sin embargo, el diagnóstico histopatológico solo se realiza cuando es necesaria la cirugía debido a un compromiso neurológico. Se presentó el caso de un paciente masculino de 41 años que acudió a consulta porque orinaba frecuentemente y bebía mucha agua. Se le realizó prueba de supresión de líquidos, seguida de prueba de la vasopresina, que fueron consistente con el diagnóstico de diabetes insípida central. La resonancia magnética de la hipófisis reveló silla turca vacía parcial y signos de infundíbulo-neurohipofisitis, coincidencia que ha sido escasamente reportada. En el seguimiento se evidenció hipogonadismo hipogonadotrópico y baja reserva adrenal. Se indicó tratamiento de reemplazo hormonal con desmopresina y testosterona, con lo cual el paciente ha mantenido buena calidad de vida. Se concluye que la diabetes insípida puede ser la primera manifestación de una panhipofisitis. La asociación de estas enfermedades con el síndrome de silla turca vacía es infrecuente, pero puede ser el curso natural de la enfermedad.

ABSTRACT Central diabetes insipidus is caused by a deficiency in the synthesis or secretion of antidiuretic hormone. It is a very low prevalence entity that can be seen associated with lymphocytic hypophysitis and empty sella turcica. However, histopathological diagnosis is only made when surgery is necessary due to neurological compromise. The case of a 41-year-old male patient who came to the clinic because he urinated frequently and drank a lot of water was presented. A fluid suppression test was performed, followed by a vasopressin test, the results of which were consistent with a diagnosis of central diabetes insipidus. Magnetic resonance imaging of the pituitary gland revealed partial empty sella turcica and signs of infundibulo-neurohypophysitis, a coincidence that has been rarely reported. In the follow-up, hypogonadotropic hypogonadism and low adrenal reserve were revealed. Hormone replacement treatment with desmopressin and testosterone was indicated, with which the patient has maintained a good quality of life. It is concluded that diabetes insipidus may be the first manifestation of panhypophysitis. The association of these diseases with the empty sella syndrome is rare, but it may be the natural course of the disease.

Snake bite induced delayed hypopituitarism: a rare case report

Hypopituitarism following snake bite induced AKI and dialysis is an uncommon complication. Often the presentation is delayed and Can present with a myriad of features. We present a case of a 27 year old male patient, with past history of snake bite and acute kidney injury (AKI) requiring dialysis and which subsequently resolved, presenting to us with fatigability and weakness, absence of secondary sexual character and poor academic performance. On examination, the patient had pale white and coarse skin. Hoarseness of voice was present. There was proximal upper and lower limb muscle weakness present with pseudo myotonia on examination. There was loss of axillary and pubic hair with low testicular volume. Routine reports suggested mildly elevated creatinine with severely elevated Triglyceride levels. Creatine phosphokinase (CPK) was raised. Hormonal profile revealed low free thyroxine (FT4) and serum 8 a.m. Cortisol but an inadequate increase in thyroid stimulating hormone (TSH) and adrenocorticotropic hormone (ACTH) levels were suggestive of central hypopituitarism. Luteinizing hormone (LH), follicle-stimulating hormone (FSH), Insulin-like growth factor 1 (IGF-1) levels were reduced. Neuroimaging revealed empty sella suggestive of pituitary apoplexy. The above were suggestive of delayed hypopituitarism following post snake bite dialysis mediated pituitary apoplexy. In all cases of snake bite, a common occurrence in our country the possibility of hypopituitarism should be kept in mind and the hormonal profile followed up closely along with the renal parameters.

Hiponatremia asociada a hipopituitarismo en síndrome de silla turca vacía

RESUMEN Paciente femenino de 53 años de edad, que ingreso por cuadro clínico de 10 días de evolución caracterizado por mialgias y edema en extremidades inferiores. Al examen físico: facie abotagada, piel seca, cabello fino, disminución del vello axilar y púbico. En laboratorio se evidencia elevación de enzimas musculares (mioglobina 3000 U/L, CPK 2876 U/L), alteración del Na sérico 112 mEq/L, perfil tiroideo alterado (TSH normal a baja 1,14 UI/mL y FT4 baja 0,08 UI/ml), cortisol AM de 7,2 mcg/dL. Se solicito resonancia magnética con protocolo de silla turca se observa; hipófisis disminuida de tamaño en todos sus diámetros que confirma el hallazgo de un síndrome de silla turca parcialmente vacía. La hiponatremia asociada a hipopituitarismo es poco común.

ABSTRACT A 53-year-old female patient was admitted by a clinical case of 10 days of evolution characterized by myalgia and edema in the lower extremities. On physical examination: facial swelling, dry skin, fine hair, decreased axillary and pubic hair. In the laboratory there is evidence of elevation of muscle enzymes (myoglobin 3000 U/L, CPK 2876 U/L), alteration of serum Na 112 mEq/L, altered thyroid profile (normal to low TSH 1.14 IU/mL and FT4 low 0.08 IU/ml), AM cortisol 7.2 mcg/dL. Magnetic resonance imaging is requested with sella Turcica protocol. It is observed a decreased pituitary gland in all its diameters confirming the finding of a partially empty sella syndrome. Hyponatremia associated with hypopituitarism is uncommon.

Insuficiencia suprarrenal secundaria: una causa subestimada de hiponatremia euvolemica

RESUMEN Introducción: La hiponatremia por insuficiencia suprarenal secundaria es subestimada tratamiento inapropiados. Objetivos: Describir las características clínicas y bioquímicas de pacientes con hiponatremia por insuficiencia suprarrenal secundaria y sus causas. Materiales y Metodos: Revisión retrospectiva de historias clínicas de pacientes consultantes a un hospital de tercer nivel entre Enero 2015 a Septiembre 2017 con hiponatremia y bioquímica de insuficiencia suprarenal secundaria. Los hallazgos fueron comparados con los reportados por estudios previamente publicados. Resultados: Todos los pacientes con insuficiencia suprarrenal secundaria se presentaron con hiponatremia euvolemica hipotónica. 54.5% eran mujeres, la edad promedio fue 57 años. Solo 1 paciente tuvo hiponatremia leve. La mediana de la concentración de cortisol fue 2.8 mcg/dL (RIQ 1.75-3.25 mcg/dL) y la de ACTH fue de 7.7 pg/nL (RIQ 4.5-9.5 pg/nL). Todos los pacientes tuvieron densidad urinaria alta indistinguible del SSIDH. El hipogonadismo hipogonadotrópico y el hipotiroidismo central fueron las alteraciones de ejes hipofisarios mas comúnmente asociados. La presencia de hipoglicemia, hipotensión e hipercaliemia fue baja. La causa más frecuente fue silla turca vacía. Conclusiones: La hiponatremia hipotonica euvolémica es una presentación común de insuficiencia suprarrenal secundaria y no suele acompañarse de otras manifestaciones de deficiencia de glucocorticoides. Es clínica y bioquímicamente indistinguible del SSIDH. Un bajo umbral de sospecha y la medición de cortisol serico matutino es esencial en estos pacientes para evitar un diagnostico y manejo inapropiados.

ABSTRACT Introduction: Hyponatremia due to secondary adrenal insufficiency is frequently underestimated and underdiagnosed. This paper underscores the importance of an adequate evaluation of euvolemic hyponatremia to avoid an inappropriate treatment and diagnosis. Objectives: To describe the clinical and biochemical characteristics of patients with hyponatremia due to secondary adrenal insufficiency and its causes. Materials and Methods: A retrospective review of the clinical records of patients presenting to a third level hospital between January 2015 to September 2017 with hyponatremia and a biochemical profile of secondary adrenal insufficiency. Findings were compared with previously published reports. Results: All patients with secondary adrenal insufficiency presented with hypotonic euvolemic hyponatremia. 54.5% of patients were females, median age was 57 years. Only 1 patient had mild hyponatremia. Cortisol median concentration was 2.8 mcg/dL (IQR 1.75-3.25 mcg/dL) and median ACTH concentration was 7.7 pg/nL (IQR 4.5-9.5 pg/nL). All the patients had high urinary density and features indistinguishable from SIADH. Hypogonadotropic hypogonadism and central hypothyroidism were the most commonly accompanying hypophyseal axis. Hypoglycemia, hypotension, and hyperkalemia were infrequent findings in these patients. The most frequent etiology identified was empty sella syndrome. Conclusions: Euvolemic hypotonic hyponatremia is a common presentation of secondary adrenal insufficiency and is often not accompanied with other manifestations of glucocorticoid deficiency. This disease is clinical and biochemical indistinguishable from SIADH. A low threshold for suspicion and a serum morning cortisol measurement in these patients is essential to avoid an inappropriate diagnosis and management.

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.

The occurrence and characteristics of ectopic pituitary adenoma in China / 中华内分泌外科杂志

Objective To determine the occurrence and clinical characteristics of ectopic pituitary adenoma (EPA) in China.Methods The study are done by searching systematically and comprehensively on major Chinese and English medical literature databases and conference papers before 2015,which are only limited to collected,summarized,sorted and analyzed EPA cases that reported by Chinese authors in English or Chinese occurred in China.Results ① Among the 86 Chinese articles and 27 English articles related to ectopic pituitary adenoma (EPA) gathered by the author,except for cases that have been confirmed as repeated reports,a total of 73 EPA cases were found.Of 70 cases with complete data,31 were male cases,accounting for 44.29％;39 were female cases,accounting for 55.71％;the ratio is 1:1.3.The frequency of EPA occurrence according to the location of the lesion,from high to low in turn,was sphenoid sinus (31 cases,42.47％),nasopharynx (7 cases,9.59％),suprasellar region (7 cases,9.59％),clivus (5 cases,6.85％),followed by the third ventricle,sphenoid sinus/clivus,nasal cavity,and the temporal lobe of the brain,with the same incidence of 4.11％.According to the functional properties of EPA,the frequency of different secreting hormones adenoma was PRL-ma(21 cases,28.77％),NF-ma (21 cases,28.77％),ACTH-ma (15 cases,20.55％),GH-ma (10 cases,13.70％),TSH-ma (2 cases,2.74％) and FSH-ma (1 cases.1.37％).Three cases of EPA were uncertain in their property due to lack of information.The incidence of PRL and nonfunctional tumors was the highest,which was different from what was reported in other countries.Among them,one case of EPA was in pineal region and one in parapharyngeal space,which was even more rare and were never reported abroad.(② Except for 3 cases with incomplete medical records,15 out of 70 cases of EPA were accompanied by empty sella,accounting for 21.43％,among which 11 (73.33％) cases involved the sphenoid sinus,and 3 (20％) cases involved clivus.The sphenoid sinus and clivus cases together accounted for 93.33％.(③ 29 out of the 69 cases of EPA with complete record were invasive pituitary adenomas,accounting for 42.03％ and including 1 case of pituitary adenocarcinoma,which accounted for 1.45％.(④ All cases were treated with surgery as the first choice,and some of them were treated with radiotherapy or drug therapy.Conc lusion Ectopic pituitary adenoma is extremely rare.By the end of 2015,the total number of cases reported in China is only 73,which are mostly located in the sphenoid sinus,suprasellar region and nasopharynx.In the functional classification,the most common types are still PRL adenoma and nonfunctional adenoma as in situ pituitary adenoma.42.03％ of EPA adenomas are invasive.Surgical resection of EPA is the first choice and some cases can be treated with radiotherapy and drug therapy.

Aracnoidocele selar como causa de hiponatremia severa. Presentación de un caso y revisión de la literatura

El síndrome primario de la silla turca vacía o aracnoidocele selar se presenta cuando una de las capas que cubre la parte externa del cerebro protruye hacia abajo en la silla y ejerce presión sobre la hipófisis. En el caso del hipopituitarismo, como causa de aracnoidocele, las manifestaciones clínicas dependen del aumento o disminución de la producción de hormonas, lo que lleva a la aparición de trastornos hidrominerales severos como la hiponatremia. Se presenta el caso de una paciente que acudió al Hospital Dr. Gustavo Aldereguía Lima con cuadro clínico de vómitos, decaimiento, malestar general, pérdida del apetito y ureas escasas. Durante su ingreso, la paciente presentó un cuadro de estado convulsivo que llevó a la ventilación mecánica. Se realizaron estudios tomográficos que fueron negativos y al analizar el resto de los exámenes de laboratorio, se diagnosticó un hipotiroidismo con hiponatremia severa para lo cual se indicó tratamiento. Se realizó una resonancia magnética nuclear donde se determinó un aracnoidocele selar grado III. Se presenta este reporte por la importancia del diagnóstico y tratamiento oportunos de esta entidad.

The primary syndrome of the ¨empty¨ sella turcica or sellar diaphragm herniation appears when one of the layers which cover the outside of the brain protrudes down into the sella and puts pressure on the pituitary. In the case of hypopituitarism, as a cause of arachnoid hernia, clinical manifestations depend on increased or decreased production of hormones, leading to the appearance of severe hydromineral disorders such as hyponatremia. It is presented a case of a patient who came to Dr. Gustavo Aldereguía Lima Hospital with clinical symptoms of vomiting, decay, general malaise, loss of appetite and scarce urination. During admission, the patient had a convulsive status which led to mechanical ventilation. We performed tomography studies that were negative and when analyzing the rest of the laboratory tests, hypothyroidism with severe hyponatremia was diagnosed for which treatment was indicated. A nuclear magnetic resonance was performed where a grade III sellar diaphragm herniation was diagnosed. This report is presented because of the importance of timely diagnosis and treatment of this entity.

A clinical analysis of 123 cases of primary empty sella / 中华内科杂志

Objective This study was conducted to analyze the clinical characteristics and pituitary function of patients with primary empty sella (PES).Methods The clinical data from 123 hospitalized adult patients with PES from January 2010 to May 2016 were retrospectively studied.Results (1) The average age of the 123 (male 43,female 80) PES patients was (59.2 ± 13.6) years (ranging 24-92 years),among whom 61％ patients were in the age group between 50-69 years.(2) The symptoms of the patients included fatigue (56.1％),headache (34.1％),nausea and vomiting (17.9％),gonadal dysfunction (17.1％),visual disturbance (5.7％) and hypopituitarism crisis (3.3％).(3) Hypopituitarism was found in 66 of the 123 patients.Among them,36.6％,31.7％ and 17.1％ were central hypoadrenalism,hypogonadism,and hypothyroidism,respectively.The percentage of hypopituitarism in complete PES was significantly higher than that in partial PES (P ＜ 0.05).(4) Sixteen patients were concomitant with other autoimmune diseases including 11 patients with Graves' disease and 2 with Cushing's syndrome due to adrenal adenoma.Conclusions The incidence of hypopituitarism in PES was 53.7％,in which the pituitary-adrenal axis hypofunction was more common.An overall evaluation of the pituitary function was essential for the patients who had headache and fatigue,or with suspected PES.The patients with hypopituitarism should be given hormone replacement therapy in time and followed up afterword.

Hormonal and radiographic features of primary empty sella / 中华内分泌代谢杂志

Clinical data of 116 cases diagnosed as primary empty sella (PES) from 2012 to 2016 in the Department of Endocrine and Metabolic Diseases, Ruijin Hospital were analyzed in this study. Insulin-induced hypoglycemia test and magnetic resonance imaging (MRI) were applied to explore the predictive factors of hypopituitarism in patients with PES. The results showed that 43 (37. 07%) patients with PES were with hypopituitarism. Pituitary height<2. 35 mm and pituitary sagittal area<23. 5 mm2indicated a high risk of hypopituitarism in patients with PES.

Approach to the patient with primary adrenal lymphoma combined with empty sella / 中华内分泌代谢杂志

[Summary] To discuss the clinical features and treatment of primary adrenal lymphoma( PAL) with empty sella(ES).The lymphoma of the patient was nonspecific,the levels of serum sodium, cortisol, adrenocorticotropic hormone, and gonadotropin were decreased.MRI confirmed ES, CT showed bilateral adrenal enlargement.CT-guided fine-needle aspiration biopsy of the adrenal gland revealed a cytological diagnosis of diffused large B cell lymphoma( DLBCL).The patient had received R-CHOP chemotherapy, which results in a complete functional recovery and the tumoral masses disappeared.PAL combined with ES was rarely reported, which is difficult to diagnose due to complex hormone levels.

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 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.

Quantitative Measurement of the Sella Turcica in Pseudotumor Cerebri

PURPOSE: In this study we evaluated the hypothesis that sella turcica enlarged in size due to increased intracranial hypertension by measuring the sella turcica area using magnetic resonance imaging (MRI) in patients with increased intracranial hypertension and compared to normal controls. METHODS: Brain magnetic resonance (MR) midsagittal images of patients diagnosed with pseudotumor cerebri from 2005 to 2012 at Dankook University Hospital and 10 normal controls who had no overt signs or symptoms of neurological disease and had normal gadolinium-enhanced MR examination of brain were compared. The area of the sella turcica was measured by the double-blind method using Dicomworks v 1.3.5b (Philippe Puech and Loic Boussel, Freeware, France). Statistical analysis was conducted using GraphPad Prism (GraphPad Software, Inc., USA) and Mann-Whitney U-test. RESULTS: The sella turcica areas of 2 pseudotumor cerebri patients were 93 mm2 and 123 mm2 and were significantly larger than in the controls (p = 0.03). CONCLUSIONS: Empty sella which commonly occurs in pseudotumor cerebri can be caused by pituitary gland atrophy but, conversely, can result from the enlargement of the bony sella in response to an abnormal cerebrospinal fluid pressure gradient.

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.

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.

Manejo quirúrgico del síndrome de silla turca vacía primaria con compromiso visual campimétrico y sin evidencia de herniación del sistema visual / Surgical treatment of primary empty sella syndrome with visual compromise and no evidence of visual system herniation

Presentamos el caso de una mujer de 36 años con síndrome de silla turca vacía primaria (STVP) caracterizado por cefalea, estrechamiento concéntrico periférico progresivo de la visión y oligomenorrea, quien fue sometida a remodelamiento selar con colocación de un autoinjerto intraselar. La evolución postoperatoria fue con mejoría importante del defecto campimétrico, en ambos ojos.

We report the case of a 36 year old woman with primary empty sella syndrome (PESS) and symptoms consisting in headache, progressive concentric peripheral narrowing of vision and oligomenorrhea, who underwent sellar remodeling with placement of an intrasellar autograft. Post operative course showed bilateral improvement in campimetric defect.

Silla turca vacía primaria (STVP): estudio de 117 casos / Primary Empty Sella (PES): A review of 117 cases

Introducción: El término Silla Turca Vacía Primaria (STVP) hace referencia a la invaginación del espacio subaracnoideo hacia el interior de la silla turca en pacientes sin antecedentes de tumor, cirugía o radioterapia de la región selar. Aunque usualmente no está asociado con disfunciones endocrinas, diferentes grados de hipopituitarismo e hiperprolactinemia han sido reportados. Objetivo: Analizar retrospectivamente datos clínicos, hallazgos radiológicos y bioquímicos de 117 pacientes con diagnóstico de STVP. Pacientes y Métodos: Se estudiaron 117 pacientes, 98 mujeres (48 ± 14.9 años). Los diagnósticos fueron realizados por Resonancia Magnética Nuclear (n=115) y Tomografía Computada (n=2). La evaluación de la función adenohipofisaria se realizó a través de determinaciones hormonales basales. Resultados: Los motivos que llevaron al pedido de las imágenes fueron: cefaleas (35 %), sospecha clínica y/o bioquímica de deficiencia pituitaria (22 %), trastornos visuales (11 %), anormalidades de la radiografía simple de la silla turca (11 %), hiperprolactinemia (2,6 %), otros (18.4 %). El 48,9 % de las mujeres eran multíparas. Cefaleas, obesidad, hipertensión arterial y autoinmunidad tiroidea fueron halladas en el 60, 67, 24,5 y 22,5 % de la población evaluada respectivamente. Hiperprolactinemia (< 50 ng/ml) estuvo presente en 6,1 % de las mujeres y 15, 8 % de los hombres. El 27 % de los pacientes estudiados presentó algún grado de hipopituitarismo, que fue más frecuente en la población masculina. Conclusiones: STVP fue más frecuente en mujeres multíparas de mediana edad. En la mayoría de los casos fue descubierta incidentalmente por estudios radiológicos, mientras que en un cuarto de los pacientes, fue encontrada durante la evaluación diagnóstica de deficiencia adenohipofisaria, lo cual fue más frecuente en hombres.

Introduction: The term Primary Empty Sella (PES) makes reference to the herniation of the subarachnoid space within the sella turcica in those patients with no history of pituitary tumor, neither surgery, nor radiotherapy. Though it is usually not associated with endocrine abnormalities, different degrees of hypopituitarism and mild hyperprolactinemia have been reported. Objective: To assess clinical features, radiological findings and biochemical endocrine function retrospectively from the records of 117 patients with diagnosis of PES. Patients and Methods: One hundred seventeen patients, 98 females, were studied. The mean age at diagnosis was 48 ± 14.9 yr. Most diagnoses were made with magnetic resonance imaging (n = 115), and only 2 through sellar computed tomography scan. Only pituitary basal hormones determinations were made, except for the TRH and ACTH tests which were performed for the diagnosis of primary hypothyroidism and secondary adrenal failure respectively. Results: Pituitary images were requested because of different reasons: headaches (35 %), clinical and biochemical suspicion of pituitary deficiency (22 %), visual disturbances (11 %), abnormalities on the simple sella turcica radiography (11 %) hyperprolactinemia (2.6 %), others (18.4 %): dizziness, seizures, rhinorrhea, loss of consciousness, skull trauma, galactorrhea. Multiple pregnancies were observed in 48.9 % of women; headaches, obesity, arterial hypertension and thyroid autoimmunity were found in 60 %, 67 %, 24.5 % and 22.5 % of the studied population respectively. Mild hyperprolactinemia (< 50 ng/ml) was present in 6.1 % of women and 15.8 % of men. Twenty seven percent of our patients had some degree of hypopituitarism. For male population hypopituitarism comprised 72 %, whereas it took up 19 % for the whole female group. Conclusions: PES seems to be more commonly found in middle-aged women (sex ratio 5/1) with history of multiple pregnancies. In most patients it was discovered as an incidental finding at image studies, while in almost a quarter of patients PES was found during the diagnosis stage of anterior pituitary deficiency, which was more frequently seen among men.

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.

Comparative study of cerebrospinal fluid flow in the cerebral aqueduct between empty sella syndrome and normal volunteer / 中国医师进修杂志

Objective To compar the cerebrospinal fluid (CSF) flow between empty sella syndrome (ESS) and normal volunteer in the cerebral aqueduct with MRI in phase contrast cine mode. Methods Thirty-eight ESS patients (ESS group) and 38 normal volunteers (control group ) were involved in this study.The aqueduct CSF flow image was positioned perpendicularly to the midbrain aqueduct at the middle sagittal T1WI or T2WI image. The waveforms were analyzed for the flow direction, flow rate, flow volume rate and cardiac cycle. Results The CSF flow of the aqueduct in control group and ESS group had two directions which was downward flow during the systolic period and upward flow during the diastolic period of the cardiac cycle. The.systolic period downward peak flow rate, diastolic period upward peak flow rate, mean downward flow rate, mean upward flow rate and mean flow rate were (5.231 ± 0.262), (4.902 ± 0.281 ),(3.083 ± 0.191 ), (3.032 ± 0.151 ), (3.151 ± 0.162) cm/s in control group, and (6.244 ± 0.356), (6.091 ±0.430), (3.916 ± 0.196), (3.812 ± 0.273 ), (3.690 ± 0.291 ) cm/s in ESS group respectively,and there was no significant difference between the two groups ( P ＞ 0.05 ). The systolic period downward peak flow volume rate, diastolic period upward peak flow volume rate, mean downward flow volume rate,mean upward flow volume rate and mean flow volume rate were (0.050 ± 0.003 ), (0.050 ± 0.004), (0.030± 0.002), (0.031 ±0.002), (0.030 ± 0.003 ), ( 0.004 ± 0.001 )ml/s in control group, and (0.058 ± 0.003 ), (0.063 ± 0.005),(0.039 ±0.002), (0.038 ±0.003), (0.038 ±0.003), (0.004 ±0.001) ml/s in ESS group respectively,and there was no significant difference between the two groups(P ＞ 0.05 ). The correspond cardiac cycle of systolic period downward peak flow rate, correspond cardiac cycle of diastolic period upward peak flow rate, mean cardiac cycle were (40.890 ± 37.096), (501.026 ± 19.374), (719.511 ± 14.946) ms in control group,and (35.921 ±6.218), (531.553 ± 16.764), (770.700 ±21.579) ms in ESS group,and there was no significant difference between the two groups (P ＞ 0.05 ). Conclusion Part of CSF flows into the area of saddle in ESS patients, but it has no effect on CSF indexes in area of cerebral aqueduct.

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.