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.

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.

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 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.

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.

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.

Isolated adrenocorticotropic hormone deficiency associated with empty sella syndrome / 대한내과학회지

Isolated adrenocorticotropic hormone (ACTH) deficiency is an uncommon disorder for which the pathogenetic mechanism has not yet been identified. It has been reported that isolated ACTH deficiency (ICD) may be accompanied by deficiencies in other pituitary hormones; impaired growth hormone (GH) secretion was noted in 20 to 30% of ICD patients. Here, we describe a female patient with isolated ACTH deficiency accompanied by empty sella syndrome presenting as hypoglycemia, which was confirmed via various endocrine tests and magnetic resonance imaging (MRI) of the sella turcica. The patient's symptoms improved rapidly with prednisolone therapy and, during follow-up, her previously impaired GH response to provocative stimuli and high TSH levels were corrected by glucocorticoid replacement alone. However, treatment failed to normalize plasma IGF-1 levels, suggesting that physiological cortisol levels are necessary for a normal plasma GH response to provocative stimuli.

A Case of Empty Sella Syndrome with Cerebrospinal Fluid Rhinorrhea / 대한이비인후과학회지

The empty sella syndrome is defined as the intrasella herniation of the chiasmal cistern resulting from a congenitally incompetent diaphragma sellae. In idiopathic or primary empty sella syndrome, sella remodelling occurs as a result of an anatomical variation in the diaphragma sella. The secondary empty sella syndrome occurs following surgery or irradiation of an intrasellar lesion. Empty sella syndrome is known to be rarely associated with Cerebrospinal fluid (CSF) rhinorrhea. We experienced a case of secondary empty sella symdrome with CSF rhinorrhea in a 56-year old female who complained of persistent rhinorrhea through the roof of the sphenoid sinus after brain surgery due to meningioma. The CSF rhinorrhea was treated successfully with transseptal trans-sphenoidal approach.

Amenorrea primaria-galactorrea asociada a hiperpro, lactinemia con síndrome de silla turca vacia primario

Objetivo: Presentar un caso clinico de una paciente con amenorrea primaria-galactorrea e hiperprolactinemia asociada al síndrome de Silla Turca Vada (SSTV). Metodos: Se presentan los hallazgos clínicos, radiológicos y de laboratorio y se hace revisión de la literatura. Resultados: Paciente femenina de 16 años evaluada por amenorrea primaria y galactorrea, con desarrollo normal de caracteres sexuales secundarios a los 12 años y desarrollo esquelético normal. Los resultados de laboratorio fueron consistentes con hipogonadismo hipogonadotrópico, función tiro idea y adrenal normal y valores elevados de prolactina. La exploración neuroradiológica (Resonancia Magnética, Neumoencefalografia) revelo una silla turcavada, quiste aracnoideo con 60% de ocupación de la fosa sellar, glandula hipofisiaria lateralizada a la derecha con características normales. Después del tratamiento con Bromocriptina (5 mg/dia), la concentración serica se redujo seguido del inicio de menstruadones espontaneas y regulares. Conclusion: El SSTVen la edad prepuberal puede estar asociado con hiperprolactinemia y amenorrea primaria.

Objective: To report a case of primary amenorreagalactorrhea and hyperprolactinemia associated to Empty Sella Turcica syndrome. Methods: Clinical, neuroradiologic and laboratory findings are presented and the literature is reviewed. Results: A16-year-old female adolescent was evaluated for primary amenorrhea, galactorrhea. Secondary sex characters were present at 12 years old with normal physical growth. Laboratory findings were consistent with hypogonadotropic hypogonadism, normal thyroid and adrenal function and high plasma levels of prolactin.Pneumoencephalography and magnetic resonance imaging revealed primary empty sella syndrome; the sella turcica was occupied by a 60% arachnoid cyst and a pituitary gland partially flattened at the right side of the sella turcica. After initiation ofbromocriptine therapy (5mg/ day), the serum prolactin level was reduced, followed by spontaneous and regular menses. Conclusion: The empty sella syndrome turcica in prepuberal girls may be associated with hyperprolactinemia and primary amenorrhea-galactorrhea.

A case of primary empty sella syndrome combined with Cushing's disease / 대한내과학회지

Empty sella is defined as a sella which, regardless of its size, is completely or partly filled with cerebrospinal fluid (CSF), and is divided into two groups, primary or secondary. Secondary empty sella syndrome is associated with a prior surgery, radiation therapy or medical treatment of pituitary gland, and otherwise primary. The endocrine function of primary empty sella syndrome is usually normal, but sometimes, this syndrome is associated with complete or partial pituitary insufficiency and rarely pituitary hypersecretion. In the cases of hypersecretion of pituitary hormones, hyperprolactinemia, acromegaly and hypersecretion of adrenocorticotrophin (ACTH) has been reported. but, hypersecretion of ACTH is rarely reported. We describe a patient of primary empty sella syndrome combined with Cushing's disease. So we present this case with a review of the literature.

A Case of Primary Empty Sella Syndrome Associated with Visual Symptom

Empty sella syndrome shows partial or total loss of pituitary tissue and enlargement of the sella turcica due to incomplete diaphragm sellae and herniation of subarachnoid space through the defect. This syndrome usually occurs in obese, hypertensive, and cephalgic women, but it is often asymptomatic. We report a case of primary empty sella syndrome diagnosed by magnetic resonance imaging scan with literature review in 57-year-old female patient who had had unilateral cataract operation and presented with bilateral visual disturbance and bitemporal hemianopsia at postoperative 2 months.

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

The empty sella syndrome is applied to the abnormal extension into the sella turcica of an arachnoid diverticulum filled with CSF, which displace and compresses the pituitary gland. Such a diverticulum can erode the sellar floor and lead to CSF rhinorrhea through the sphenoid sinus. Recently we experienced a case of the spontaneous CSF rhinorrhea associated with the empty sella syndrome in a 55 year-old woman. The CSF leakage was repaired successfully by sublabial, transseptal, transsph-enoidal approach.

Primary empty sella syndrome / 대한내분비학회지

BACKGROUND: Primary empty sella syndrome (PES) is thought to arise from an incompetent diaphragma allowing progressive herniation of arachnoid membrane with secondary compression and atrophy of the pituitary gland. As a consequence of the improvement and widespread use of neuroradiological techniques, such as computerized tomography (CT) and magnetic resonance imaging (MRI), empty sella is more frequently disclosed. The aim of this study is to assess the associated clinical characteristics and endocrinologic disturbance in empty sella syndrome. METHODS: From January 1986 to June 1996, 171 patients with empty sella syndrome have undergone analysis for clinical characteristics and associated disease. RESULT: In our study, PES was diagnosed in 131 of the 171 patients (77%). Primary empty sella syndrome was frequent in middle aged women (female:male 115:16, mean age: 50.6+12.6 years). The common clinical features were headache (80.2%), obesity (72.5%), and hypertension (27.5%). Most of patients with PES have normal pituitary function (75%). The frequent pituitary dysfunction was hyperprolactinemia in PES (21%). Partial and total emptiness of sella on sella CT or MRI were in 111 (84.7%) patients, and in 20 (15.4%) patients, respectively. The most common associated disease with empty sella syndrome was pituitary adenoma. CONCLUSION: PES should be considered as a possible cause in obese middle aged women with unexplained headache. The combined pituitary function test should be considered for evaluation of pituitary dysfunction when clinically suspected.

Two Cases of Graves Disease Associated The Empty Sella Syndrome / 대한내분비학회지

The empty sella syndrome is characterized by obesity, frequent pregnancy, headache and high blood pressure, but its exact cause remains unknown. Usually the incomplete diaphragmatic sella has been considered as the cause of the empty sella syndrome, but some authors recently have suggested that the antipituitary antibody way be related to development of pituitary atrophy and the pituitary empty sella syndrome, and thus it may be clinically useful as screening test for the empty sella syndrome. We experienced two empty sella syndromes associated Graves disease and applied the antipituitary antibody as the diagnostic tool of the empty sella syndrome. But none of this two patients had antipituitary antibody and we report these cases with reviews of literatures.