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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.
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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.2-5 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 al3 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.
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Síndrome da Sela Vazia/cirurgia , Microcirurgia/métodos , Procedimentos Neurocirúrgicos/métodos , Procedimentos de Cirurgia Plástica/métodos , Sela Túrcica/cirurgia , Aracnoide-Máter/diagnóstico por imagem , Transplante Ósseo , Síndrome da Sela Vazia/diagnóstico por imagem , Humanos , Imageamento Tridimensional , Imageamento por Ressonância Magnética , Sela Túrcica/diagnóstico por imagemRESUMO
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.
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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.
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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.
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Humanos , Feminino , Idoso , Neoplasias Hipofisárias/complicações , Acromegalia/complicações , Acromegalia/diagnóstico , Síndrome da Sela Vazia/complicações , Sela Túrcica/patologia , Fator de Crescimento Insulin-Like I/análise , Hormônio do Crescimento/análise , Imageamento por Ressonância Magnética , Teste de Tolerância a GlucoseRESUMO
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.
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Brain and optic chiasm herniation has been rarely reported following dopamine agonist treatment for large prolactinomas. We report a case of brain and optical chiasm herniation, secondary to an empty sella due to apoplexy of a prolactinoma, and we focus on the specific presentation of this case. A 32-year-old female presented to a neurologist complaining of headaches. Her past medical history was significant for acute vision loss in both eyes accompanied by right third nerve palsy when she was 16 years old. She does not recall any endocrine or imaging evaluation at that time and she had spontaneous partial recovery of left eye vision within 3 months, with permanent blindness of right eye. She did not return to any follow-up until her neurologist consultation. Brain magnetic resonance imaging (MRI) revealed herniation of frontal lobe and optic chiasm into the pituitary sella, as well as a pituitary hypointense lesion measuring 5 mm × 5 mm after gadolinium injection. Prolactin levels were 206 ng/ml (4.79-23.3 ng/ml). Repeated prolactin was 258 ng/ml (4.79-23.3 ng/ml). She was started on bromocriptine 2.5 mg/day. Prolactin levels and menstrual cycles normalized. A repeat brain MRI performed 5 months later showed disappearance of pituitary mass, with no changes in brain and chiasmal herniation. To our knowledge, this is the first reported case of brain associated with chiasm herniation secondary to pituitary apoplexy of a prolactinoma. In conclusion, this case highlights that frontal lobe herniation in combination with optic chiasm herniation can be a complication of pituitary tumor apoplexy. Long-term surveillance of patients with pituitary apoplexy is warranted to detect delayed complications.
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BACKGROUND: Primary empty sella is a herniation of the sellar diaphragm into the pituitary space. It is an incidental finding and patients may manifest neurological, ophthalmological and/or endocrine disorders. Episodes of vertigo, dizziness, and hearing loss, have been reported. OBJECTIVE: To determine the conditional probability, as well as the statistical dependency, through the Bayesian analysis in patients with primary empty sella and audiovestibular disorders. PATIENTS: Individuals who attended the National Rehabilitation Institute from January 2010 to December 2011, diagnosed with primary empty sella and audiovestibular disorders. MATERIAL AND METHODS: An analysis was performed on a sample of 18 patients with a diagnosis of primary empty sella confirmed with magnetic resonance studies and who had signs of vertigo, hearing loss and dizziness. RESULTS: Of the 18 patients studied, 3 (16.66%) had primary empty sella as the only clinical evidence. In 9 patients (50%) empty sella was associated with vertigo, and 16 patients (88.88%) were diagnosed with hearing loss, with sensorineural hearing loss being the most frequent (77.77%). The intersection between the proportions of primary empty sella with the presence and type of hearing loss was calculated. Thus for sensorineural hearing loss, the calculated ratio was P(AB)=0.6912, and for conductive and mixed hearing loss the value of P(AB)=0.0493 in both cases. CONCLUSIONS: Bayesian analysis and conditional probability enables the dependence between two or more variables to be calculated. In this study both mathematical models were used to analyse comorbidities and audiovestibular disorders in patients diagnosed with primary empty sella.
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Síndrome da Sela Vazia/epidemiologia , Perda Auditiva/epidemiologia , Vertigem/epidemiologia , Adulto , Idoso , Aracnoide-Máter/patologia , Teorema de Bayes , Comorbidade , Estudos Transversais , Diplopia/epidemiologia , Diplopia/etiologia , Síndrome da Sela Vazia/patologia , Doenças do Sistema Endócrino/epidemiologia , Doenças do Sistema Endócrino/etiologia , Feminino , Cefaleia/epidemiologia , Cefaleia/etiologia , Humanos , Hipertensão/epidemiologia , Hipopituitarismo/epidemiologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Sobrepeso/epidemiologia , Hipófise/patologia , Estudos de AmostragemRESUMO
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...
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Humanos , Feminino , Pessoa de Meia-Idade , Síndrome da Sela Vazia , Hipopituitarismo/diagnóstico , Hipopituitarismo/terapiaRESUMO
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.
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BACKGROUND: Pituitary adenomas are a common cause of endocrinal dysfunction, which comprise 10-20% of all intracranial tumors. Although almost all of them arise within the sella turcica, there are some rare cases in which a pituitary adenoma is located outside the intrasellar region, so it is defined as an ectopic pituitary adenoma (EPA). CASE DESCRIPTION: We described a case of a 31-year-old male with a serum prolactin (PRL) value of 240 ng/ml Magnetic resonance imaging (MRI) showed a space-occupying mass within the sphenoid sinus (SS) which partially enhanced by gadolinium. MRI did not reveal any sellar floor defect and an empty sella was detected. As dopamine agonist treatment had failed in lowering the serum PRL level, he underwent surgical treatment. A transsphenoidal approach without opening the sellar floor was performed using an operating microscope and the lesion within the SS was completely removed. CONCLUSION: Although intrasphenoidal EPAs are rare findings, the presence of an endocrine disorder related to pituitary hormones, and a space-occupying mass within the SS associated with either a normal sellar pituitary gland or an empty sella must lead us to suspect this diagnosis.
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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.
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Antecedentes: La silla turca vacía es una entidad que sólo en raras ocasiones presenta signos y síntomas, dentro de los cuales la afección en el campo visual es una indicación para el manejo quirúrgico. Materiales y Metodos: Se analizaron 20 pacientes con silla turca vacía primaria y alteraciones en los campos visuales, que fueron manejados quirúrgicamente con una técnica que denominamos remodelación selar. Fueron 19 mujeres y un hombre; todos ellos, además del déficit visual presentaban cefalea y tres casos elevación en el nivel sérico de prolactina. Se excluyeron los que mostraron aumento en la presión del líquido cefalorraquídeo. El procedimiento quirúrgico consistió en la colocación, por vía transesfenoidal, de un injerto autólogo formado por grasa, aponeurosis y dos láminas de hueso, con dimensiones precisas de acuerdo al tamaño de la silla turca del paciente. Resultados: Con la cirugía se logró mejorar el déficit visual en 18 pacientes y la cefalea en 17; finalmente, dos de ellos normalizaron su nivel de prolactina. No se presentaron complicaciones serias. Conclusiones: La remodelación selar es una técnica precisa, sencilla, segura y barata que permite mejorar los síntomas del síndrome de la silla turca vacía primaria, en especial las alteraciones visuales y la cefalea.
BACKGROUND: The empty sella is an entity that only rarely presents signs and symptoms. When noted, visual field deficits are an indication for surgical management. MATERIAL AND METHODS: We studied twenty patients with primary empty sella and visual field deficits surgically treated with a technique termed by us as [quot ]sellar remodeling.[quot ] We treated 19 females and 1 male. Aside from visual deficits, all participants reported headache. We reported an increase in prolactin serum level in three cases. Patients with an increase in cerebrospinal fluid pressure were excluded. The surgical procedure involved placing through a transsphenoidal route an autologus graft formed by fat, aponeurosis and two bone lamina, with precise dimensions according to each patient's sella turcica. RESULTS: After surgery, visual deficits improved in 18 patients and headache in 17. Two patients displayed normal prolactin levels. No serious complications were reported during surgery. CONCLUSIONS: Sellar remodeling is a precise, simple, safe and inexpensive technique that significantly improves symptoms such as visual deficits and headache observed in primary empty sella syndrome.
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Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Síndrome da Sela Vazia/cirurgia , Transplante Ósseo , Procedimentos Cirúrgicos Operatórios/métodos , Tecido Adiposo/transplanteRESUMO
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.