Tumor de hipófise em paciente com hidrocefalia: um desafio diagnóstico / Pituitary tumor in a patient with hydrocephalus: a diagnostic challenge

RESUMO A hidrocefalia é definida como a dilatação ventricular pelo aumento da pressão intraventricular e intracraniana quando não tratada ou por insucesso do tratamento. Muitas vezes, leva ao dano das vias ópticas, podendo causar atrofia óptica, devido à proximidade dessas vias com o ventrículo lateral quando ocorre a dilatação. Assim como a hidrocefalia pode levar à atrofia óptica, outras patologias também podem. Tumores hipofisários compartilham desse mesmo sinal, além de causar hemianospsia bitemporal quando o tumor comprime quiasma óptico. Ademais, a hemianopsia bitemporal é o distúrbio visual mais comum encontrado em pacientes com tumor de hipófise. Os tumores de hipófise, por exemplo, geram manifestações clínicas que podem estar relacionadas à disfunção da glândula ou aos efeitos mecânicos da expansão tumoral. Sinais e sintomas visuais estão mais ligados ao efeito mecânico do tumor. Assim, muitas vezes, o paciente procura o oftalmologista antes do endocrinologista. Neste caso, analisaremos uma paciente portadora de hidrocefalia que apresentava, concomitantemente, um tumor hipofisário, e a investigação oftalmológica fez toda a diferença no tratamento da paciente.

ABSTRACT Hydrocephalus is defined as ventricular dilation caused by increased intraventricular and intracranial pressure when untreated or due to treatment failure. Optical pathways can often cause optic atrophy due to the proximity to the lateral hazard when dilation occurs. Hydrocephalus can lead to optic atrophy, as well as other pathologies. Pituitary tumors share this same sign, in addition to causing bitemporal hemianopia when it compresses the optic chiasm. In addition, bitemporal hemianopia is the visual disturbance most commonly found in patients with pituitary tumors. Pituitary tumors, for example, have clinical manifestations that may be related to gland dysfunction, or to mechanisms of tumor expansion. Visual signs and symptoms are more linked to the mechanical effect of the tumor. Therefore, the patient usually seeks the ophthalmologist before the endocrinologist. In this case, we analyzed a patient with hydrocephalus who presented, at the same time, a pituitary tumor, and the ophthalmological investigation made all the difference in the treatment of the patient.

Resolution of headache after reduction of prolactin levels in hyperprolactinemic patients / Resolução de cefaleia após redução dos níveis de prolactina em pacientes com hiperprolactinemia

Abstract Prolactin (PRL) secreting adenomas are associated with high incidence of headache. The role of hyperprolactinemia in the headache context is not clear, nor is the effect of its treatment on headache. Methods: The present longitudinal study evaluated hyperprolactinemic patients (69), in terms of presence and characteristics of headache before and after hyperprolactinemia treatment. Results: Headache was reported by 45 (65.2%) patients, independent of the etiology of hyperprolactinemia. The migraine phenotype was the most prevalent (66.6%). Medications used in the treatment of headache not changed during the study. The first line of treatment of hyperprolactinemia was dopaminergic agonists. In the last reevaluation, PRL level under treatment was within the reference range in 54.7% of the cases, and it was observed complete or partial resolution of the headache in 75% of the cases. The median PRL at this time in patients with complete headache resolution was 17 ng/mL, in those who reported partial recovery was 21 ng/mL, and in those in whom the headache did not change was 66 ng/mL, with a significant difference between the group with complete headache resolution vs. the group with unchanged headache (p=0.022). In the cases with complete headache resolution, the median fall on PRL levels was 89% and in those cases with partial headache resolution 86%, both significantly different (p<0.001) from the fall in the cases with an unchanged headache. Conclusion: Data allow us to conclude that, in this series, in the majority of cases the reduction in the level of PRL was followe3d by cessation or relief of the pain.

Resumo Os adenomas secretores de prolactina (PRL) estão associados à alta incidência de cefaleia. O papel da hiperprolactinemia no contexto da dor de cabeça não está claro, nem o efeito da redução dos níveis da PRL na cefaleia. Métodos: O presente estudo longitudinal avaliou pacientes hiperprolactinêmicos (69), quanto à presença e às características da cefaleia antes e após o tratamento da hiperprolactinemia. Resultados: Cefaleia foi relatada por 45 (65,2%) pacientes, independente da etiologia da hiperprolactinemia. O fenótipo de enxaqueca foi mais prevalente (66,6%). Os medicamentos usados ​​no tratamento da cefaleia não foram alterados durante o estudo. A primeira linha de tratamento da hiperprolactinemia foram os agonistas dopaminérgicos. Na última reavaliação, o nível de PRL sob tratamento estava dentro da faixa de referência em 54,7% dos casos, observando-se resolução completa ou parcial da cefaleia em 75% dos casos. A mediana de PRL neste momento em pacientes com resolução completa da cefaleia foi de 17 ng/mL, nos que relataram recuperação parcial foi de 21 ng/mL, e naqueles em que a cefaleia não se alterou foi de 66 ng/mL, com uma diferença significativa entre o grupo com resolução completa da cefaleia versus o grupo com cefaleia inalterada (p=0,022). Nos casos com resolução completa da cefaleia, a queda mediana nos níveis de PRL foi de 89% e nos casos com resolução parcial de cefaleia de 86%, ambos significativamente diferentes (p<0,001) da queda nos casos com cefaleia inalterada. Conclusão: Os dados permitem concluir que, nesta série, na maioria dos casos, a redução do nível de PRL foi seguida pela cessação ou alívio da dor.

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.

Adenomas no funcionantes: análisis retrospectivo de 202 pacientes / Non-functional adenomas: retrospective analysis of 202 patients

RESUMEN Los pacientes con adenomas hipofisarios constituyen una población heterogénea y requieren un enfoque individualizado. El objetivo de nuestro trabajo fue analizar nuestra población con adenomas hipofisarios no funcionantes (ACNF) y evaluar factores pronóstico de crecimiento (como el Ki-67) que ayuden en la toma de decisiones. Se realizó un análisis retrospectivo de 202 pacientes, incluyendo evaluación basal, enfoque terapéutico y evolución tumoral en 2 grupos: pacientes con conducta expectante (n = 69) y pacientes con cirugía (n = 133). La serie tuvo 55% de pacientes mujeres y la edad media al diagnóstico fue de 49 años. Los motivos de consulta más frecuentes fueron incidentaloma hipofisario y alteraciones visuales. Radiológicamente, 83% fueron macroadenomas, 77% invasivos y 55% mostraron compromiso visual. Entre los adenomas invasores, el 53% tenían disfunción hipofisaria, siendo el hipogonadismo el hallazgo más frecuente. El tratamiento inicial fue la cirugía en el 65,8% realizándose por vía transnasal en el 79% de los casos. Las complicaciones más frecuentes fueron diabetes insípida transitoria e hiponatremia, con mayor incidencia de diabetes insípida permanente en la cirugía transcraneal. La inmunohistoquímica mostró gonatropinomas en el 43,4% de los casos y fue negativa en el 37,7%. Doce adenomas tuvieron índice de proliferación Ki-67 ≥3%. Luego de la cirugía 56,8% de los pacientes mejoraron el campo visual, 22,6% recuperó alguna función endocrina y 18,8% agregó un nuevo déficit. En pacientes no operados, se observó crecimiento tumoral en 5,6% de los adenomas Hardy 1-2 y en el 21% de los Hardy 3-4. Entre los adenomas operados, aquellos sin resto tumoral postoperatorio no presentaron recurrencia. De los tumores con remanente postoperatorio (78,6%) no irradiados, el 41,5% mostró recrecimiento lesional al seguimiento. Este porcentaje se eleva a 66,6% en aquellos con Ki-67 ≥3% y disminuye a 12% en los que recibieron radioterapia.

ABSTRACT Patients with pituitary adenomas are a heterogeneous population and require an individualized approach. The aim of our study was to analyze our population of patients with nonfunctioning pituitary adenomas (NFA) and to evaluate prognostic growth factors (such as Ki-67) that help in decision making. A retrospective analysis of 202 patients, including baseline assessment, therapeutic approach and tumor evolution was performed in 2 groups: expectant management (n = 69) and surgery (n = 133). The mean age at diagnosis was 49 years, 55% women. The most frequent reasons for consultation were pituitary incidentaloma and visual impairment. Eighty three percent were macroadenomas, 77% invasive, and 55% with visual impairment. Among the invasive adenomas, 53% had pituitary dysfunction, with hypogonadism being the most frequent finding. The initial treatment was surgery in 65.8%, 79% of them through transnasal approach. The most frequent complications were transient diabetes insipidus and hyponatremia, with a higher incidence of permanent diabetes insipidus in transcranial surgery. The immunohistochemistry showed: 43.4% gonadotropinomas, 37.7% negative. Twelve adenomas had proliferation index Ki-67 ≥3%. After surgery, 56.8% improved the visual fields, 22.6% recovered some endocrine function and 18.8% added a new deficit. In non-operated patients, tumor growth was observed in 5.6% of the Hardy 1-2 adenomas and 21% of the Hardy 3-4 adenomas. Among the operated adenomas, those without postoperative tumor residue did not present recurrence. In tumors with non-irradiated postoperative remnant (78.6%), 41.5% increased. This percentage rises to 66.6% in those with Ki-67 ≥3%, and decreases to 12% in those who received radiotherapy.

Acromegaly: a rare disease?

Acromegaly is generally considered a benign and uncommon disease. However, some recent data bring support to the idea that it is more frequent than previously thought. Besides, acromegaly can significantly shorten the length of life due to its cardiovascular and metabolic complications. Since its clinical signs are insidiously progressive for many years, there is a considerable delay in its detection. Usually, many different specialists have been consulted before reaching diagnosis of acromegaly. Those specialists include cardiologists, pulmonologists, dentists, rheumatologists, and diabetes specialists. Possible means to achieve earlier detection are based on increasing awareness of doctors and the public in general. In this paper, the author analyzes the factors related to delayed diagnosis and the potential ways to ameliorate awareness of the disease with particular attention to screening procedures.

Existe la idea generalizada de que la acromegalia es una enfermedad benigna e infrecuente. Sin embargo, el paciente acromegálico ve comprometida su vida a causa de complicaciones cardiovasculares y metabólicas. Por otra parte, trabajos recientes muestran que su frecuencia parece mucho mayor que lo supuesto previamente. Dado que los signos y síntomas de la enfermedad se instalan lenta e insidiosamente, existe una demora considerable en su diagnóstico. Habitualmente, los pacientes han consultado diversos especialistas antes de que el trastorno sea detectado. Los mismos incluyen cardiólogos, neumonólogos, odontólogos, reumatólogos y diabetólogos. Un camino posible para lograr una detección temprana es el incremento del grado de concientización de los médicos y de la comunidad. En este artículo se analizan los factores vinculados al retraso diagnóstico y los medios posibles para mejorar el conocimiento y detección precoz de la enfermedad.

Caracterización de familia hondureña afectada por neoplasia endócrina múltiple (NEM-2A), Instituto Nacional Cardiopulmonar, Tegucigalpa, Honduras / Characterization of Honduran family affected by Multiple Endocrine Neoplasia (MEN-2A), Instituto Nacional Cardio-Pulmonar, Tegucigalpa, Honduras

RESUMEN. Introducción . Las Neoplasias Endocrinas Múltiples (NEM) son síndromes clínicos autosómico-dominantes. Se maniies - tan por tumores benignos y malignos que involucran órganos endócrinos y no endócrinos. Incidencia de 1/30,000 personas. Objetivo : Caracterizar una familia hondureña cuyos miembros presentan tumores endócrinos, Instituto Nacional Cardio-Pulmonar (INCP), Tegu - cigalpa, 2017. Métodos . Estudio descriptivo longitudinal. Análisis de información clínica, epidemiológica y de laboratorio, consignada en expedientes clínicos (INCP) y brindada por pacientes, previo consentimiento informado. Conirmación de dos casos mediante estudios genéticos. Resultados . Familia de 9 hijos, procedente de Olancho. Madre falleció por Adenocarcinoma Pancreático; padre sin información. Hija3, caso índice, se le realizó Tiroidectomía Total por Cáncer Medular de Tiroides (CMT). Hijo2 no evaluado. Hija4 portadora asintomática de la mutación del proto-oncogén RET. Hija1 se realizó Tiroidectomía Proiláctica por Hiperplasia Folicular de Tiroides e Hiperplasia Linfática Benigna en nódulo post-auricular derecho. Hija7 se practicó Parotidectomía izquierda por Adenoma Pleomórico Benigno. Hijo5 fue diagnosticado con CMT multifocal. Hija9 se le hizo Tiroidectomía Total con linfadenectomía por CMT multifocal. Hijos 6 y 8, residentes en Estados Unidos de América, se les realizó Adrenalectomía por Feocromocitoma y Tiroidectomía Total por CMT multifocal, respectivamente. Además, se les realizaron pruebas genéticas y moleculares, cuyos resultados conirmaron la mutación en los exones 10, 11, 13, 14, 15 y 16 del proto-oncogén RET, del codón 634 con la sustitución del amino ácido cisteína por triptófano (Cys634Trp), conirmatorio de NEM-2A. Discusión . Constituye el primer trabajo publicado de esta patología en Honduras. Sugerimos la búsqueda epidemiológica de NEM en todo paciente diagnosticado con CMT.

Hipercortisolismo cíclico por adenoma hipofisario / Cyclic hypercortisolism due to pituitary adenoma

A case study of a 41 years old woman with cyclic hypercortisolism is explained. AT the beginning, its manegment was shrinking the tumor, however afterward she needs medical treatment during cycles. The fluctuating clinical and discrepant bioquemical findings make it hard to diagnose. A review of this rare disorder is explained.

Síndrome de QT largo y taquicardia ventricular en torsade de pointes secundarios a hipopituitarismo: una asociación a tener en cuenta: caso clínico / Long QT syndrome and polymorphic ventricular tachycardia due to hypopituitarism: report of one case

Symptoms of hypopituitarism are usually chronic and nonspecific, but rarely the disease can have acute and life threatening manifestations. We report a 53 years old female with a pituitary adenoma that was admitted to our hospital because of syncope. The electrocardiogram showed sinus bradycardia with a prolonged QT interval. Frequent runs of non-sustained polymorphic ventricular tachycardia were noted on telemetry. The patient had a history of severe acute headaches in the previous days and laboratory tests revealed severe secondary hypothyroidism, adrenal insufficiency and a decrease in pituitary hormones. A magnetic resonance imaging of the head showed changes in the size and contrast enhancement of the adenoma. A diagnosis of hypopituitarism secondary to pituitary apoplexy was made and treatment with hydrocortisone and, subsequently, levothyroxine was started. Hormonal disorders such as hypothyroidism, adrenal insufficiency or hypopituitarism should be considered as unusual causes for reversible cardiomyopathy, long QT syndrome and ventricular arrhythmias.

Asociación de craneofaringioma y síndrome de Klinefelter en la transición puberal: un desafío diagnóstico / Craniopharyngioma and Klinefelter syndrome during the pubertal transition: A diagnostic challenge

Los craneofaringiomas son de los tumores hipofisarios más frecuentes en la niñez y, sea por su evolución o por el tratamiento que requieren, pueden comprometer el desarrollo puberal. El síndrome de Klinefelter es la causa más frecuente de hipogonadismo hipergonadotrópico en el varón. La presentación concomitante de ambas entidades es extremadamente baja (1/10(9)) y plantea un interrogante acerca de una probable asociación fisiopatológica. Se presenta el caso de un paciente belga de 18 años, con diagnóstico de craneofaringioma en la niñez y panhipopituitarismo luego del tratamiento quirúrgico y radioterápico. Al llegar a los 14 años, se inició la inducción puberal con gonadotropinas. Ante la falta de respuesta clínica, se completó una evaluación genética, que evidenció, de manera homogénea, una trisomía XXY. La falta de respuesta al tratamiento de inducción con gonadotropina exógena reveló la asociación de hipogonadismo primario y secundario, que demostró la importancia del seguimiento multidisciplinario que estos pacientes requieren.

Craniopharyngioma is the most common pituitary tumor in childhood. It can compromise the pubertal development because of its evolution or treatment. Syndrome of Klinefelter is the most common cause of hipergonadotrophic hypogonadism in males. The concomitant presentation of both entities is extremely low (1/10(9)) and the pathophysiological association is questionned. We present the case of a 18-year-old Belgian patient. He had a diagnosis of craniopharyngioma in childhood and he presented with panhypopituitarism after radiotherapy and surgical treatment. At the age of 14, he started pubertal induction with gonadotropin therapy without clinical response. A genetic evaluation confirmed a homogeneous 47, XXY karyotype. Failure of exogenous gonadotropin therapy revealed the hidden association of primary and secondary hypogonadism, demonstrating the importance of the followup and a multidisciplinary approach in these patients.

Síndrome meningeo como manifestación de apoplejia hipofisiaria, análisis prospectivo de siete casos / Meningeal syndrome as manifestation of pituitary apoplexy, prospective analysis of seven cases

Introducción: la apoplejía hipofisiaria es una grave pero poco frecuente emergencia médico -neuroquirúrgica, con una incidencia global reportada del 1 por ciento a 2 por ciento Es causado por una hemorragia o infarto en relación a la glándula pituitaria, pudiendo existir extravasación de contenido necrótico o hemorrágico al espacio subaracnoideo, manifestándose como un síndrome meníngeo aséptico o hemorrágico. Sin embargo, su frecuencia no está estudiada. Material y Métodos: Se realizó un registro prospectivo entre enero de 2013 y agosto de 2014, para el estudio de líquido céfalo raquídeo, en pacientes con diagnóstico clínico- imagenológico de apoplejía hipofisiaria. Además un registró detallado de las manifestaciones clínicas y de laboratorio. Resultados: En este período se reclutaron 8 casos con apoplejía hipofisiaria clínica, de los cuales 7 fueron incluidos, siendo excluido un paciente por rechazar su participación en el estudio. De los 7 pacientes restantes, se evidenciaron signos meníngeos clínicos en el 86 por ciento (6/7), confirmando alteraciones del estudio cito-químico en todos ellos. En cada caso se descartó patología infecciosa o vascular como etiología. Conclusión: Si bien el debut de una apoplejía hipofisiaria como un síndrome meníngeo aséptico o hemorragia subaracnoidea, se encuentra documentado como casos anecdóticos, nuestros resultados apuntan a que sería una manifestación frecuente e importante a considerar para un adecuado diagnóstico diferencial y monitoreo de complicaciones infrecuentes.

Introduction: pituitary apoplexy is a serious but rare neurosurgical emergency, with an overall reported incidence of 1 percent to 2 percent. It is caused by bleeding or infarction related to the pituitary gland, there may be necrotic or hemorrhagic extravasation content to the subarachnoid space, manifesting as an aseptic o hemorrhagic meningeal syndrome. However, their frequency is not studied. Material and Methods: A prospective registry between January 2013 and August 2014, for the study of cerebro spinal fluid in patients with clinical and imaging diagnosis of pituitary apoplexy was performed. In addition, a detailed analysis of the clinical sintoms and laboratory was recorded. Results: In this period, 8 cases with clinical pituitary apoplexy were recluted, of which 7 were included, being excluded from a patient who refuses to participate in the study. Of the remaining 7 patients,clinical meningeal signs were evident in 86 percent (6/7), confirming alterations cyto-chemical study all of them. In each case infectious or vascular pathology was ruled out as a cause. Conclusion: While the debut of a pituitary apoplexy as an aseptic meningeal syndrome or subarachnoid hemorrhage, is documented as anecdotal cases, our results would suggest that is a common and important manifestation, to consider an appropriate differential diagnosis and monitoring of rare complications.

The Application of a New Maximum Color Contrast Sensitivity Test to the Early Prediction of Chiasma Damage in Cases of Pituitary Adenoma: The Pilot Study

PURPOSE: Our objective was to estimate the maximum color contrast sensitivity (MCCS) thresholds in individuals with chiasma opticum damage. METHODS: The pilot study tested 41 people with pituitary adenoma (PA) and 100 age- and gender-matched controls. Patients were divided into two groups according to PA size, PA ≤1 cm or PA >1 cm. A new MCCS test program was used for color discrimination. RESULTS: The mean total error score (TES) of MCCS was 1.8 in the PA ≤1 cm group (standard deviation [SD], 0.38), 3.5 in the PA >1 cm group (SD, 0.96), and 1.4 in the control group (SD, 0.31; p 1 cm (p < 0.01). In PA patients with normal VA, the TES was 2.35 times worse than that of healthy persons (p < 0.01).

Anatomía radiológica del seno esfenoidal: análisis en 120 pacientes intervenidos / Radiologic anatomy of sphenoidal sinus, analysis of 120 surgically intervened patients

Introducción: La patología tumoral hipofisiaria es una consulta frecuente en Neurocirugía. La vía de abordaje a elegir preferentemente es transesfenoidal. Por esto, es de suma importancia conocer la anatomía de los senos paranasales cuando se planifica este abordaje. Materiales y Métodos: Se describe la anatomía radiológica de estudios por Tomografía Computada (TC) y Resonancia Magnética (RM) de 120 pacientes con diagnóstico de adenoma hipofisiario, que se sometieron a cirugía transesfenoidal de hipófisis, prestando especial interés en la morfología de las cavidades paranasales esfenoidales. Resultados: Tipo de neumatización: preselar 25 por ciento y postselar 75 por ciento; Septos: simples 45 por ciento y múltiples 55 por ciento; diámetros promedio: antero-posterior 2,3 cm, transverso: 3,3 cm y vertical: 2,25 cm. Los Senos esfenoidales con neumatización postselar tienen un diámetro anteroposterior significativamente mayor (P < 0,005) que los preselares. Los senos esfenoidales con septo simple tienen un diámetro transverso significativamente mayor (P < 0,007) que los con múltiples septos. El 50 por ciento de los septos siguen una trayectoria paramediana, y el 50 por ciento se desvía hacia una de las arterias carótidas. Conclusión: se confirma la gran variación anatómica del seno esfenoidal siendo independiente del sexo del paciente, además hemos constatado que la relación establecida por los septos intraselares con elementos vasculares intracraneales es muy variable, siendo este hallazgo de suma importancia en la planificación preoperatoria. Los hallazgos realizados por Tomografía computada (TC), pueden ser homologables a lo descrito por otros autores en disecciones cadavéricas.

Introduction: The pituitary tumor pathology is a common query in Neurosurgery. The choice of approach is transsphenoidal preferably, so it is extremely important to know the anatomy of the sinuses when planning this approach. Materials and Methods: We describe the radiological anatomy studies Computerized tomography (CT) and Magnetic Resonance (MR) of 120 patients with pituitary adenoma who underwent transsphenoidal pituitary surgery, with special attention to the morphology of the sphenoid sinuses. Results: Neumatization type: pre-sellar 25 percent and 75 percent post-sellar; Septa: Single 45 percent, multiple 55 percent, mean diameters: 2.3 cm anteroposterior, transverse: 3.3 cm and vertical: 2.25 cm. The sphenoid sinuses with postsellar neumatization have anteroposterior diameter significantly higher (P < 0.005) than the pre-sellar. The sphenoid sinuses are single septum transverse diameter significantly higher (P < 0.007) than those with multiple septa. The 50 percent of the septa paramedian follow a path, and 50 percent is diverted to one of the carotid arteries. Conclusion: Results support the large sphenoid sinus anatomical variation is independent of patient sex, and we found that the relationship established by the intrasellar septa with intracranial vascular elements is very variable, this finding being paramount in preoperative planning. The findings by computed tomography (CT) may be comparable to that described by other authors in cadaveric dissections.

Gasto energético, composición corporal, trastornos metabólicos e ingesta energética en pacientes con antecedentes de craneofaringioma / Energy expenditure, body composition, metabolic disorders, and energy intake in patients with a history of craniopharyngioma

Introducción: Los craneofaringiomas son malformaciones histológicamente benignas que se sitúan entre el hipotálamo y la hipófisis, zonas con un rol determinante en la modulación de la saciedad. Aun siendo tumores benignos, presentan una considerable morbilidad. La obesidad está presente hasta en un 52% de los pacientes. Objetivo: evaluar factores de riesgo cardiovascular, composición corporal y gasto energético en pacientes con craneofaringioma, y compararlos con un grupo de obesos multifactoriales. Material y métodos: Se incluyeron todos los pacientes con resección quirúrgica de craneofaringioma, menores de 21 años, en seguimiento en nuestro centro entre mayo 2012 hasta abril 2013 que aceptaron participar por medio del consentimiento informado. Se realizó valoración antropométrica, composición corporal con impedanciometría, gasto energético con calorimetría indirecta y valoración de ingesta energética y de macronutrientes. Se determinó resistencia a la insulina (HOMA-IR) y dislipemia. Se comparó a los pacientes con craneofaringioma con obesidad, con un grupo de pacientes con obesidad multifactorial. Resultados: se estudiaron 39 pacientes. El 59% era obeso y presentó significativamente menor% de masa magra (62.4 vs 67.5 p=0.01) y mayor% de masa grasa (37.5 vs 32.5 p=0.01) comparados con los obesos multifactoriales. No se encontró diferencias en el compromiso metabólico entre los obesos con y sin antecedente de craneofaringioma. Se dividieron los pacientes en tertilos según% de gasto energético para categorizar en gasto bajo vs normal. Se encontró asociación positiva entre% de gasto energético y% de masa magra en obesos multifactoriales (68±1%; en los gasto normal vs 62.6± 1% en los gasto bajo: p 0,04). Sin diferencias dentro de la población de obesos con antecedente de craneofaringioma (62±2.7 en los gasto normal/alto vs 61.2±1.8% en los gasto bajo: p 0,8). El gasto energético basal (REE) fue menor en los pacientes con antecedente de craneofaringioma vs obesos multifactoriales, independientemente de la masa magra, lo que sustenta que existirían otros factores que actuarían disminuyendo el gasto energético. No hubo diferencia con respecto a la ingesta en ambos grupos estudiados. Conclusiones: los pacientes con antecedente de craneofaringioma presentan menor gasto energético no relacionado a la masa magra y similar ingesta energética comparado con obesos multifactoriales. No hubo diferencias en el compromiso metabólico entre los obesos con y sin antecedentes de craneofaringioma (AU)

Introduction: Craniopharyngiomas are histologically benign malformations located between hypothalamus and the pituitary gland, areas that play an important role in satiety modulation. Although the tumors are benign, they may cause significant morbidity. Obesity is found in up to 52% of patients. Aim: To assess cardiovascular risk factors, body composition, and energy expenditure in patients with craniopharyngioma, and to compare them to results in a group of children with multifactorial obesity. Material and methods: All patients who underwent surgical resection of craniopharyngioma, younger than 21 years of age, who were being followed-up at our center between May 2012 and April 2013 who gave their informed consent to participate were enrolled in the study. Anthropometric measurements, body composition with impedanciometer, energy expenditure with indirect calorimetry, and energy and macronutrient intake were evaluated. Insulin resistance (HOMA-IR) and dyslipidemia were determined. Patients with craniopharyngioma associated with obesity were compared to patients with multifactorial obesity. Results: Of 39 patients studied, 59% were obese and a significantly lower percentage of lean mass (62.4 vs 67.5 p=0.01) and a higher percentage of fat mass (37.5 vs 32.5 p=0.01) compared to multifactorial obese subjects. No differences were found in metabolic involvement between obese subjects with and those without a history of craniopharyngioma. Patients were divided into tertiles according to percentage of energy expenditure to categorize low versus normal expenditure. A positive correlation was found between percentage of energy expenditure and lean mass percentage in subjects with multifactorial obesity (68±1%; in those with normal energy expenditure versus 62.6±1% in those with low energy expenditure: p 0.04). No difference was found within the group of obese patients with a history of craniopharyngioma (62±2.7 in those with normal/high expenditure versus 61.2±1.8% in those with low expenditure: p 0.8). Baseline energy expenditure (BEE) was lower in craniopharyngioma patients than in those with multifactorial obesity, regardless of lean mass percentage, supporting the hypothesis that other factors may be involved in the decrease of energy expenditure. There was no difference in the food intake between both groups. Conclusions: Patients with a history of craniopharyngioma had a lower energy expenditure unrelated to lean mass and a similar energy intake compared to subjects with multifactorial obesity. No differences were found in metabolic involvement between obese subject with and those without a history of craniopharyngioma (AU)

Malignant ventricular tachycardia in acromegaly: a case report / Taquicardia ventricular maligna na acromegalia: relato de caso

CONTEXT: In patients with acromegaly, cardiovascular complications are the main cause of death; sudden death has been associated with ventricular tachyarrhythmias. In other patients with life-threatening malignant ventricular tachyarrhythmias, surgical placement of an implantable cardioverter-defibrillator (ICD) has proved highly effective in reducing sudden death rates. CASE REPORT: The present article reports the case of a 50-year-old male acromegalic patient who presented symptoms of syncope induced by ventricular tachycardia. An ICD was surgically implanted and a pituitary adenoma, which was responsible for the acromegaly, was completely removed in the same procedure. The surgery was successful and the ventricular arrhythmias were effectively terminated. During six months of follow-up, no documented arrhythmic episodes occurred. CONCLUSION: In patients with acromegaly, malignant ventricular tachyarrhythmia might be effectively controlled by implantation of an ICD and surgical removal of the pituitary adenoma. .

CONTEXTO: As complicações cardiovasculares são a principal causa de morte em pacientes com acromegalia, e a morte súbita tem sido associada a taquiarritmias ventriculares. Em outros pacientes com risco de vida por taquiarritmias ventriculares malignas, a aplicação cirúrgica de um cardioversor-desfibrilador implantável (CDI) provou ser altamente eficaz na redução das taxas de morte súbita. RELATO DE CASO: O presente artigo relata o caso de um paciente acromegálico de 50 anos de idade e do sexo masculino, que apresentava sintomas de síncope induzida por taquicardia ventricular. Foi implantado cirurgicamente nesse paciente um CDI e na mesma intervenção cirúrgica foi completamente removido um adenoma hipofisário responsável pela acromegalia. A cirurgia foi bem-sucedida e o paciente deixou de sofrer de arritmias ventriculares. Durante seis meses de acompanhamento, não se documentaram, nesse paciente, episódios arrítmicos. CONCLUSÃO: A taquiarritmia ventricular maligna pode ser efetivamente controlada em pacientes com acromegalia pela implantação de um CDI combinado com a remoção cirúrgica do adenoma hipofisário. .

Macroadenoma hipofisário: alterações campimétricas visuais / Pituitary macroadenoma: visual field defects

O macroadenoma de hipófise é um tumor de evolução lenta e que apresenta importantes distúrbios visuais como baixa acuidade visual e alterações campimétricas. Estes sintomas levam os pacientes a procurarem, muitas vezes, os oftalmologistas. Neste caso, analisaremos uma paciente portadora de glaucoma que apresentava, associadamente, um macroadenoma hipofisário.

The pituitary adenoma is a tumor of slow evolution and has important visual disturbances such as low visual acuity and field defects. These symptoms lead patients to seek often ophthalmologists. In this case, we will analyze a patient with glaucoma who had an associated pituitary macroadenoma.

Clinical and laboratorial characterization and post-surgical follow-up of 87 patients with non-functioning pituitary macroadenomas / Caracterização clínica e laboratorial e seguimento pós-cirúrgico de 87 portadores de macroadenomas hipofisários não funcionantes

Objective: It was to assess the main characteristics of patients undergoing pituitary tumor surgery. Method: Eighty-seven patients (44 men; 44.8±13 years old) were included. Results: The main symptoms were visual alterations (87.3%), headache (70.1%), diminished libido (34.4%), galactorrhea (22.9%) and hair loss (19.5%). The axes affected were gonadotropic (72.6%), thyrotropic (48.4%) and corticotropic (38.7%), without significant changes after surgery. The average largest tumor diameter was 3.1 cm before surgery and 1.56 cm after surgery. The most frequent postoperative complications were hydro-electrolyte and acid-base disorders (12%), diabetes insipidus (9%), visual field alterations (9%), liquoric fistula (8%) and nasal obstruction (7%). The patients were affected by more than one complication. Conclusion: Although a decrease in tumor volume was achieved through surgery, hormonal deficiencies persisted in most of the patients and new surgical approaches were necessary for dealing with tumor recurrence or persistence. .

Objetivo: Avaliar as principais características de pacientes operados de tumor de hipófise. Método: Foram incluídos 87 pacientes (44 homens; 44,8±13 anos). Resultados: Os principais sintomas foram alterações visuais (87,3%), cefaleia (70,1%), diminuição da libido (34,4%), galactorreia (22,9%) e queda de pelos (19,5%). Os eixos afetados foram gonadotrófico (72,6%), tireotrófico (48,4%) e corticotrófico (38,7%). Não houve mudanças significativas após a cirurgia. A média do maior diâmetro do tumor foi 3,1 cm antes da cirurgia e 1,56 cm após a cirurgia. As complicações pós-cirúrgicas mais frequentes foram distúrbios hidroeletrolíticos e ácido-básicos (12%), diabetes insipidus (9%), alterações do campo visual (9%), fístula liquórica (8%) e obstrução nasal (7%). Ocorreu mais de uma complicação no mesmo paciente. Conclusão: Embora tenha obtido diminuição da massa tumoral com a cirurgia, as deficiências hormonais persistiram na maioria dos pacientes e ocorreu necessidade de novas abordagens por recidiva ou persistência do tumor. .

Enfermedad de Cushing en un adulto mayor / Cushing's disease in the elderly patient

Introducción: la enfermedad de Cushing se presenta por secreción aumentada de ACTH, debido a tumor hipofisario que estimula la zona fascicular de la corteza suprarrenal y produce hipersecreción de glucocorticoides. Objetivos: explicar manifestaciones clínico-humorales y causales de la enfermedad de Cushing en un adulto mayor. Material y método:estudio descriptivo, retrospectivo. Presentamos paciente de 62 años que ingresa en nuestro centro por manifestaciones de plétora facial, obesidad centrípeta, debuta con hipertensión arterial y diabetes mellitus. Examen físico: datos referidos anteriormente. Programa de estudio: cortisol basal 690 nmol/L. Ritmo circadiano para el cortisol: basal 8:00 am 900 nmol/L y 11.00 pm 754 nmol/L. Inhibiciones con 2mg y 8mg de dexametasona 468 nmo/L y 429 nmol/L respectivamente. Tomografía contrastada de silla turca: aumento del diámetro de la silla turca que no confirma aumento de la glándula, realizar siguiente estudio. Resonancia magnética nuclear de silla turca: tumor intraselar en relación con macroadenoma. Resultado: macroadenoma con secreción aumentada de ACTH. Conclusiones: Enfermedad de Cushing.

Introduction: Cushing's disease arises from increased ACTH secretion from a pituitary tumor that stimulates the area fasciculata of the adrenal cortex and produces hypersecretion of glucocorticoids. Objectives: To explain the clinical and humoral manifestations of Cushing's disease in elderly adults. Methods: The article is a descriptive and retrospective study of a case report on a 62 year old patient that is admitted to our Center with manifestations of facial plethora and centripetal obesity. Onset was characterized by hypertension and diabetes mellitus. Definitive diagnosis was Cushing's disease from a macroadenoma with increased ACTH secretion.

Bromocriptine for Control of Hyperthermia in a Patient with Mixed Autonomic Hyperactivity after Neurosurgery: A Case Report

Mixed autonomic hyperactivity disorder (MAHD) among patients with acquired brain injury can be rare. A delayed diagnosis of MAHD might exacerbate the clinical outcome and increase healthcare expenses with unnecessary testing. However, MAHD is still an underrecognized and evolving disease entity. A 25-yr-old woman was admitted the clinic due to craniopharyngioma. After an extensive tumor resection, she complained of sustained fever, papillary contraction, hiccup, lacrimation, and sighing. An extensive evaluation of the sustained fever was conducted. Finally, the cause for MAHD was suspected, and the patient was successfully treated with bromocriptine for a month.

Atualização terapêutica no tratamento dos craniofaringiomas / Therapeutic update on the treatment of craniopharyngiomas

O craniofaringioma é uma neoplasia de natureza benigna, pouco frequente, responsável por 1 por cento a 3 por cento de todos os tumores intracranianos, sendo a mais frequente neoplasia intracraniana não neuroepitelial na criança. Geralmente o tumor é restrito à região selar e ao III ventrículo, mas, em decorrência da infiltração e frequente aderência ao sistema nervoso central, apresenta comportamento clínico muitas vezes desfavorável, sendo classificado pela Organização Mundial de Saúde (OMS) como grau I, caracterizado como tumor de baixo ou incerto potencial de malignização. As sequelas endocrinológicas ganham destaque devido ao importante impacto na qualidade de vida dos pacientes, na maioria das vezes crianças. O hipopituitarismo e a obesidade hipotalâmica são complicações frequentes, sendo o tratamento desse tumor um grande desafio para endocrinologistas e neurocirurgiões. A combinação da cirurgia, radioterapia e aplicação de drogas e radioisótopos intratumorais tem como objetivo maximizar as chances de cura e tentar minimizar as sequelas pós-operatórias, mas, mesmo assim, a recidiva ainda é frequente. A escolha da modalidade de tratamento mais adequado para os craniofaringiomas é uma decisão difícil e que deve sempre ser individualizada para cada paciente. Com o objetivo de explorar as múltiplas opções terapêuticas para o craniofaringioma, foi realizada revisão na literatura com ênfase nas possibilidades terapêuticas e complicações inerentes ao tratamento dessa patologia.

Craniopharyngioma is an uncommon benign neoplasm, accounting for 1 percent-3 percent of all intracranial tumors, and the most common non-neuroepithelial intracranial neoplasm in childhood. Usually, the tumor is confined to the sellar region and the third ventricle, but due to frequent infiltration and adherence to the central nervous system, it often has an unfavorable clinical behavior. Therefore, it is classified by the World Health Organization (WHO) as a tumor of low or uncertain malignant potential. Endocrine after effects, mainly hypothalamic hypopituitarism, obesity and diabetes insipidus are highlighted due to their important impact on the quality of life of patients, mostly children. Optimal treatment of this tumor is a major challenge for neurosurgeons and endocrinologists. The combination of surgery, radiation, and application of radioisotopes and intratumoral drugs, aims at maximizing the chances of cure with minimal complications. Yet, recurrence is still frequent. Choosing the best treatment modality for craniopharyngiomas is a difficult decision, and it should always be specific for each case. In order to explore the multiple therapeutic options for craniopharyngiomas, we reviewed the literature with emphasis on the therapeutic possibilities and complications inherent to the treatment of this disease.