Updated Recommendations on the Prevention and Treatment of Infections in Children With Asplenia/Hyposplenism.

Children with congenital or acquired asplenia or hyposplenism have an increased risk for severe and even life-threatening infections mainly due to encapsulated bacteria. Current practice focuses on preventing severe infections with timely administration of vaccinations, antibacterial prophylaxis when indicated, and urgent evaluation and treatment of febrile events. As new vaccines are now available for both children and adults with asplenia/hyposplenism, we present an up-to-date recommendation on the prevention and management of acute infections in children with asplenia/hyposplenism.

ORAI1 inhibition as an efficient preclinical therapy for tubular aggregate myopathy and Stormorken syndrome.

Tubular aggregate myopathy (TAM) and Stormorken syndrome (STRMK) are clinically overlapping disorders characterized by childhood-onset muscle weakness and a variable occurrence of multisystemic signs, including short stature, thrombocytopenia, and hyposplenism. TAM/STRMK is caused by gain-of-function mutations in the Ca2+ sensor STIM1 or the Ca2+ channel ORAI1, both of which regulate Ca2+ homeostasis through the ubiquitous store-operated Ca2+ entry (SOCE) mechanism. Functional experiments in cells have demonstrated that the TAM/STRMK mutations induce SOCE overactivation, resulting in excessive influx of extracellular Ca2+. There is currently no treatment for TAM/STRMK, but SOCE is amenable to manipulation. Here, we crossed Stim1R304W/+ mice harboring the most common TAM/STRMK mutation with Orai1R93W/+ mice carrying an ORAI1 mutation partially obstructing Ca2+ influx. Compared with Stim1R304W/+ littermates, Stim1R304W/+Orai1R93W/+ offspring showed a normalization of bone architecture, spleen histology, and muscle morphology; an increase of thrombocytes; and improved muscle contraction and relaxation kinetics. Accordingly, comparative RNA-Seq detected more than 1,200 dysregulated genes in Stim1R304W/+ muscle and revealed a major restoration of gene expression in Stim1R304W/+Orai1R93W/+ mice. Altogether, we provide physiological, morphological, functional, and molecular data highlighting the therapeutic potential of ORAI1 inhibition to rescue the multisystemic TAM/STRMK signs, and we identified myostatin as a promising biomarker for TAM/STRMK in humans and mice.

Presencia y biometría de bazo accesorio en individuos chilenos: estudio por tomografía computarizada / Presence and biometry of accessory spleen in Chilean individuals: study by computerized tomography

El bazo se localiza en el cuadrante superior izquierdo del abdomen, relacionándose posteriormente con la 9a a 11a costilla, de las que se separa por el diafragma y el receso costodiafragmático, se localiza por detrás del estómago y lateralmente al riñón izquierdo. Por alteraciones en su desarrollo pueden generarse bazos accesorios (BA), considerándose un tejido ectópico del bazo. Se consideran tejido normal, con los mismos procesos fisiológicos que el bazo principal. Con el propósito de localizar y determinar aspectos biométricos de los mismos, se realizó un estudio de corte transversal y de carácter descriptivo, sobre una muestra de 220 exámenes de TC pertenecientes a pacientes mayores de 18 años del Hospital Regional Hernán Henríquez Aravena, Temuco, Chile. Para este estudio se excluyeron toda aquellas TC con antecedentes de esplenectomía y lesiones de Bazo o peri-esplénicas. El análisis de los datos mostró una prevalencia de 32,3 % de BA, pudiendo ser de una única presencia, dos e incluso tres BA por paciente.De un total de 71 personas que tienen al menos un BA, 34 (47,89 %) fueron de sexo femenino y 37 (52,11 %) de sexo masculino. Hubo 56 pacientes (78,9 %) con un BA, 29 (40,85 %) del sexo femenino y 27 (38,03 %) del masculino; 15 (21,1 %) presentaron más de un BA, 5 (7,04 %) de sexo femenino y 10 (14,08 %) de sexo masculino, si bien se puede observar variación en la cantidad de BA según sexo, no existe una relación estadísticamente significativa entre dichas variables. La ubicación más frecuente encontrada en el plano axial fue la zona antero-medial con 59 casos (66,29 %); asimismo, en el plano sagital, la localización más frecuente fue en el polo inferior con 40 casos (44,44 %). Datos biométricos de estos BA son mostrados en Tablas. Esta información será de gran valor morfológico y médico debido a la escasa literatura existente sobre esta materia en individuos chilenos.

The spleen is located in the upper left quadrant of the abdomen, subsequently related to the 9th to 11th rib, from which it is separated by the diaphragm and the cost-diaphragmatic recess, it is located behind the stomach and laterally to the left kidney. Due to alterations in its development, accessory spleens (AS) can be generated, being considered an ectopic tissue of the spleen. The AS are considered normal tissue, with the same physiological processes as the main spleen. With the purpose of locating and determining biometric aspects of them, a cross-sectional and descriptive study was carried out on a sample of 220 CT scans belonging to patients over 18 years of age at the Hernán Henríquez Aravena Regional Hospital, Temuco, Chile. For this study, all CT scans with a history of splenectomy and spleen or peri-splenic lesions were excluded. The analysis of the data showed a prevalence of 32.3 % of AS, being able to be of a single presence, two and even three AS per patient. Of a total of 71 people who have at least one AS, 34 (47.89 %) were female and 37 (52.11 %) male. There were 56 patients (78.9 %) with a one AS, 29 (40.85 %) of the female sex and 27 (38.03 %) of the male; 15 (21.1 %) presented more than one AS, 5 (7.04 %) female and 10 (14.08 %) male, although variation in the amount of AS according to sex can be observed, no there is a statistically significant relationship between these variables. The most frequent location found in the axial plane was the anteromedial zone with 59 cases (66.29 %); also, in the sagittal plane, the most frequent location was in the lower pole with 40 cases (44.44 %). Biometric data of these AS are shown in tables. This information will be of great morphological and medical value due to the limited existing literature on this subject in Chilean individuals.

Ruptura esplênica secundária à infeção por Plasmodium falciparum / Splenic rupture secondary to Plasmodium falciparum infection

A ruptura esplênica é uma complicação possível da malária. É importante pela dificuldade diagnóstica, pois um elevado índice de suspeição é necessário para um diagnóstico atempado. Pode condicionar uma hemorragia intraperitoneal e deve ser considerada no diagnóstico diferencial de quadros de dor abdominal, hipotensão e diminuição do hematócrito. Os autores descrevem o caso de um homem de 59 anos, com ruptura esplênica secundária à malária por Plasmodium falciparum, tendo realizado esplenectomia urgente. Com a apresentação do caso, os autores pretendem chamar a atenção para a necessidade de incluir esta afecção no diagnóstico diferencial dos doentes com malária e hipotensão refractária.

Splenic rupture is a possible complication of malaria. Due to its difficult diagnosis, it is important, because a high level of suspicion is needed for a timely diagnosis. It results in intraperitoneal bleeding and should be considered in the differential diagnosis of abdominal pain, hypotension and low hematocrit. The authors report the case of a 59-year old man with splenic rupture secondary to malarial infection by Plasmodium Falciparum, who was treated with urgent splenectomy. This case is presented to remind the clinicians of include this entity in the differential diagnosis of patients with malaria and refractory hypotension.

Sepsis neumocócica del adulto: ¿debemos descartar una asplenia congénita? / Adult pneumoccocical sepsis: Should we rule out congenital anesplenia?

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Ecografía de bazo y retroperitoneo / Ultrasound of spleen and retroperitoneum

La ecografía aporta datos de extraordinario valor a la hora de estudiar la enfermedad esplénica, resultando diagnóstica en la esplenomegalia y traumatismos esplénicos, y muy orientadora en enfermedad focal tanto benigna como maligna, con especial mención del linfoma. En la evaluación de la enfermedad suprarrenal y retroperitoneal, sin embargo, la ecografía se ve superada por otras técnicas como la TAC o la RMN, si bien sigue constituyendo un excelente método de screening y de seguimiento, y resulta también en ocasiones útil a la hora de un abordaje terapéutico no invasivo (AU)

Ultrasound provides data of extremely great value when studying spleen pathology, being diagnostic in splenomegaly and splenic trauma, as well as offering a good approach to the diagnosis of both benign and malignant focal pathology, particularly lymphoma. However, for the evaluation of adrenal and retroperitoneal diseases, other techniques such as CT or MRI are more suitable, even though ultrasound is still an excellent screening and monitoring method, as well as being useful in non-invasive therapeutic approaches (AU)

El bazo pediátrico no traumático. Valoración por imagen / Non-traumatic spleen disorders in children. Assessment by imaging

Para la mayoría de radiólogos y pediatras, el bazo es el "órgano olvidado", a pesar de estar afectado en múltiples situaciones clínicas de la infancia. Mientras que en el traumatismo abdominal pediátrico es el órgano más implicado, la patología esplénica no traumática es menos conocida. El bazo se visualiza bien mediante cualquier técnica de imagen: ecografía, tomografía computarizada, resonancia magnética, y de ellas, la primera es la más utilizada en niños. Conocer las características por imagen de las anomalías esplénicas, tanto congénitas como adquiridas, permite realizar una aproximación diagnóstica correcta, evitar procedimientos quirúrgicos o biopsias innecesarias y guiar al clínico hacia un tratamiento adecuado. Nuestro objetivo es mostrar el comportamiento del bazo en edad pediátrica con las diferentes técnicas de imagen: su anatomía normal, las principales variantes anatómicas y la patología esplénica no traumática más frecuente, correlacionando con clínica, serología o histología

The spleen is considered a "forgotten organ" by most radiologists and paediatricians despite being affected in many clinical paediatric situations. While it is the organ most often affected in paediatric abdominal trauma, non-traumatic spleen disorders are less well known. The spleen is well visualised by any imaging technique: ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI); the former is used most often in children. Using imaging techniques to determine the features of splenic anomalies, both congenital and acquired, enables a correct diagnostic approach, avoids unnecessary surgical procedures or biopsies, and helps the clinician to prescribe appropriate treatment. Our aim was to show the behaviour of the spleen in children using the different imaging techniques: its normal anatomy, the principal anatomical variants and the most common spleen disorder correlating with clinical symptoms, serology and histology

Diagnóstico por imagen de anomalías en el número y localización del bazo / Diagnostic imaging of abnormalities in the number and location of the spleen

En las exploraciones radiológicas abdominales, algunas anomalías del desarrollo, como el bazo accesorio periesplénico, son fácilmente reconocibles debido a su elevada incidencia. Sin embargo, otras menos frecuentes, como el bazo accesorio intrapancreático, la fusión esplenopancreática o esplenogonadal, la heterotaxia y el bazo errante, así como anomalías adquiridas como la esplenosis, pueden plantear dificultades diagnósticas. El propósito de nuestra revisión es mostrar los hallazgos radiológicos y el diagnóstico diferencial de dichas anomalías esplénicas poco habituales

In imaging studies, some developmental anomalies such as perisplenic accessory spleen are easily recognizable due to their high incidence. However, other, less common anomalies such as intrapancreatic accessory spleen, splenopancreatic fusion, splenogonadal fusion, heterotaxy, and wandering spleen, as well as acquired conditions such as splenosis, can pose diagnostic difficulties. This aim of this review is to show the imaging diagnosis and differential diagnoses of these uncommon splenic anomalies

A veces las cosas son lo que parecen / Sometimes things are what they seem

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Diagnóstico post mortem de septicemia neumocócica en una mujer joven con hiposplenia / Post-mortem diagnosis of pneumococcal septicaemia in young woman with hyposplenia

La enfermedad neumocócica es una importante causa de morbimortalidad. La descripción de casos de identificación post mortem de Streptococcus pneumoniae (SP) es infrecuente. Presentamos un caso de fallecimiento por sepsis fulminante secundaria a SP en una mujer joven con factores de riesgo. Se trata de un fallecimiento comunicado por urgencias hospitalarias como una parada cardiaca extrahospitalaria de posible origen abdominal. La autopsia judicial reveló hallazgos compatibles con un cuadro de sepsis fulminante, por lo que se comunicó a vigilancia epidemiológica de salud pública. Se realizaron estudios microbiológicos post mortem, que permitieron aislar SP en el cultivo de líquido pleural, posteriormente la PCR en sangre identificó SP y se caracterizó el serotipo (22F). A pesar de que la hipoesplenia era conocida desde la adolescencia y ser una indicación de vacunación, la paciente no estaba vacunada frente al SP por ninguna de las 2 vacunas actualmente recomendadas en España

Pneumococcal infections are a significant cause of morbidity and mortality. Post-mortem identification of Streptococcus pneumoniae (SP) is uncommon. A case of death due to fulminant pneumococcal septicaemia in a young woman with hyposplenia is presented. The death was notified by the hospital emergency department as an out-of-hospital cardiac arrest of possible abdominal aetiology. Forensic autopsy found changes suggestive of fulminant septicaemia and the public health epidemiology service was informed. Post-mortem microbiology studies were carried out, in which SP was isolated in the pleural fluid culture, and later PCR in blood identified SP, as well as determining and the serotype (22F). Although hyposplenia was known since adolescence, and was an indication of vaccination, the patient was not vaccinated against SP by any of the two currently recommended vaccines in Spain

Manifestaciones abdominales de las anomalías del Situs Ambiguous en el adulto: a propósito de cuatro casos / Situs Ambiguous abnormalities in adults: a study of four cases

Las Anomalías del Situs (ASIT) han sido descritas principalmente en la población pediátrica y existe escasa información sobre éstas en el adulto. Son anomalías muy raras; en el caso del Situs Ambiguous (SAMB) en el adulto sólo se han publicado pocas series de casos. SAMB, también llamado heterotaxia o Síndrome de heterotaxia, es definido como la posición anormal o ambigua de los órganos y vasos, que además pueden asociarse a alteraciones morfológicas características de cada órgano. Existen dos grandes grupos dentro de los SAMB: Poliesplenia y Asplenia. En la presente publicación se expone una revisión bibliográfica específica, la cual tiene énfasis en clasificación de las ASIT del adulto y los hallazgos abdominales en los casos de SAMB. Será complementada clínica e imaginológicamente con cuatro casos de estudios tomográficos abdominales.

Anomalies Situs (ASIT) has been described mainly in the pediatric population and there is little information on this in adults. These are very rare abnormalities; in the case of Situs Ambiguous (SAMB) in adults, there have only been a few published case series. SAMB, also called heterotaxy or heterotaxy Syndrome, is defined as the abnormal or ambiguous positioning of organs and vessels, that could also be associated with morphological alterations characteristic of each organ. There are two main groups within SAMB: polysplenia and asplenia. This publication present a specific literature review, with emphasis on the classification of adult ASIT and abdominal findings in cases of SAMB. It will be complemented clinically and radiologically with four cases of abdominal tomographic imaging studies.

Bazo accesorio simulando tumor suprarrenal / Aberrant spleen simulating an adrenal mass

Introduction: The diagnosis of adrenal incidentalomas is common in current clinical practice. Clinical case: We report a 69 years-old female patient with hypertension, who underwent an abdominal CAT Scan, finding a left adrenal mass of 8 cm diameter. Subsequent studies showed elevated urinary metanephrine levels. With the suspicion of a pheochromocytoma, a laparoscopic surgery was performed. The mass resulted to be an aberrant spleen.

Introducción: El diagnóstico de los así llamados "incidentalomas" suprarrenales, cada vez más frecuente en la práctica clínica, plantea un diagnóstico diferencial importante. Caso clínico: Se presenta el caso clínico de una paciente de 69 años, hipertensa, con el hallazgo de una masa suprarrenal izquierda aparentemente funcionante, operada por vía laparoscópica y que resultó ser un bazo aberrante. Se discute el diagnóstico diferencial entre masa suprarrenal y pseudo-tumores adrenales y la embriología y presentación clínica del bazo aberrante.

Fusión esplenogonadal como causa de masa testicular pediátrica: Caso clínico / Splenogonadal fusion, a rare cause of pediatric testicular mass: clinical case

La fusión esplenogonadal es una variación anatómica infrecuente, con un pequeño número de reportes en la literatura. La estrecha relación entre el bazo y la gónada primitiva izquierda en un momento del desarrollo embrionario logra explicar la existencia de este fenómeno. Objetivo: Comunicar el primer reporte en la literatura nacional de fusión esplenogonadal. Caso clínico: Preescolar de 2 años 10 meses que consultó por aumento de volumen silente en testículo izquierdo estudiado mediante ecografía, la cual reveló una masa hipervascular en su polo superior. Se decidió su exploración quirúrgica donde se apreció una estructura sólida, bien adherida al teste, de superficie similar a la del hígado. Fue resecada y enviada a biopsia rápida la cual se informó como un tejido esplénico benigno. Discusión: La literatura revisada describe los aspectos más importantes de ésta entidad, su desarrollo embrionario, clasificación, presentación clínica, métodos diagnósticos y tratamiento. El conocimiento de esta condición por parte del equipo médico y su inclusión en el diagnóstico diferencial de masa testicular, permite tomar medidas diagnósticas en el intraoperatorio para un tratamiento conservador. Una vez establecido el diagnóstico, habitualmente en el intraoperatorio, es suficiente la enucleación completa del tejido esplénico, evitando orquiectomías innecesarias.

The splenogonadal fusion is a rare anatomical variation, with only a few reports in the literature. The close relationship between the spleen and the primitive left gonad in a specific moment of the embryonic development could explain this phenomenon. Objective: To point out the clinical presentation and treatment of this condition, through the first case published in our country. Clinical case: We report the case of a male child, 2 years 10-month-old, who consulted for silent swelling of the left testis, which Doppler ultrasound study revealed a hypervascular, polar mass. A surgical exploration was performed, and a solid, adherent, liver-like structure was observed over the testicle. The intraoperatory biopsy was informed as benign splenic tissue. Conclusion: The reviewed articles describe important information about the development, classification, presentation and management of this condition. The knowledge and inclusion of this condition in the differential diagnosis of pediatric testicular masses allows the medical team to choose for conservative therapeutic measures. Once the diagnosis is established, often intraoperatively, a complete resection of the splenic tissue is enough for these patients, avoiding an unnecessary orchiectomy.

Splenogonadal fusion and testicular cancer: case report and review of the literature / Fusão esplenogonadal e câncer testicular: relato de caso e revisão da literatura

A 36 year-old man after tests for assessing male infertility was diagnosed with primary infertility, bilateral cryptorchidism, nonobstructive azoospermia and discontinuous splenogonadal fusion. Carcinoma in situ was found in his left testicle, which was intraabdominal and associated with splenogonadal fusion. To our knowledge, this is the fourth case of splenogonadal fusion associated with testicular cancer reported. One should always bear in mind the possibility of this association for the left cryptorchid testicle.

Um homem de 36 anos, depois de ser submetido a exames para avaliação de infertilidade masculina, foi diagnosticado com infertilidade masculina primária, criptorquidia bilateral, azoospermia não obstrutiva e fusão esplenogonadal descontínua. Carcinoma in situ estava presente no testículo esquerdo, que tinha localização intra-abdominal e estava associado à fusão esplenogonadal. Esse é o quarto caso de fusão esplenogonadal associada a câncer testicular, segundo nossa avaliação. Deve-se sempre ter em mente a possibilidade dessa associação em testículos criptorquídicos à esquerda.

A study on the variations of size, shape and external features of the spleen in south Indian population / Estudio sobre la variación del tamaño, forma y características externas del bazo en la población del sur de la India

The spleen is the largest lymphoid organ. It is wedge shaped and measures about 1 inch thick, 3 inches broad and 5 inches long. It presents characteristic notches on its superior margin and has a hilum on the visceral surface. The aim was to study the anomalies related to size, shape and external features of the spleen and discuss their clinical importance. As there are not many reports on variations in size shape and features of spleen in the South Indian population, we decided to do this study on cadaveric specimens. We observed 50 spleens fixed with 10 percent formalin. Among the 50 spleens observed, 25 spleens (50 percent) were normal and had all the features explained in the textbooks. 25 spleens (50 percent) did not have any notches on the superior margin, 2 spleens (4 percent) did not have a hilum and 4 spleens (8 percent) were small sized i.e. about 3 inches long. One of the spleens (2 percent) was "liver shaped" with 2 lobes. The knowledge of external features and dimensions of spleen are important for radiologists and surgeons as it may lead to confusions in interpreting radiographs and in the procedures of ultrasound examinations.

El bazo es el mayor órgano linfoide. Tiene forma de cuña y mide alrededor de 2,5 cm de espesor, 7,5 cm de ancho y 12,5 cm de largo. Presenta incisuras características en su margen superior y tiene un hilio en la superficie visceral. Debido a que no existen muchos informes sobre las variaciones del bazo en la población del sur de la India, el objetivo de esta investigación fue estudiar dichas variaciones y discutir su importancia clínica. Se observaron 50 bazos fijados con formalina al 10 por ciento. 25 bazos (50 por ciento) fueron normales y tenían las características que se explican en los libros anatómicos. 25 bazos (50 por ciento) no tenían ninguna incisura en el margen superior, 2 bazos (4 por ciento) no tenían hilio y 4 bazos (8 por ciento) eran de tamaño pequeño, con alrededor de 7,2 cm de largo. Uno de los bazos (2 por ciento) tenía "forma de hígado" con dos lóbulos. El conocimiento de las características externas y las dimensiones del bazo son importantes para los radiólogos y cirujanos, ya que pueden dar lugar a confusiones en la interpretación de radiografías y en los procedimientos de examen ultrasonográfico.

Asplenia congénita y purpura fulminans neumocóccico en paciente pediátrico: reporte de caso con necropsia y revisión del tema / Congenital asplenia and pneumococcal purpura fulminans in a pediatric patient: case report with pathological findings and review

Absent or defective splenic function is associated with a high risk of fulminant bacterial infections, especially due to encapsulated bacteria. Not knowing this condition may delay medical treatment. Streptococcus pneumoniae is the leading cause of sepsis in these patients. Asplenic patients are at high risk for septic shock and eventually purpura fulminans, a life-threatening condition. We report the case of a 3 years oíd girl, with mitral stenosis and recurrent pneumonía that was admitted due to fever but in the next few hours presented hypotension, purpura and livedo reticularis. Laboratory test showed leucopenia (3.400/mm³), bandemia (43 percent of immature forms), thrombocytopenia, hypoprothombinemia and severe lactic acidosis (ph: 7.0 and lactic acid 11 mmol/1). The patient developed septic shock and multiorganic failure. Mechanical ventilation, volume resuscitation, vasoactive drugs and antibiotic therapy was initiated. Ultrasound was performed on the second day, demostrating asplenia. Peripheral blood smear showed Howell-Jolly bodies. Patient had a positive blood culture for penicillin-resistant Streptococcus pneumoniae (serotype 19F). Patient died of intracerebral hemorrhage after 8 days of admission. Necropsy confirmed asplenia and bilateral suprarenal haemorrhage. Absence of spleen can lead to life threatening infections, it is important to recognize it because vaccination and antibiotic prophylaxis can provide life-saving protection. This case provides a reminder to pursue asplenia as a potential underlying mechanism for invasive bacterial infection in children.

La condición de asplenia predispone a infecciones invasoras por bacterias capsuladas. Desconocer previamente ese antecedente dificulta y retarda el tratamiento médico. Streptococcus pneumoniae es el agente habitualmente causal de sepsis en estos pacientes. Los individuos asplénicos son particularmente proclives a evolucionar con shock séptico y eventual-mente al desarrollo de purpura fulminans, entidad altamente letal. Comunicamos el caso de una paciente con 3 años de edad y antecedente de cardiopatía y neumonías a repetición. Ingresó con compromiso sensorial, febril, hipotensa, con púrpura y livedo reticularis. En los exámenes de laboratorio destacaba la presencia de leucopenia (3.400/ mm³) trombopenia e hipoprotrombinemia (39 por ciento). Se inició ventilación mecánica, reanimación con volumen, fármacos vasoactivos y antibioterapia con vancomicina, clindamicina y ceftriaxona. Evolucionó con shock séptico refractario y síndrome de disfunción orgánica múltiple. Al segundo día de evolución una ecograña de abdomen comprobó la ausencia de bazo. En el frotis sanguíneo se evidenciaron corpúsculos de Howell-Jolly. Hemocul-tivo (+) S. pneumoniae resistente a penicilina (serotipo 19F). Un infarto hemorrágico cerebral ocasionó su deceso al octavo día. El estudio necrópsico corroboró la asplenia y evidenció necrohemorragia suprarrenal bilateral. La sepsis en un paciente asplénico puede ser de alguna forma prevenible mediante profilaxis antimicrobiana y vacunación neumocóccica. Dado los antecedentes de la paciente la búsqueda de asplenia era fundamental.

Baço acessório intrapancreático: achados de imagem / Intrapancreatic accessory spleen: imaging findings

Baços acessórios consistem em proliferações de tecido esplênico, com prevalência relatada de 10% a 30% em estudos de autópsias e de 45% a 65% em pacientes submetidos a esplenectomia. Quando este tecido se localiza em meio ao tecido pancreático, são denominados baços acessóriosintrapancreáticos, sendo esta a segunda localização mais frequente, representando cerca de 16,8%dos casos. Esta entidade apresenta um interesse particular, por tratar-se de l esão benigna, geralmente incidental, cujo diagnóstico diferencial deve ser feito com lesões primárias pancreáticas.Neste caso devem ser afastados tumores neuroendócrinos (de ilhotas pancreáticas), adenocarcinoma e metástases. Na investigação por imagem, os baços acessórios intrapancreáticos se manifestamcom aspecto semelhante ao baço nativo em todos os métodos. À ultrassonografia, aparecem como imagem circunscrita hipoecóica homogênea, e na tomografia computadorizada e ressonânciamagnética apresentam, respectivamente, densidade, características de sinal e realce semelhantes ao baço. Nos casos em que o aspecto de imagem permanecer inespecífico, o diagnósticopode ser sugerido pela cintilografia com hemácias marcadas com tecnécio-99m, atualmente considerado o exame padrão ouro. Todas estas modalidades fornecem elementos para o diagnósticocom segurança, evitando procedimentos invasivos desnecessários.

Accessory spleens consist of spleen tissue proliferations, with an estimated prevalence of 10% to 30% in autopsies studies and 45% to 65% in patients submitted to splenectomy. Accessory spleens can occur within the pancreatic tissue (intrapancreatic accessoryspleen), representing the second most frequent localization (about 16.8% of the cases). This entity is of particular interest, since it is a benign lesion, generally incidentally detected, that should be differentiated from pancreatic endocrine neoplasm, pancreatic adenocarcinoma and metastasis. Intrapancreatic accessory spleen has similar imaging manifestations to native spleen in all imaging modalities. At ultrasound, it appears as a homogeneous, hypoechoic, circumscribed image. At computed tomography and magnetic resonance imaging it has similar density, signal characteristics and enhancement pattern in comparison to the spleen. In somecases, where the image findings remain nonspecific, the diagnosis may be suggested by technetium-99m radiolabeled red blood cells scintigraphy, currently considered the gold standard test. This aspect allows a confident imaging diagnosis, preventing unnecessary invasive procedures.