Quiste hidático esplénico, una rara topografía de una rara enfermedad / Splenic hydatid cyst, a rare topography of a rare disease / Cisto hidatico esplénico, uma topografia rara de uma doença rara

Primary splenic hydatid cyst is a rare entity even in endemic areas. In most cases it presents asymptomatically being diagnosed by imaging findings. Treatment is multimodal and individualized, being surgical treatment the one that allows an integral solution of the disease. Conventional surgery continues to be the "Gold standard" in its approach. We present the case of a patient with this condition and the resolution approached by the surgical team.

O cisto hidatides esplénico primário é uma entidade rara mesmo em áreas endémicas. Na maioria dos casos apresenta-se de forma assintomática, sendo diagnosticada através de resultados de imagem. O tratamento é multimodal e individualizado, com um tratamento cirúrgico que proporciona uma solução abrangente para a condição. A cirurgia convencional continua a ser a abordagem "padrão de ouro". Apresentamos o caso de um paciente com esta condição e a resolução abordada pela equipa cirúrgica.

El quiste hidático primario esplénico es una entidad poco frecuente aún en áreas endémicas. Se presenta en la mayoría de los casos de forma asintomática, siendo diagnosticado por hallazgo imagenológico. El tratamiento es multimodal e individualizado, siendo el tratamiento quirúrgico el que permite una solución integral de la dolencia. La cirugía convencional sigue siendo el "Gold estándar" en su abordaje. Presentamos el caso de una paciente con esta afección y la resolución abordada por el equipo de cirugía.

Quiste esplénico gigante en una adolescente: Caso clínico / Giant splenic cyst in a teenager girl: Case report

Los quistes esplénicos gigantes y no parasitarios son infrecuentes. Estas lesiones pueden manifestarse como dolor abdominal, si bien a menudo son asintomáticas y se diagnostican en forma incidental. Objetivo: Presentar el caso de un quiste esplénico epitelial gigante por su interés en la toma de decisiones diagnósticas y terapéuticas, al ser una entidad muy poco frecuente.
 Caso clínico: Paciente de sexo femenino de 13 años de edad que consultó por dolor abdominal de dos meses de evolución. En la exploración física se palpaba una masa de consistencia dura en hemiabdomen izquierdo. En los estudios de imágenes se visualizó una masa esplénica quística gigante. Se realizó esplenectomía obteniendo un quiste subcapsular que comprometía la mayor parte del bazo, el estudio anatomopatológico e inmunohistoquímico fueron compatible con quiste epitelial. La evolución postoperatoria y el posterior seguimiento ambulatorio fueron favorables. Conclusiones: El tratamiento de los quiste esplénicos gigantes no parasitarios es quirúrgico, intentando conservar, en lo posible, la mayor cantidad de tejido esplénico. Este procedimiento debe ser individualizado, considerando el tamaño, las posibilidades diagnósticas, el tejido esplénico residual, la edad del paciente y comorbilidades.

Giant nonparasitic splenic epidermoid cysts are relatively uncommon. These lesions can lead abdominal pain, but most of then are asymptomatic, and they are discovered incidentally. We report a 13-y old female with a giant splenic epidermoid cystic, given the special interest of diagnostic and therapeutic decision-making of this rare entity. Case report: A 13-y old female with clinical history of abdominal pain since the last two months. On physical examination a firm, tender mass was palpable in left hypochondrium. Diagnosis of a large cystic splenic mass was made based on ultrasound and abdominal computed tomography scan. Splenectomy was performed, and histopathological-immunohistochemistry studies revealed findings suggestive of primary epithelial cyst. The post-operative clinical course was satisfactory and uneventful. Conclusions: Treatment of giant nonparasitic splenic cysts is surgical. Preserve splenic parenchyma must be the aim in an individualized decision-making. The different types of surgical modalities will be according to the diagnosis and clinical situation (cyst size, age, comorbidities).

Inflammatory pseudotumor of the spleen masquerading as splenic malignancy

Inflammatory pseudotumors [IPT[s]] of the spleen are extremely rare, benign tumors of unknown etiology, and are most frequently detected incidentally. We report a case of IPT of the spleen in a 19-year-old male, who presented to the Hamdard Institute of Medical Sciences and Research, New Delhi, with a history of pain and heaviness in the left hypochondrium. On clinical examination, splenomegaly was detected. Ultrasonography and contrast-enhanced computed tomography of the abdomen revealed an enlarged spleen with a mass lesion completely occupying the lower pole of the spleen. Therefore, a diagnosis of splenomegaly with a malignant splenic lesion was suggested. Open splenectomy was performed. On gross examination, a well-circumscribed nodular growth measuring 9 x 8 x 5 cm in diameter was seen on the lower pole of the spleen, which on cut section appeared tan white with foci of yellowish discoloration. Microscopic examination of the nodular growth revealed spindle cells in a hyalinized stroma with inflammatory infiltration of predominantly plasma cells and lymphocytes. On immunohistochemistry, the spindle cells were positive for smooth muscle actin. A diagnosis of IPT of the spleen was rendered following histopathology testing. Splenectomy is both diagnostic and curative for this rare entity, and prognosis is usually favorable following the procedure

A Case of Splenic Tuberculosis Forming a Gastro-splenic Fistula / 대한소화기학회지

We report a case of a 61-year-old man who presented with a cough and abdominal discomfort. CT scan of the chest showed two lesions across both lungs, and an abdominal CT scan revealed multiple hypodense lesions in the spleen with cystic lesions on the splenic hilum. Upper gastrointestinal tract endoscopy found creamy yellowish discharge through a fistula between the stomach and splenic hilum. Under fluoroscopic guidance, forceps was inserted into the fistula tract, and forcep biopsy was done. The pathology was consistent with tuberculosis, and a nine-month anti-tuberculosis medication regimen was started. Imaging performed three months after finishing medication indicated improvement of splenic lesions, and the gastro-splenic tract was sealed off. This case is a very rare clinical example of secondary splenic tuberculosis with a gastro-splenic fistula formation in an immunocompetent patient.

Schistosoma mansoni-Related Hepatosplenic Morbidity in Adult Population on Kome Island, Sengerema District, Tanzania

Schistosomiasis is one of the important neglected tropical diseases (NTDs) in Tanzania, particularly in Lake Victoria zone. This baseline survey was a part of the main study of integrated control of schistosomiasis and soil-transmitted helminths (STHs) aimed at describing morbidity patterns due to intestinal schistosomiasis among adults living on Kome Island, Sengerema District, Tanzania. Total 388 adults from Kome Islands (about 50 people from each village) aged between 12 and 85 years, were examined by abdominal ultrasound according to the Niamey protocol. Liver image patterns (LIPs) A and B were considered normal, and C-F as distinct periportal fibrosis (PPF). The overall prevalence of PPF was 42.2%; much higher in males than in females (47.0% in male vs 34.4% in females, P=0.007). Abnormal increase of segmental branch wall thickness (SBWT) and dilated portal vein diameter (PVD) were also more common in males than in females. Hepatosplenomegaly was frequently encountered; 68.1% had left liver lobe hepatomegaly and 55.2% had splenomegaly. Schistosoma mansoni-related morbidity is quite high among adults in this community justifying the implementation of integrated control strategies through mass drug administration, improved water supply (pumped wells), and health education that had already started in the study area.

Revisiting the concept of hepatosplenic schistosomiasis and its challenges using traditional and new tools

Different aspects of hepatosplenic schistosomiasis are revisited here. Manson's schistosomiasis causes periportal fibrosis and portal hypertension in approximately 6% of infected subjects, usually with preservation of their hepatic function. The assessment of liver involvement is of major importance in determining the prognosis and risk of complications from schistosomiasis, such as upper digestive bleeding secondary to variceal rupture. For many years, the diagnosis of hepatosplenic schistosomiasis and liver fibrosis was made by abdominal palpation and the finding of liver and/or spleen enlargement. However, there is no consensus regarding the clinical parameters of the liver and spleen to be considered in this physical evaluation. For the last three decades, abdominal ultrasound (US) has become the best imaging technique to evaluate liver fibrosis caused by schistosomiasis mansoni. However, US is a subjective procedure and is therefore examiner-dependent. Magnetic resonance imaging (MRI) findings have provided valuable information in addition to ultrasound and clinical examination. The combination of a comprehensive history and physical examination, basic laboratory tests (a stool examination for Schistosoma mansoni eggs and a blood cell count), biomarkers for liver fibrosis/portal hypertension and imaging methods seem to offer the best approach for evaluating patients with this disease. In situations where research is involved or in patients with severe disease, MRI may be considered.

Thrombocytopenia as a surrogate marker of hepatosplenic schistosomiasis in endemic areas for Schistosomiasis mansoni

Introduction This study aimed to evaluate whether a low platelet count is a good surrogate marker of hepatosplenic schistosomiasis (HSS) in a rural area of Brazil. A small district in southeastern Brazil, with a population of 1,543 individuals and a 23% prevalence of schistosomiasis, was selected for this investigation. Methods In July 2012, 384 volunteers were subjected to clinical, ultrasonography (US), and laboratory examinations, including stool sample analysis. The HSS patients were classified into four groups: Group 1 consisted of patients with a spleen >13cm and liver fibrosis; Group 2 consisted of patients with a palpable spleen and spleen>13cm measured by US; Group 3 consisted of patients with a spleen >13cm measured by US; and Group 4 consisted of patients with a palpable spleen. Results Eight patients were in Group 1 (2.1%), twenty-one were in Group 2 (5.5%), eight were in Group 3 (2.1%), and eighteen were in Group 4 (4.7%). A significant difference in the mean platelet counts was observed between the patients with and without HSS (p<0.01). Based on the receiver operating characteristic (ROC) curve (platelet count <143,000/mm3), the sensitivity was greater than 92% in all groups, and the specificity varied from 44.4% to 75%. Conclusions We concluded that in endemic areas, thrombocytopenia demonstrates good sensitivity for detecting HSS and may be used as a screening tool to identify patients with HSS. .

A Case of Splenic Hamartoma Diagnosed by Contrast-enhanced Ultrasonography and Magnetic Resonance Imaging / 대한소화기학회지

Splenic hamartoma is a very rare benign tumor, which is usually found incidentally after splenectomy or autopsy. Although percutaneous needle biopsy can be performed, it carries a high risk of bleeding after the procedure. Therefore, diagnosis is usually made by surgical resection. Herein, we report a case of splenic hamartoma diagnosed by magnetic resonance imaging and contrast-enhanced ultrasonography, which enables visualization of the unique signals of microbubbles in the vessels in real time. Relevant literature is also reviewed.

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.

Clinical characteristics and the usefulness of the QuantiFERON-TB Gold In-Tube test in hematologic patients with hepatic or splenic lesions

BACKGROUND/AIMS: Hepatic or splenic lesions in hematologic patients are not defined well because they are not easy to evaluate due to limitations of invasive procedures. Management typically depends on the clinical diagnosis with few microbiological data. METHODS: We reviewed the medical records of consecutive hematologic patients with hepatic or splenic lesions in the infectious diseases unit from April 2009 to December 2010 at the Catholic Hematopoietic Stem Cell Transplantation Center in Korea. RESULTS: Twenty-six patients were identified. Their mean age was 46.0 +/- 14.7 years, and 16 (61.5%) were male. Underlying diseases were acute myelogenous leukemia (n = 15, 57.7%) and myelodysplastic syndrome (n = 6, 23.1%). Among the nine nontuberculous infectious lesions, two bacterial, six fungal, and one combined infection were identified. The numbers of confirmed, probable, and possible tuberculosis (TB) cases were one, three, and four, respectively. Two patients had concurrent pulmonary TB. QuantiFERON-TB Gold In-Tube (QFT-GIT, Cellestis Ltd.) was positive in seven cases, among which six were diagnosed with TB. The sensitivity and specificity of QFT-GIT were 75% and 81.3%. Nine (34.6%) were defined as noninfectious causes. CONCLUSIONS: Causes of hepatic or splenic lesion in hematologic patients were diverse including TB, non-TB organisms, and noninfectious origins. TB should be considered for patients not responding to antibacterial or antifungal drugs, even in the absence of direct microbiological evidence. QFT-GIT may be useful for a differential diagnosis of hepatosplenic lesions in hematologic patients.

Cisto esplênico não parasitário: tratamento por laparoscopia / Non-parasitic splenic cyst: treatment by laparoscopy

Contexto: Os cistos esplênicos não parasitários são eventos raros, muitas vezes assintomáticos e encontradosincidentalmente durante exames de imagem.Descrição do caso: Paciente do sexo feminino, 25 anos, parda, foi atendida pelo Grupo de Fígado e Hipertensão Portalda Faculdade de Ciências Médicas da Santa Casa de São Paulo, e o quadro clínico inicial corroborou com o achadoincidental do cisto. O diagnóstico foi confirmado pela ressonância nuclear magnética de abdome superior e pelo examehistopatológico de fragmento obtido no intraoperatório. O tratamento instituído foi o destelhamento do cisto por viavideolaparoscópica, com a colocação de epíplon no interior do leito cístico.Discussão: A paciente encaixa-se no clássico quadro de achado incidental em exames de imagem, sendo os primeirossintomas inespecíficos. Além disso, este caso se sobressai devido à sua raridade, tanto entre os vistos diariamenteno serviço como nos casos descritos na literatura. Foi optado pelo tratamento videolaparoscópico que apresentouexcelente resultado, com melhora dos sintomas, tempo cirúrgico curto e evitou a esplenectomia total.Conclusões: Os cistos esplênicos são achados incidentais dentro de um quadro clínico assintomático ou inespecífico.Apesar da raridade, devem estar sempre no acervo de conhecimento de todo médico para que a conduta seja adequadae os resultados satisfatórios.

Isolated tubercular splenic abscess: Can we defer splenectomy? Our single experience with anti-tuberculous therapy alone.

Isolated splenic tuberculosis is an exceedingly rare clinical condition. Microbiological confirmation of diagnosis in such cases is quite difficult. We encountered the case of a 35-year-old female, who presented with persistent low-grade fever and weight loss. The CT scan of the abdomen revealed multiple hypodense splenic lesions. No primary focus of infection was detected in any other organs. Fine needle aspiration of splenic lesion revealed acid-fast bacilli on Ziehl-Neelsen stain. With anti-tuberculous therapy, the lesions regressed significantly in size. We stress that splenic tuberculosis should be considered as a diagnostic possibility even in immunocompetent individuals and choose combination antituberculous therapy as the first line treatment with consideration of splenectomy depending on response.

Two Cases of Primary Splenic Hydatid Cyst in Greece

Cystic disease of the spleen is an uncommon entity in general population. Most cases result from parasitic infection by Echinococcus granulosus, a form called splenic hydatid disease (SHD), with a reported frequency of 0.5-6.0% within abdominal hydatidosis. On the contrary, an isolated splenic involvement of hydatid disease is very uncommon even in endemic regions. Two cases of primary SHD managed with open and laparoscopic radical surgery in our department are reported herein. Primary SHD is a rare entity with non-specific symptoms underlying clinical suspicion by the physician for prompt diagnosis. Surgical treatment is the mainstay therapy, while laparoscopic approach when feasible is safe, offering the advantages of laparoscopic surgery.

Splenic Abscess: A Single Institution Study and Review of the Literature

PURPOSE: The aim of this study was to review our experience with splenic abscesses, with respect to the relevant aspects of splenic abscesses and treatment outcomes. MATERIALS AND METHODS: We reviewed the cases of 18 patients who had splenic abscesses and who were treated at our hospital from November 1993 to December 2008. RESULTS: The most common symptom at presentation was abdominal pain in 12 patients (66.7%). The median duration from symptom onset until establishment of a diagnosis was 22 days. Streptococcus viridians was the most common pathogen (27.8%), follow by Klebsiella pneumoniae (22.2%). The mortality rate during the inpatient period and the previous 90 days was 16.6%. Three of four patients with Klebsiella pneumoniae showed a single abscess pocket. Four patients (22.2%) underwent percutaneous drainage, eight (44.5%) recieved antibiotic treatment only and six (33.3%) underwent splenectomy. CONCLUSION: There is no gold standard for treating splenic abscesses. Treatment should be customized for each patient.

Glomerulonephritis in schistosomiasis mansoni: a time to reappraise / Glomerulonefrite na esquistossomose mansônica: um tempo para reavaliar

INTRODUCTION: The current prevalence of glomerulonephritis in patients with hepatosplenic schistosomiasis mansoni in Brazil was evaluated. METHODS: Sixty three patients (mean age 45.5±11 years) attending the outpatient infectious disease clinic of a University Hospital in Belo Horizonte, Brazil, from 2007 to 2009, were consecutively examined and enrolled in the present investigation. Diagnosis of hepatosplenic schistosomiasis was based on epidemiological, clinical and parasitological data and imaging techniques. Eight patients, who presented >30mg/day albuminuria, were submitted to percutaneous ultrasound guided renal biopsy. Kidney tissue fragments were examined under light, direct immunofluorescence and electron microscopy. RESULTS: All patients showed mesangial enlargement. In five, mesangial hypercellularity was observed and four presented duplication of the glomerular basement membrane. Areas of glomerular sclerosis were diagnosed in four. Deposits of immunoglobulin M and C3 were present in six samples; deposits of IgG in four, IgA in three and C1q in two samples. In all patients, immunoglobulin A was reported in the lumen of renal tubules. Deposits of kappa and lambda were observed in six samples. Electron microscopy revealed dense deposits in the glomerular tissue of three patients. Arterial hypertension, small esophageal varices, slight increases in serum creatinine and decreases in serum albumin were associated with glomerular disease. CONCLUSIONS: Renal disease associated with hepatosplenic schistosomiasis was verified in 12.7 percent of patients and type I membranoproliferative glomerulonephritis was observed in 50 percent of them. Schistosomal glomerulopathy still is an important problem in patients with hepatosplenic schistosomiasis in Brazil.

INTRODUÇÃO: Avaliou-se a frequência de glomerulonefrite em pacientes com esquistossomose hepatosplênica no Brasil. MÉTODOS: Selecionou-se para o estudo, 63 pacientes (idade média de 45,5±11 anos) avaliados consecutivamente no ambulatório de doenças infecciosas de um hospital universitário de Belo Horizonte, Brasil, no período de 2007 a 2009. O diagnóstico da esquistossomose foi baseado em dados epidemiológicos, clínicos, parasitológicos e de imagem. Os oito pacientes que apresentaram albuminúria acima de 30mg em 24 horas submeteram-se a biópsia renal percutânea dirigida por ultrassonografia. As amostras de tecido renal foram analisadas à microscopia óptica, eletrônica e de fluorescência direta. RESULTADOS: Havia expansão do mesângio em todos. Em cinco, houve proliferação de células mesangiais e em quatro observou-se duplicação da membrana basal glomerular. Áreas de esclerose glomerular foram diagnosticadas em quatro. Depósitos de imunglobulinas M e C3 foram patentes em seis amostras; IgG em quatro, IgA em três e C1q em duas. Em todos os pacientes relatou-se fluorescência para IgA dentro dos túbulos renais. Depósitos de kappa e lambda foram vistos em seis amostras. A microscopia eletrônica demonstrou depósitos eletrondensos em tecido glomerular. A presença de hipertensão arterial, varizes do esôfago de pequeno calibre, pequenos aumentos de creatinina e diminuição de albumina sérica associaram-se à ocorrência de dano renal. CONCLUSÕES: A frequência de lesão renal foi de 12,7 por cento, no presente estudo, e a glomerulonefrite membranoproliferativa do tipo I foi encontrada em 50 por cento. A lesão renal associada à esquistossomose permanece um problema importante no Brasil.

Cistos esplênicos não-parasitários / Non-parasitic splenic cysts

Este artigo apresenta uma revisão geral do tema, com ênfase em conceitos atuais e no manejo das situações clínicas em questão, em especial a conceitos referentes ao tratamento. Também é feita uma revisão extensa quanto à etiologia e seguimento dos pacientes com cisto esplênico não parasitário. São citadas informações derivadas dos principais estudos clínicos publicados na literatura médica atual.

This article gives an overview of the topic, with emphasis on current concepts and management of the clinical situations in question, in particular the concepts related to treatment. An extensive review of the etiology and monitoring of patients with non-parasitic splenic cysts is also made. These reports are derived from major clinical studies published in the current medical literature.

Comparison between clinical and ultrasonographic findings in cases of periportal fibrosis in an endemic area for schistosomiasis mansoni in Brazil / Comparação entre achados clínicos e ultrassonográficos no diagnóstico da fibrose periportal em uma área endêmica de esquistossomose no Brasil

INTRODUCTION: Abdominal palpation and ultrasound findings among patients from an endemic area for schistosomiasis in Brazil who had been followed up for 27 years were compared. METHODS: In 2004, 411 patients from Brejo do Espírito Santo, in the State of Bahia, were selected for the present investigation after giving their written informed consent. Based on clinical data, they were divided into three groups: 41 patients with evidence of liver fibrosis in 2004 (Group 1); 102 patients with evidence of liver fibrosis in the past (1976-1989) but not in 2004 (Group 2); and 268 patients without evidence of liver fibrosis at any time during the 27-year follow-up (Group 3). All of the patients underwent abdominal ultrasound in which the examiner did not know the result from the clinical examination. The data were stored in a database. RESULTS: The prevalence of periportal fibrosis on ultrasound was 82.9 percent, 56.9 percent and 13.4 percent in Groups 1, 2 and 3, respectively. In the presence of hard, nodular liver or prominent left lobe and a hard palpable spleen, ultrasound revealed periportal fibrosis in 70.9 percent. However, periportal fibrosis was diagnosed using ultrasound in 25.4 percent of the patients in the absence of clinical evidence of liver involvement. Thus, ultrasound diagnosed periportal fibrosis 3.1 times more frequently than clinical examination did. CONCLUSIONS: Although clinical examination is important in evaluating morbidity due to Manson's schistosomiasis in endemic areas, ultrasound is more accurate in diagnosing liver involvement and periportal fibrosis.

INTRODUÇÃO: Neste estudo, se comparou os achados da palpação abdominal e do ultrassom em pacientes de área endêmica de esquistossomose que foram acompanhados por 27 anos no Brasil. MÉTODOS: Em 2004, 411 pacientes de Brejo do Espírito Santo, no estado da Bahia, após consentimento informado e por escrito foram selecionados para o presente estudo. Baseando-se no exame clínico eles foram divididos em 3 grupos: 41 (Grupo 1) com evidência de fibrose hepática no ano de 2004; 102 (Grupo 2) com evidência de fibrose hepática no passado (1976-1989) mas não em 2004; e 268 (Grupo 3) sem evidência de fibrose hepática em 27 anos de seguimento. Todos foram submetidos a exame ultrassonográfico do abdome em que o examinador não sabia o resultado do exame clínico. Os dados foram armazenados em banco de dados. RESULTADOS: A prevalência de fibrose periportal ao ultrassom foi de 82,9 por cento, 56,9 por cento e 13,4 por cento nos Grupos 1, 2 e 3, respectivamente. Na presença de fígado duro, nodular ou lobo esquerdo proeminente e baço palpável duro, o ultra-som revelou fibrose periportal em 70,9 por cento. Porém, fibrose periportal foi diagnosticada através do ultrassom em 25,4 por cento dos pacientes, na ausência de evidência clínica de envolvimento hepático. Assim, o ultrassom diagnosticou fibrose periportal 3,1 vezes mais frequentemente que o exame clínico. CONCLUSÕES: O exame clínico tem importância na avaliação da morbidade da esquistossomose mansônica em áreas endêmicas, mas o ultrassom mostra-se mais preciso quando se pretende diagnosticar o envolvimento hepático e a fibrose periportal.