Colangiopatía hipertensiva portal por transformación portal cavernomatosa de origen no cirrótica: a propósito de un caso

La colangiopatía portal hace referencia a anomalías colangiográficas que se producen en pacientes con cavernomatosis portal, siendo progresiva, cursando con enfermedad biliar sintomática y anomalías graves de las vías biliares. Y, representa una complicación infrecuente de la hipertensión portal. Se describe el caso de un hombre de 53 años, con historia de larga data de hipertensión portal nocirrótica y cavernomatosis portal, quien presentó un episodio de enfermedad biliar obstructiva sintomática, y en estudios se documentó tejido fibrótico de extensión periportal ascendente con compresión extrínseca del colédoco distal y dilatación de la vía biliar extra e intrahepática. Por lo que se procedió a colangiopancreatografía retrógrada endoscópica, realizándose tratamiento paliativo, con papilotomía pequeña y colocación de endoprótesis biliar plástica, siendo exitoso por ausencia de complicaciones procedimentales, y mejoría clínica y parámetros bioquímicos. Finalmente, recibiendo de alta con indicación de seguimiento prioritario para recambios periódicos de endoprótesis biliares, y valoración por hepatología. La colangiopatía portal es una entidad rara que debe sospecharse en sujetos con hipertensión portal de origen no-cirrótico, con hallazgos imagenológicos de estenosis, angulaciones o dilataciones segmentarias, su tratamiento debe ser individualizado, y la terapia endoscópica es de elección en enfermedad biliar sintomática.

Portal cholangiopathy refers to cholangiographic abnormalities occurring in patients with portal cavernomatosis, being progressive, presenting with symptomatic biliary disease and severe biliary tract abnormalities. And, it represents an infrequent complication of portal hypertension. We describe the case of a 53-year-old man with a long history of non-cirrhotic portal hypertension and portal cavernomatosis, who presented an episode of symptomatic obstructive biliary disease, and studies documented fibrotic tissue of ascending periportal extension with extrinsic compression of the distal common bile duct and dilatation of the extra and intrahepatic biliary tract. Therefore, endoscopic retrograde cholangiopancreatography was performed, and palliative treatment with small papillotomy and placement of a plastic biliary endoprosthesis was successful due to the absence of procedural complications, and clinical improvement and biochemical parameters. Finally, the patient was discharged with indication of priority follow-up for periodic replacement of biliary stents, and evaluation by hepatology. Portal cholangiopathy is a rare entity that should be suspected in subjects with portal hypertension of non-cirrhotic origin, with imaging findings of stenosis, angulations or segmental dilatations, its treatment should be individualized, and endoscopic therapy is of choice in symptomatic biliary disease.

Transjugular intrahepatic portosystemic shunt in decompensated cirrhotic patients in a tertiary hospital in southern Brazil

SUMMARY OBJECTIVE: The aim of the present study was to evaluate the outcomes of cirrhotic patients undergoing transjugular intrahepatic portosystemic shunt. METHODS: A retrospective longitudinal observational study was carried out evaluating 38 cirrhotic patients undergoing transjugular intrahepatic portosystemic shunt. The outcomes were evaluated in an outpatient follow-up period of 3 months. The assumed significance level was 5%. RESULTS: The indications for transjugular intrahepatic portosystemic shunt were refractory ascites in 21 (55.3%), variceal hemorrhage in 13 (34.2%), and hydrothorax in 4 (10.5%) patients. There was development of hepatic encephalopathy in 10 (35.7%) patients after transjugular intrahepatic portosystemic shunt. From the 21 patients with refractory ascites, resolution was observed in 1 (3.1%) patient, and in 16 (50.0%) patients, there was ascites control. Regarding transjugular intrahepatic portosystemic shunt after variceal bleeding, 10 (76.9%) patients remained without new bleeding or hospitalizations in the follow-up period. The global survival in the follow-up period in patients with and without hepatic encephalopathy was 60 vs. 82%, respectively (p=0.032). CONCLUSION: Transjugular intrahepatic portosystemic shunt can be considered in decompensated cirrhotic patients; however, the development of hepatic encephalopathy which can shorten survival should be focused.

Caracterización de pacientes con hepatopatías crónicas de etiología alcohólica mediante ecografía Doppler. Cienfuegos, 2020 / Characterization of patients with chronic liver disease of alcoholic etiology by Doppler ultrasound. Cienfuegos, 2020

RESUMEN Fundamento las hepatopatías crónicas constituyen enfermedades frecuentes a nivel mundial. La cirrosis hepática, cuya etiología más frecuente es el alcoholismo, representa el final de las lesiones hepáticas difusas crónicas y progresivas. Objetivo caracterizar los pacientes con hepatopatías crónicas alcohólicas mediante ecografía Doppler. Métodos se realizó un estudio descriptivo, de serie de casos, en 36 pacientes con diagnóstico clínico endoscópico y/o laparoscópico hepatopatías crónicas de etiología alcohólica, en el Hospital General Universitario Dr. Gustavo Aldereguía Lima, de Cienfuegos, en el período comprendido de enero a diciembre de 2020. Además de la edad y el sexo, se analizaron variables ecográficas según ecografía bidimensional (modo B) y Doppler. Resultados tuvieron mayor representatividad los pacientes masculinos y fue más numeroso el grupo etario de 42 a 51 años. El hígado y el bazo fueron de tamaño normal en la mayoría de los casos (44,5 % y 58,4 %, respectivamente). El hígado mostró ecoestructura predominantemente heterogénea (27,7 %) y nodular (58,4 %). Atendiendo al calibre de la porta extrahepática y de la vena esplénica, predominó la normalidad, ambas con 58,4 %. La ascitis, el derrame pleural y la circulación colateral no mostraron una frecuencia alta. Prevaleció la dirección de flujo hepatopedal (72,3 %) y velocidad de la porta normal (77,8 %). Conclusión en pacientes con hepatopatías crónicas de etiología alcohólica resulta de vital importancia la atención médica oportuna. La ecografía Doppler complementa la información morfológica aportada por la ecografía convencional.

ABSTRACT Background chronic liver diseases are frequent diseases worldwide. Liver cirrhosis, whose most frequent etiology is alcoholism, represents the end of chronic and progressive diffuse liver lesions. Objective to characterize patients with chronic alcoholic liver disease by Doppler ultrasound. Methods a descriptive case series study was carried out in 36 patients with clinical endoscopic and/or laparoscopic diagnosis of chronic hepatopathies of alcoholic etiology, at the Dr. Gustavo Aldereguía Lima General University Hospital, Cienfuegos, from January to December 2020. In addition to age and sex, ultrasound variables were analyzed according to two-dimensional ultrasound (B-mode) and Doppler. Results male patients were more representative and the age group from 42 to 51 years old was more numerous. The liver and spleen were of normal size in most cases (44.5% and 58.4%, respectively). The liver showed predominantly heterogeneous (27.7%) and nodular (58.4%) echostructure. Considering the caliber of the extrahepatic portal vein and the splenic vein, normality prevailed, both with 58.4%. Ascites, pleural effusion and collateral circulation did not show a high frequency. Hepatopedal flow direction (72.3%) and normal portal vein velocity (77.8%) prevailed. Conclusion in patients with chronic liver diseases of alcoholic etiology, timely medical care is of vital importance. Doppler ultrasound complements the morphological information provided by conventional ultrasound.

Portal hypertension as an uncommon presentation of hepatic epithelioid hemangioendothelioma: a case report

Hepatic epithelioid hemangioendothelioma (HEHE) is a vascular tumor with a low incidence rate. We report a case of a 26-year-old man who was referred to our hospital with a misdiagnosis of liver cirrhosis. On physical examination, ascites was noted. Chest and abdominal computer tomography scans showed coalescent lesions involving the peripheral liver with heterogeneous contrast enhancement and portal vein dilation due to portal hypertension. Extrahepatic metastasis was not observed. The biopsy with immunohistochemical stains suggested HEHE (Factor VIII, CD31, and CD34). This report describes an uncommon case of HEHE with non-cirrhotic portal hypertension.

El hemangioendotelioma epitelioide hepático (HEHE) es un tumor vascular con una tasa de incidencia baja. Presentamos el caso de un varón de 26 años que fue remitido a nuestro hospital con un diagnóstico erróneo de cirrosis hepática. En el examen físico se observó ascitis. La tomografía computarizada de tórax y abdomen mostró lesiones coalescentes que afectaban al hígado periférico con realce heterogéneo de contraste y dilatación de la vena porta por hipertensión portal. No se observó metástasis extrahepática. La biopsia con tinciones inmunohistoquímicas sugirió HEHE (Factor VIII, CD31 y CD34). Este informe describe un caso poco común de HEHE con hipertensión portal no cirrótica.

O índice esplênico como preditor de sangramento e recidiva varicosa no seguimento tardio de paciente esquistossomóticos após tratamento endoscópico exclusivo / The splenic index as predictor of bleeding and variceal recurrence in the late follow-up of schistosomotic patients after exclusive endoscopic treatment

RESUMO - RACIONAL: O tratamento endoscópico das varizes esofágicas tem sido utilizado como principal intervenção em pacientes com hipertensão portal secundária à esquistossomose, mas com taxas significativas de recorrência de varizes esofágicas e ressangramento. Os resultados em longo prazo do tratamento endoscópico exclusivo são pouco estudados quanto à relação das dimensões esplênicas neste contexto. OBJETIVO: Avaliar, por meio da ultrassonografia, o índice esplênico e a dimensão longitudinal (craniocaudal) do baço como preditores de ressangramento e recorrência de varizes no seguimento tardio de pacientes esquistossomóticos não operados, após erradicação endoscópica das varizes esofágicas. MÉTODOS: Estudo observacional retrospectivo por meio da análise de prontuários de pacientes com diagnóstico de esquistossomose hepatoesplênica. A curva ROC foi usada para determinar o melhor ponto de corte para o índice esplênico médio como preditor de recorrência e sangramento. RESULTADOS: Foram analisados 54 pacientes, durante o período de 2002 a 2018. O tempo médio de seguimento foi de 8 anos. O índice esplênico provou ser um teste sensível em valores acima de 144 como preditor de ressangramento. Na análise da dimensão longitudinal, o valor acima de 20 cm apresentou teste estatisticamente significativo para recorrência de varizes e valor acima de 19 cm apresentou-se como teste muito sensível e estatisticamente significativo para ressangramento. CONCLUSÃO: A análise do índice esplênico e da dimensão craniocaudal, obtidos por ultrassonografia, podem predizer recorrência de varizes e ressangramento após erradicação endoscópica exclusiva.

ABSTRACT - BACKGROUND: Endoscopic treatment for esophageal variceal has been used as the main intervention in patients with portal hypertension secondary to schistosomiasis, but with significant rates of recurrence of esophageal variceal and rebleeding. The long-term results of exclusive endoscopic treatment are poorly studied as the relationship of the splenic dimensions in this context. AIM: The aim of this study was to identify, through ultrasonography, whether the splenic index and the longitudinal (craniocaudal) dimension of the spleen are the predictors of rebleeding and variceal recurrence in late follow-up of patients with nonoperated schistosomiasis, after endoscopic eradication of esophageal variceal. METHODS: This is a retrospective and observational study analyzing the medical records of patients diagnosed with hepatosplenic schistosomiasis. The receiver operating characteristic curve was used to determine the best cutoff point for the mean splenic index as a predictor of recurrence and bleeding. Results: A follow-up of 54 patients were analyzed during the period from 2002 to 2018. The mean follow-up time was 8 years. The splenic index with value >144 was proved to be a sensitive test for rebleeding. In the analysis of the longitudinal dimension, the spleen length of >20 cm showed a statistically significant test for recurrence of variceal and a length >19 cm presented as a very sensitive and statistically significant test for rebleeding. CONCLUSION: Splenic index and craniocaudal dimension analysis, obtained by ultrasonography, can predict recurrence of varicose veins and rebleeding after exclusive endoscopic treatment.

Hipertensión portal no cirrótica: presentación de cuatro casos y revisión breve de la literatura / Non-cirrhotic portal hypertension: presentation of four cases and a brief review of the literatura

La hipertensión portal es un síndrome hemodinámico de la circulación venosa portal que condiciona múltiples complicaciones potencialmente mortales, debido a las alteraciones generadas en la circulación esplácnica y sistémica. En las últimas décadas se ha identificado la presencia de hipertensión portal en ausencia de cirrosis con un fenómeno de vasculopatía característico. Esta entidad puede clasificarse como prehepática, hepática y posthepática, según la localización del compromiso, identificado mediante estudios hemodinámicos. Se presentan cuatro casos de pacientes con hipertensión portal no cirrótica secundaria a trombosis portal. Adicionalmente, se realiza una revisión breve de la literatura, haciendo énfasis en las dos causas más frecuentes de hipertensión portal no cirrótica; la trombosis venosa portal crónica y la enfermedad vascular porto-sinusoidal.

Portal hypertension is a hemodynamic syndrome of the portal venous circulation that causes multiple life-threatening complications due to the alterations generated in the splanchnic and systemic circulation. In recent decades, the presence of portal hypertension in the absence of cirrhosis has been identified with a characteristic vasculopathy phenomenon. This condition can be classified as pre-hepatic, hepatic and post-hepatic, according to the location of the involvement, identified by hemodynamic studies. Four case reports of patients with non-cirrhotic portal hypertension secondary to portal thrombosis are presented. Additionally, a brief review of the literature is included, with emphasis in the two most frequent causes of non-cirrhotic portal hypertension; chronic portal venous thrombosis and portosinusoidal vascular disease.

The ABCD of portal vein thrombosis: a systematic approach.

Portal vein thrombosis refers to complete or partial obstruction of the portal venous system, in the intrahepatic or extrahepatic venous tract or even in the splenic or superior mesenteric veins. This common and potentially fatal condition can develop in various clinical contexts, especially those of liver cirrhosis, hepatocellular carcinoma, and other solid tumors. Certain characteristics, such as the time since the onset of the thrombus (acute or chronic), its biology (hematic or tumoral), the presence of collateral vessels, and the magnetic resonance imaging aspects, are important components of a thorough, careful analysis, as well as informing decisions regarding the appropriate therapeutic strategy. Here, we present a brief review of the anatomy of the portal venous system and a systematic approach to analyzing the condition, using a mnemonic (ABCD, for age, biology, collaterals, and diffusion). We discuss the various imaging methods and illustrate our discussion with images selected from the case files archived at our facility.

Trombose da veia porta refere-se à obstrução completa ou parcial do sistema venoso portal, localizada nos tratos venosos intra-hepáticos ou extra-hepáticos e até mesmo nas veias esplênica ou mesentérica superior. Vários contextos clínicos podem ser responsáveis pelo desenvolvimento desta condição frequente e potencialmente fatal, especialmente a cirrose hepática, o carcinoma hepatocelular e outros tumores sólidos. Algumas características como o tempo de aparecimento do trombo (agudo ou crônico), sua biologia (hemático ou tumoral), a presença de vasos colaterais e o seu comportamento na ressonância magnética são importantes para uma análise completa e criteriosa, assim como para o gerenciamento adequado da estratégia terapêutica. No presente artigo apresentamos breve revisão da anatomia do trato venoso portal, seguida de uma abordagem sistemática usando um mnemônico (ABCD) para análise da trombose da veia porta por diferentes métodos de imagem, utilizando imagens de casos selecionados do arquivo de ensino do nosso serviço.

The ABCD of portal vein thrombosis: a systematic approach / O ABCD da trombose da veia porta: uma abordagem sistemática

Abstract Portal vein thrombosis refers to complete or partial obstruction of the portal venous system, in the intrahepatic or extrahepatic venous tract or even in the splenic or superior mesenteric veins. This common and potentially fatal condition can develop in various clinical contexts, especially those of liver cirrhosis, hepatocellular carcinoma, and other solid tumors. Certain characteristics, such as the time since the onset of the thrombus (acute or chronic), its biology (hematic or tumoral), the presence of collateral vessels, and the magnetic resonance imaging aspects, are important components of a thorough, careful analysis, as well as informing decisions regarding the appropriate therapeutic strategy. Here, we present a brief review of the anatomy of the portal venous system and a systematic approach to analyzing the condition, using a mnemonic (ABCD, for age, biology, collaterals, and diffusion). We discuss the various imaging methods and illustrate our discussion with images selected from the case files archived at our facility.

Resumo Trombose da veia porta refere-se à obstrução completa ou parcial do sistema venoso portal, localizada nos tratos venosos intra-hepáticos ou extra-hepáticos e até mesmo nas veias esplênica ou mesentérica superior. Vários contextos clínicos podem ser responsáveis pelo desenvolvimento desta condição frequente e potencialmente fatal, especialmente a cirrose hepática, o carcinoma hepatocelular e outros tumores sólidos. Algumas características como o tempo de aparecimento do trombo (agudo ou crônico), sua biologia (hemático ou tumoral), a presença de vasos colaterais e o seu comportamento na ressonância magnética são importantes para uma análise completa e criteriosa, assim como para o gerenciamento adequado da estratégia terapêutica. No presente artigo apresentamos breve revisão da anatomia do trato venoso portal, seguida de uma abordagem sistemática usando um mnemônico (ABCD) para análise da trombose da veia porta por diferentes métodos de imagem, utilizando imagens de casos selecionados do arquivo de ensino do nosso serviço.

Tratamiento de quilotórax y quiloascitis refractaria en paciente cirrótico. Caso clínico

Chylous Ascites (CA) and chylothorax (CTx) are associated with obstruction, disruption or insufficiency of the lymphatic system. We report a 68-year-old male, with a history of alcoholic cirrhosis, who had recurrent events of CTx and CA. After a complete study, no other etiologies other than portal hypertension were found. Therapy with diuretics, nothing per mouth, parenteral feeding plus octreotide did not relieve symptoms. A transjugular intrahepatic portosystemic shunt (TIPS) was successfully placed and pleural effusion subsided. This case shows that CA and CTx can be caused by portal hypertension and they may subside employing a multimodal management strategy.

Elevated Gastric Antrum Erosions in Portal Hypertension Patients: Peptic Disease or Mucosal Congestion?

BACKGROUND/AIMS: Portal hypertension (PH) is a syndrome characterized by chronic increase in the pressure gradient between the portal vein and inferior vena cava. Previous studies have suggested an increased frequency of antral elevated erosive gastritis in patients with PH, as well as an etiologic association; however, there has not been any histological evidence of this hypothesis to date. Our aim was to evaluate the histological features found in elevated antral erosions in patients with portal hypertension. METHODS: Sixty-nine patients were included; 28 with and 41 without PH. All patients underwent endoscopy, and areas with elevated antral erosion were biopsied. RESULTS: In the PH group, 24 patients had inflammatory infiltration with or without edema and vascular congestion, and 4 patients had no inflammation. In the group without PH, all patients showed inflammatory infiltration of variable intensity. There was no statistical significance between the two groups in the presence of Helicobacter pylori. There as a histological similarity between the two groups, if PH patients without inflammation were excluded; however, more edema and vascular congestion were observed in the PH group (p=0.002). CONCLUSIONS: The findings show that elevated antral erosions in patients with PH have more evident edema and vascular congestion in addition to lymphocytic infiltration.

Acquired portosystemic collaterals: anatomy and imaging.

Portosystemic shunts are enlarged vessels that form collateral pathological pathways between the splanchnic circulation and the systemic circulation. Although their causes are multifactorial, portosystemic shunts all have one mechanism in common-increased portal venous pressure, which diverts the blood flow from the gastrointestinal tract to the systemic circulation. Congenital and acquired collateral pathways have both been described in the literature. The aim of this pictorial essay was to discuss the distinct anatomic and imaging features of portosystemic shunts, as well as to provide a robust method of differentiating between acquired portosystemic shunts and similar pathologies, through the use of illustrations and schematic drawings. Imaging of portosystemic shunts provides subclinical markers of increased portal venous pressure. Therefore, radiologists play a crucial role in the identification of portosystemic shunts. Early detection of portosystemic shunts can allow ample time to perform endovascular shunt operations, which can relieve portal hypertension and prevent acute or chronic complications in at-risk patient populations.

As vias colaterais ou shunts portossistêmicos são trajetos vasculares calibrosos de comunicação patológica entre a circulação esplâncnica e a sistêmica. Suas causas são multifatoriais, compartilhando um mecanismo de elevação da pressão venosa portal, a qual promove o desvio do fluxo sanguíneo do trato gastrintestinal para a circulação sistêmica. Múltiplas vias de colaterais estão descritas na literatura, sendo congênitas ou adquiridas. Ambas as causas, congênitas e adquiridas, resultam na redistribuição de volume vascular do trato gastrintestinal de veias sistêmicas e um aumento concomitante na pressão venosa portal. Os objetivos deste ensaio são: 1) discutir as características anatômicas e de imagem dos shunts portossistêmicos; 2) fornecer uma revisão robusta (com ilustrações e desenhos esquemáticos) para detectar e reconhecer os shunts portossistêmicos adquiridos. A importância do seu reconhecimento recai sobre o fato que, em alguns casos, eles são os únicos sinais que predizem a presença de hipertensão portal, sendo a avaliação do radiologista de grande valia na escolha de tratamentos endovasculares e na detecção de suas complicações.

Amyloidosis: an unusual cause of portal hypertension.

Amyloidosis comprises a group of diseases that occurs in five to nine cases per million patients per year worldwide irrespective of its classification. Although the hepatic involvement in primary amyloidosis is frequent, the clinical manifestations of liver amyloidosis are mild or even absent. The authors report the case of an aged man who complained of diffuse abdominal pain and marked weight loss and presented clinical signs of hepatopathy. Clinical workup revealed portal hypertension with ascites, hemorrhoids, and esophageal varices. The laboratory tests showed the cholestatic pattern of liver enzymes, hyperbilirubinemia, renal insufficiency and massive proteinuria accompanied by the presence of serum pike of monoclonal lambda light chain protein. The outcome was unfavorable, and the patient died. The autopsy findings revealed the diagnosis of amyloidosis predominantly involving the liver and kidneys. The bone marrow examination demonstrated the deposition of amyloid material associated with clonal plasma cells infiltration. The authors call attention to portal hypertension as a rare manifestation of primary amyloidosis. Meanwhile, this diagnosis should be taken into account whenever the hepatopathy is accompanied by laboratory abnormalities consistent with hepatic space-occupying lesions concomitantly with other organs involvement. In the case reported herein, kidney involvement was also present with renal failure, massive proteinuria with monoclonal serum gammopathy, what reinforced the diagnostic possibility of primary amyloidosis.

Acquired portosystemic collaterals: anatomy and imaging / Colaterais portossistêmicas adquiridas: aspectos anatômicos e imaginológicos

Abstract Portosystemic shunts are enlarged vessels that form collateral pathological pathways between the splanchnic circulation and the systemic circulation. Although their causes are multifactorial, portosystemic shunts all have one mechanism in common-increased portal venous pressure, which diverts the blood flow from the gastrointestinal tract to the systemic circulation. Congenital and acquired collateral pathways have both been described in the literature. The aim of this pictorial essay was to discuss the distinct anatomic and imaging features of portosystemic shunts, as well as to provide a robust method of differentiating between acquired portosystemic shunts and similar pathologies, through the use of illustrations and schematic drawings. Imaging of portosystemic shunts provides subclinical markers of increased portal venous pressure. Therefore, radiologists play a crucial role in the identification of portosystemic shunts. Early detection of portosystemic shunts can allow ample time to perform endovascular shunt operations, which can relieve portal hypertension and prevent acute or chronic complications in at-risk patient populations.

Resumo As vias colaterais ou shunts portossistêmicos são trajetos vasculares calibrosos de comunicação patológica entre a circulação esplâncnica e a sistêmica. Suas causas são multifatoriais, compartilhando um mecanismo de elevação da pressão venosa portal, a qual promove o desvio do fluxo sanguíneo do trato gastrintestinal para a circulação sistêmica. Múltiplas vias de colaterais estão descritas na literatura, sendo congênitas ou adquiridas. Ambas as causas, congênitas e adquiridas, resultam na redistribuição de volume vascular do trato gastrintestinal de veias sistêmicas e um aumento concomitante na pressão venosa portal. Os objetivos deste ensaio são: 1) discutir as características anatômicas e de imagem dos shunts portossistêmicos; 2) fornecer uma revisão robusta (com ilustrações e desenhos esquemáticos) para detectar e reconhecer os shunts portossistêmicos adquiridos. A importância do seu reconhecimento recai sobre o fato que, em alguns casos, eles são os únicos sinais que predizem a presença de hipertensão portal, sendo a avaliação do radiologista de grande valia na escolha de tratamentos endovasculares e na detecção de suas complicações.

Enfrentamiento de pacientes pediátricos con várices esofágicas / Management of pediatric patients with esophageal varices

Background: There is a paucity of good quality research about the diagnosis of esophageal varices and the prophylaxis and treatment of variceal bleeding in pediatric patients with portal hypertension There is little consensus and practically no evidence-based approach about the management of these patients. Aim: To describe the behavior and preferences of pediatric gastroenterologists in Chile in the management of portal hypertension in children. Material and Methods: An online survey was sent to Chilean pediatric gastroenterologists, with questions evaluating the physicians’ approaches to screening of esophageal varices in children with portal hypertension, and their preferred methods of prophylaxis and initial management of variceal bleeding. Results: Thirty five of 69 contacted physicians answered the survey (51%). Twenty nine pediatric gastroenterologists (83%) screen for esophageal varices in patients with clinical evidence of portal hypertension, and 12 (34%) in every patient with chronic liver disease. Twenty eight respondents (80%) use primary prophylaxis, mainly beta blockers. Octreotide, proton pump inhibitors and endoscopy are the most common practices in the initial management of an esophageal varix bleed. The methods mostly used as secondary prophylaxis are band ligation and beta blockers. In the case of recurrent hemorrhage, besides band ligation, management with Transjugular Intrahepatic Portosystemic Shunt (TIPS) and hepatic transplantation are more likely. Conclusions: Even though most pediatric gastroenterologists in this survey are inclined to offer endoscopic screening of esophageal varices and prophylaxis to patients with portal hypertension, this is not a universal behavior. There are different approaches mainly in the election of secondary prophylaxis and the initial management of variceal bleeding.

Budd-Chiari Syndrome: an unnoticed diagnosis.

Budd-Chiari syndrome (BCS) encompasses a group of disorders caused by the obstruction to the hepatic venous outflow at the level of the small or large hepatic veins, the inferior vena cava, or any combination thereof. Clinical manifestation of the subacute form is characterized by supramesocolic abdominal discomfort, abdominal distension, fever, and lower limbs edema. Imaging work-up with hepatic Doppler ultrasound and abdominal computed tomography (CT) enables the diagnosis in the majority of cases. Treatment comprises long-term anticoagulation associated with measures that attempt to re-establish the flow in the thrombosed vessel (thrombolysis or angioplasty) or through the venous blood flow bypasses (transjugular intrahepatic portosystemic shunt or surgical bypass); however, the outcome is often dismal. The authors report the case of a 37-year-old woman presenting a 2-month history of dyspeptic complaints and abdominal distention. Fever was present at the beginning of symptoms. The laboratory work-up disclosed mild hepatic dysfunction, and the ultrasound showed evidence of chronic liver disease. Despite a thorough etiologic investigation, diagnosis was missed and, therefore, management could not be directed towards the physiopathogenetic process. The outcome was characterized by portal hypertension and esophageal varices bleeding. The patient died and the autopsy findings were characteristic of BCS, although an abdominal CT, close to death, had showed signs consistent with this diagnosis. The authors highlight the importance of knowledge of this entity, the diagnostic methods, and the multidisciplinary approach. BCS should be considered whenever investigating etiology for chronic or acute hepatopathy.

Constriction rate variation produced by partial ligation of the portal vein at pre-hepatic portal hypertension induced in rats / Variação da taxa de constrição produzida pela ligadura parcial da veia porta na hipertensão portal pré-hepática induzida em ratos

BACKGROUND: Partial portal vein ligation causes an increase in portal pressure that remains stable even after the appearance of collateral circulation, with functional adaptation to prolonged decrease in portal blood flow. AIM: To assess whether different constriction rates produced by partial ligation of the vein interfere with the results of this experimental model in rats. METHODS: Three groups of five rats each were used; in group 1 (sham-operated), dissection and measurement of portal vein diameters were performed. Portal hypertension was induced by partial portal vein ligation, reducing its size to 0.9 mm in the remaining 10 animals, regardless of the initial diameter of the veins. Five animals with portal hypertension (group 2) underwent reoperation after 15 days and the rats in group 3 after 30 days. The calculation of the constriction rate was performed using a specific mathematical formula (1 - π r 2 / π R2) x 100% and the statistical analysis with the Student t test. RESULTS: The initial diameter of the animal's portal vein was 2.06 mm, with an average constriction rate of the 55.88%; although the diameter of the veins and the constriction rate in group 2 were lower than in group 3 (2.06 mm - 55,25% and 2.08 mm - 56.51%, respectively), portal hypertension was induced in all rats and no significant macroscopic differences were found between the animals that were reoperated after 15 days and after 30 days respectively, being the shorter period considered enough for the evaluation. Comparing the initial diameter of the vein and the rate of constriction performed in groups 2 and 3, no statistic significance was found (p>0.05). CONCLUSION: Pre-hepatic portal hypertension in rat can be induced by the reduction of the portal vein diameter to 0.9 mm, regardless the initial diameter of the vein and the vessel constriction rate. .

RACIONAL: A ligadura parcial da veia porta acarreta aumento da pressão portal que se mantém estável mesmo após o aparecimento da circulação colateral, havendo adaptação funcional à diminuição prolongada do fluxo sanguíneo hepático. OBJETIVO: Avaliar se diferentes taxas de constrição produzidas por esta ligadura interferem em modelo experimental no rato. MÉTODOS: Foram utilizados três grupos de cinco animais; no grupo 1 (sham-operated) foi realizada dissecção e medida do diâmetro da veia porta. A hipertensão portal foi induzida pela ligadura parcial da veia porta, reduzindo seu calibre para 0,9 mm nos 10 animais restantes, independente do diâmetro inicial das veias. Cinco animais com hipertensão portal (grupo 2) foram reoperados após 15 dias e os ratos do grupo 3 após 30 dias. O cálculo da taxa de constrição foi realizado através de fórmula matemática específica (1 - π r2 / π R2) x 100% e a análise estatística com o teste t de Student. RESULTADOS: O diâmetro médio inicial da veia porta dos animais foi de 2,06 mm, com taxa de constrição média de 55,88%; embora o diâmetro das veias e a taxa de constrição no grupo 2 tenha sido menor que no grupo 3 (2,06 mm - 55,25% e 2,08 mm - 56,51% respectivamente), em todos conseguiu-se induzir hipertensão portal e não foram encontradas diferenças macroscópicas significativas entre os animais reoperados após 15 ou 30 dias, sendo considerado o prazo menor suficiente para avaliação. Quando comparados o diâmetro inicial da veia e a taxa de constrição realizadas nos grupos 2 e 3 não houve significância estatística (p>0,05). CONCLUSÃO: A hipertensão portal pré-hepática no rato pode ser induzida pela redução de calibre da veia porta para 0,9 mm de diâmetro, independente do diâmetro inicial da veia e da taxa de constrição a que este vaso foi submetido. .

Evaluación de la realización de paracentesis en pacientes cirróticos ingresados a dos servicios de medicina interna de hospitales mediana complejidad / Assessment of the fulfillment of paracentesis in cirrotic patients admited in two services of internal medicine in hospitals of median complexity

La peritonitis bacteriana espontánea (PBE) es una complicación severa de la cirrosis hepática (CH). Su pronóstico depende del momento de inicio de la terapia para lo que se requiere un diagnóstico oportuno. Objetivo: Evaluar la realización de paracentesis diagnóstica (PD) en cirróticos con ascitis durante su hospitalización. Materiales y métodos: Estudio observacional, analítico y prospectivo, realizado en un período de 11 meses consecutivos. Resultados: Se registraron 92 ingresos, el promedio de edad fue de 60,3 años (DE 11,7), correspondiendo un 57,6% a hombres, la etiología de CH más frecuente fue alcohólica (48,9%). Se realizaron 40 PD (43,5%), de ellas 35% con PBE positiva. Del total de PD, un 47,5% se efectuaron al ingreso y/o al presentar signos de alarma. El promedio de días de hospitalización fue mayor en aquellos en que se realizó la PD tardíamente. El MELD (Model for the End stage Liver Disease) tanto al ingreso como egreso es significativamente mayor en los pacientes puncionados al ingreso. Conclusión: Se realiza PD en menos de la mitad de los cirróticos hospitalizados, siendo su realización oportuna solo en 1 de cada 5 de los ingresos. El realizar PD en el momento adecuado disminuye los días de hospitalización y morbimortalidad.

The Spontaneous Bacterial Peritonitis (SBP) is a severe complication of cirrhosis. The prognosis depends on the time of initiation of therapy that is required for early diagnosis. Objective: To evaluate the performance of diagnostic paracentesis (DP) in cirrhotic patients with ascites during hospitalization. Materials and methods: An observational, analytical, prospective, study conducted during October 2009 to June 2010. Results: There were 92 income, average age was 60.3 years (SD 11.7), corresponding to 57.6% men, the most common etiology of CH was alcohol (48.9%). There were 40 PD (43.5%), of which 35% positive SBP. Of the DP, 47.5% were performed on admission and / or submission of warning signs. The average period of hospitalization was higher in those who underwent late DP. The MELD score both at admission and discharge was significantly higher in patients on admission punctured. Conclusion: DP was done in less than half of hospitalized cirrhotic, and its timely completion only 1 in 5 of revenues. The DP perform at the right time reduces hospital days.

Eficacia de la gradiente de albúmina sangre-ascitis y los análisis de proteínas en líquido ascítico en el diagnóstico de ascitis hipertensiva portal: accuracy of the serum-ascites albumin gradient and protein analises in ascitic fluid / Diagnostic of ascites due to portal hypertension

Objetivo: Evaluar la exactitud diagnóstica de la gradiente albúmina sangre/ascitis (GASA), proteínas totales en líquido ascítico (PTLA), albúmina en líquido ascítico (CAA) e índice de proteínas ascitis/suero (IPAS) para el diagnóstico de ascitis por hipertensión portal. Materiales y métodos: Se realizó un estudio, observacional, retrospectivo, de validez de pruebas diagnósticas. La población estudiada fueron pacientes mayores de 15 años con diagnóstico de ascitis a los cuales se les tomó una muestra para estudio del líquido ascítico mediante la técnica estándar de paracentesis, analizando proteínas totales y albúmina, además de estudio de proteínas totales y albúmina en sangre en el Hospital de Salud Pública Nacional Daniel Alcides Carrión del Callao, Perú (HNDCA), durante el periodo de enero a diciembre del 2012. Se obtuvo la exactitud diagnóstica, sensibilidad, especificidad, VPP y VPN de la gradiente albumina sangre/ascitis (GASA), proteínas totales en líquido ascítico (PTLA), albúmina en líquido ascítico (CAA) e índice de proteínas ascitis/suero (IPAS) para el diagnóstico de ascitis por hipertensión portal o no HTP. Para determinar ascitis por HTP según las pruebas diagnósticas se tomo en cuentas: GASA≥1,1, PTLA<2,5, CAA<1,1 o IPAS<0,5. Resultados: se obtuvieron 126 pacientes con diagnóstico de ascitis a los cuales se excluyó 10 pacientes por tener datos incompletos. De los 116 pacientes finales la edad promedio fue de 53,03 ± 15,73 años, pacientes de sexo masculino fueron 65 (56%) y femenino 51 (44%). Se encontró 61 (52%) líquidos ascíticos debido a HTP por cirrosis hepática, y 55 (48%) de ascitis por NO HTP. La sensibilidad y especificidad para el GASA fue de 93% y 47% respectivamente, para PTLA fue de 80% y 89% respectivamente, para CAA fue de 85% y 87% respectivamente y para el IPAS fue de 83% y 80% respectivamente. El área bajo la curva ROC para el GASA fue de 0,70, de las PTLA fue de 0,84, del IPAS fue de 0,81 y de la CAA fue de 0,86; encontrándose diferencia estadísticamente significativa entre el GASA comparado con los otros tres parámetros (p<0,01). Conclusión: La exactitud diagnóstica de la CAA, PTLA y IPAS es superior a la del GASA para discriminar entre ascitis por HTP o NO HTP, por lo que podrían ser usados en la práctica clínica de forma aislada, o en conjunto para lograr una aproximación diagnóstica más acertada.

Objective: To evaluate the diagnostic accuracy of the Serum-Ascites Albumin Gradient (GASA), Protein Concentration in the Ascitic Fluid (PTLA), Albumin Concentration in the ascitic fluid (CAA) and the Protein Ascites/Serum Ratio (IPAS) for the diagnosis of ascites due to portal hypertension. Materials and methods: it was an observational and retrospective study of validation of diagnostic tests. The study population was patients from a National Public Health Hospital Daniel Alcides Carrion of Callao, Peru, during the period January to December of 2012, patients over 15 years old with a diagnosis of ascites which samples were taken for study by paracentesis with an standard technique, it was analyzed total protein and albumin, as well as study of total protein and albumin in blood. We obtained the diagnostic accuracy, sensitivity, specificity, PPV and NPV of the Serum-Ascites Albumin Gradient (GASA), Protein Concentration in the Ascitic Fluid (PTLA), Albumin Concentration in the ascitic fluid (CAA) and the Protein Ascites/Serum Ratio (IPAS) for the diagnosis of ascites due to portal hypertension. To determine ascites by HTP as diagnostic tests we took into account: GASA ≥ 1.1, PTLA <2.5, CAA <1.1 or IPAS< 0.5. Results: There were 126 patients diagnosed with ascites, 10 patients was excluded for having incomplete data. Of the 116 patients, the average age was 53.03 +/- 15.73 years old, male 65 (56%) and female 51 (44%). 61 (52%) had ascites due to portal hypertension from liver cirrhosis, and 55 (48%) of ascites due to NO HTP. The sensitivity and specificity for GASA was 93% and 47% respectively, for PTLA was 80% and 89% respectively, for CAA was 85% and 87% respectively and for the IPAS was 83% and 80% respectively. The area under the ROC curve for GASA was 0.70, ATPL was 0.84, IPAS was 0.81 and CAA was 0.86, we found statistically significant differences between GASA compared to the other three parameters (p<0.01 ). Conclusion: The diagnostic accuracy of CAA, ATPL and IPAS is higher than the GASA to discriminate between ascites due to HTP or NO HTP, so that they could be used in clinical practice alone or together to achieve a diagnostic approach more successful.

Hipertensão portal idiopática e suas alterações ultrassonográficas / Idiopathic portal hypertension and related ultrasound findings

Objetivo: caracterizar clínica e pela ultrassonografia (US) a hipertensão portal idiopática. Pacientes e métodos: foram avaliados, retrospectivamente, por US com doppler colorido, 60 pacientes com diagnóstico clínico de hepatopatia crônica e hipertensão portal, de janeiro de 1996 a janeiro de 2006, em clínica privada, encontrando dois casosde hipertensão portal idiopática. Comentários: em jovens com esplenomegalia, hematêmese recorrente, anemia, ascite, funções hepáticas normais e hipertensão portal sem evidência de cirrose, foram observados à US fígado de tamanho usual, textura heterogênea e superfície lisa, ausência de deformidades das ramificações das veias portais intra-hepáticas, espessamento da parede da veia porta maior ou igual a 3 mm e estreitamento abrupto das veias portais secundárias intra-hepáticas acompanhado de esplenomegalia.

Objective: to characterize idiopathic portal hypertension clinically and by ultrasound (US). Patients and methods: 60 patients with clinical diagnosis of chronic liver disease and portal hypertension were retrospectively assessed by color Doppler US, from January 1996 to January 2006, in a privately owned clinic. Two cases of idiopathic portal hypertension were found. Comments: in young patients with splenomegaly, recurring hematemesis, anemia, ascites, normal liver function and portal hypertension without evidence of cirrhosis, US revealed livers of usual size, heterogeneous texture and flat surface, without deformities in the branches of the intra-hepatic portal veins. The portal vein walls were also thickened starting at 3 mm and marked by an abrupt narrowing of the secondary intra-hepatic portal veins, accompanied by splenomegaly.

Aumento do volume do baço em modelo experimental de hipertensão portal pré-hepática em ratos / Increase in spleen volume of rats on experimental model of pre-hepatic portal hypertension

RACIONAL: O aumento da pressão no sistema venoso portal, induzido pela redução do aporte sanguíneo ao fígado, provoca o desenvolvimento de circulação colateral para que o sangue dos vasos esplâncnicos alcance o sistema venoso da veia cava superior. O baço funciona como um sistema de capacitância que auxilia a compensação do aumento da pressão portal, podendo ocorrer esplenomegalia. OBJETIVO: Reproduzir modelo experimental de hipertensão portal pré-hepática por meio da redução do calibre da veia porta do rato, permitindo o estudo das alterações de volume do baço nesta situação. MÉTODO: Foram utilizados 15 ratos da linhagem Wistar, divididos em três grupos de cinco. Todos os animais foram operados; os primeiros cinco foram utilizados como controle (grupo 1), e reoperados após 15 dias (sham-operated), assim como cinco animais onde foi induzida a hipertensão portal (grupo 2). Os cinco últimos animais, também com hipertensão portal, foram reoperados após 30 dias (grupo 3). A hipertensão portal do tipo pré-hepática foi provocada através da ligadura parcial da veia porta no hilo hepático. O baço foi medido durante a primeira e a segunda operação, permitindo o cálculo de seu volume nos três grupos estudados através de fórmula matemática específica. RESULTADOS: Não foram encontradas diferenças macroscópicas significativas ou alterações maiores do volume do baço quando foram comparados os animais que foram reoperados após 15 dias (grupo 2) e os reoperados após 30 dias (grupo 3), sendo considerado o prazo menor (15 dias), suficiente para avaliação das repercussões no volume após a ligadura parcial da veia porta. Embora o volume dos baços estudados tanto no grupo 2 quanto no grupo 3 tenha aumentado após a primeira operação - variando em média de 0,5417 cm³ para 0,6345 cm³ na reoperação, com aumento de aproximadamente 17% entre a primeira e a segunda fase do estudo -, estes resultados não apresentaram significância estatística (p>0,05). CONCLUSÃO: Não houve aumento com significância estatística do volume do baço após a ligadura parcial da veia porta quando se comparou o volume calculado na primeira operação com o volume encontrado na reoperação. Também não houve diferença quando foram comparados os volumes dos baços dos animais do grupo de reoperados após 15 dias, com o grupo reoperado após 30 dias.

BACKGROUND: Pressure increase in the portal venous system, caused by reduction of blood supply to the liver, develops collateral circulation of splanchnic vessel that reaches the venous system of superior vena cava. The spleen works as an auxiliary capacitance system which compensates the increase in portal pressure that may cause splenomegaly. AIM: To reproduce experimental model of pre-hepatic portal hypertension by reducing the caliber of the vein, allowing the study of alterations in the rat's spleen in this situation. METHODS: Fifteen Wistar rats were divided into three groups of five. All animals were operated, being the first five used as control (group 1) and reoperated after 15 days (sham-operated), along with five other animals had portal hypertension induced (group 2). The last five animals, also with portal hypertension were reoperated after 30 days (group 3). Prehepatic portal hypertension was caused in experimental animals by partial ligation of portal vein in hepatic hilum. The spleen was measured during the first and second operations, allowing the calculation of its volume in the three groups studied using specific mathematical formula. RESULTS: No macroscopic changes in spleen were significant when compared the animals reoperated after 15 days (group 2) to the ones after 30 days (group 3), being 15 days considered enough for the evaluation of effects on spleen after partial ligation. Although the volume of spleens studied in groups 2 and 3 have increased after the first operation - ranging on average from 0.5417 cm³ to 0.6345 cm³ at reoperation with increase of approximately 17% in volume between the first and second phase - the rsults were not statistically significant (p> 0.05). CONCLUSION: There was no statistically significant increase in the spleen volume after partial ligation of the portal vein when comparing the calculated volume in the first operation to the volume found at reoperation. There was no difference when comparing the spleen volumes of the animals reoperated after 15 days and the ones reoperated after 30 days.