Urgent liver retransplantation due to transmission of intrahepatic cholangiocarcinoma by donor: the first report in the literature / Retransplante de fígado urgente devido à transmissão de colangiocarcinoma intra-hepático pelo doador: o primeiro relato da literatura

ABSTRACT BACKGROUND: Liver transplantation represents the best therapeutic modality in end-stage chronic liver disease, severe acute hepatitis, and selected cases of liver tumors. AIMS: To describe a double retransplant in a male patient diagnosed with Crohn's disease and complicated with primary sclerosing cholangitis, severe portal hypertension, and cholangiocarcinoma diagnosed in the transplanted liver. METHODS: A 48-year-old male patient diagnosed with Crohn's disease 25 years ago, complicated with primary sclerosing cholangitis and severe portal hypertension. He underwent a liver transplantation in 2018 due to secondary biliary cirrhosis. In 2021, a primary sclerosing cholangitis recurrence was diagnosed and a liver retransplantation was indicated. Recipient's hepatectomy was very difficult by reason of complex portal vein thrombosis requiring extensive thromboendovenectomy. Intraoperative ultrasound with liver doppler evaluation was performed. Two suspicious nodules were incidentally diagnosed in the donor's liver and immediately removed for anatomopathological evaluation. RESULTS: After pathological confirmation of carcinoma, probable cholangiocarcinoma, at frozen section, the patient was re-listed as national priority and a new liver transplantation was performed within 24 hours. The patient was discharged after 2 weeks. CONCLUSIONS: The screening for neoplasms in donated organs should be part of our strict daily diagnostic arsenal. Moreover, we argue that, for the benefit of an adequate diagnosis and the feasibility of a safer procedure, the adoption of imaging tests routine for the liver donor is essential, allowing a reduction of the costs and some potential risks of liver transplant procedure.

RESUMO RACIONAL: O transplante de fígado representa a melhor modalidade terapêutica na doença hepática crônica terminal, hepatite aguda grave e casos selecionados de tumores hepáticos. OBJETIVOS: Descrever um retransplante duplo em paciente do sexo masculino, diagnosticado com doença de Crohn e complicado com colangite esclerosante primária, hipertensão portal grave e colangiocarcinoma diagnosticado no fígado transplantado. MÉTODOS: Paciente do sexo masculino, 48 anos, diagnosticado com doença de Crohn há 25 anos e complicado com colangite esclerosante primária e hipertensão portal grave. Foi submetido a um transplante de fígado em 2018 devido a cirrose biliar secundária. Em 2021, foi diagnosticada recidiva de colangite esclerosante primária e indicado retransplante hepático. A hepatectomia do receptor foi de alta complexidade devido à trombose complexa da veia porta, exigindo extensa tromboendovenectomia. Foi realizada ultrassonografia intraoperatória com doppler hepático. Dois nódulos suspeitos foram diagnosticados incidentalmente no fígado do doador e imediatamente removidos para avaliação anatomopatológica. RESULTADOS: Após confirmação patológica de carcinoma, provável colangiocarcinoma, pela congelação, o paciente foi relistado como prioridade nacional, e novo transplante hepático foi realizado em 24 horas. O paciente teve alta após 2 semanas. CONCLUSÕES: O rastreamento de neoplasias em órgãos doados deve fazer parte de nosso estrito arsenal diagnóstico diário. Além disso, defendemos que, em benefício de um diagnóstico correto e da viabilidade de um procedimento mais seguro, a adoção de uma rotina de exames de imagem é essencial em doadores hepáticos, permitindo a redução dos custos e alguns riscos potenciais do procedimento de transplante hepático.

Frecuencia de síndrome hepatopulmonar en pacientes pediátricos trasplantados en un hospital de alta complejidad en Medellín, Colombia / Frequency of hepato-pulmonary syndrome in pediatric transplanted patients in a high complexity hospital in Medellín, Colombia

Introducción. El síndrome hepatopulmonar (SHP) es una complicación grave en los pacientes con enfermedad hepática crónica y/o hipertensión portal. La frecuencia descrita en adultos es entre 4 % y 47 %. En pediatría, los reportes también son muy variables y van desde el 3 % hasta el 40 %, desconociendo la real incidencia de este. El objetivo de esta descripción es conocer la frecuencia del SHP en pacientes pediátricos en un hospital de alta complejidad, por medio de una búsqueda activa del SHP en los estudios pretrasplante hepático hallados en las historias clínicas. Metodolo-gía. Estudio retrospectivo de 5 años, en un hospital de alta complejidad en Colombia, en menores de 15 años con un primer trasplante hepático. Resultados. Se contó con la información de 24 pacientes, se analizaron variables demográficas y se confirmó el SHP en 18 pacientes (75 %), encontrando una gravedad leve o moderada en el 33 % y 44 %, respectivamente, siendo en este grupo la cirrosis con complicaciones por hipertensión portal la indicación más frecuente para el trasplante, y como etiología de base, la atresia de vías biliares en un 61 %. Conclusión. El SHP en nuestra población se encontró con una alta frecuencia de presentación, por encima de lo reportado en la literatura, llevando a recomendar una búsqueda activa, con el objetivo de brindar un manejo integral y oportuno.

Introduction. Hepatopulmonary syndrome (HPS) is a serious complication in patients with chronic liver disease and/or portal hypertension. The frequency described in adults is between 4% and 47%. In pediatrics, reports are also highly variable and range from 3% to 40%, with the real incidence not clear yet. The objective of this study is to know the frequency of HPS in our population through an active search for HPS in pre-liver transplant studies in clinical records. Methodology. This is a 5-year retrospective study, in a reference hospital in Colombia, that included children under 15 years of age with a first liver transplant. Results. In 24 patients, the information was available, demographic variables were analyzed, and HPS was confirmed in 18 patients (75%), finding mild and moderate severity in 33% and 44%, respectively. In this group, cirrhosis with complications due to portal hyper-tension was the most frequent indication for transplantation and biliary atresia was the main etiology in 61%. Conclusion. HPS in our population was found with a high frequency, higher than is described in the literature, leading to the recommendation of an active search, with the aim of providing com-prehensive and timely management.

Remisión a largo plazo de la hipertensión portopulmonar mediante trasplante hepático en paciente con cirrosis asociada a hepatitis autoinmune / Long term remission of portopulmonary hypertension with liver transplantation in a patient with cirrhosis associated to autoimmune hepatitis

La hipertensión portopulmonar (HTPP) es una complicación infrecuente de la hipertensión portal, que sigue un curso progresivo con un pronóstico sombrío. Los reportes en pacientes pediátricos son escasos y con períodos de seguimiento cortos. Se describe una paciente con cirrosis descompensada que desarrolló HTPP resuelta mediante trasplante hepático, que permanece asintomática tras diez años de seguimiento.

Portopulmonary hypertension is an uncommon complication of portal hypertension, running a progressive course with a negative prognosis. Reports in pediatric patients are scarce with short follow up. We describe the case of decompensated cirrhosis who developed PoPH and resolved with liver transplantation, remaining asymptomatic after ten years of follow up.

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.

Síndrome de Budd-Chiari por obstrução da via de saída da veia hepática por membrana: tratamento percutâneo por angioplastia com balão / Budd-Chiari syndrome due to hepatic venous web outflow obstruction: percutaneous treatment with balloon angioplasty

Resumo A síndrome de Budd-Chiari é uma doença venosa hepática rara, mais incidente em adultos jovens, podendo se apresentar na forma aguda, subaguda ou crônica, o que resulta em hipertensão portal. O tratamento tradicional consiste em técnicas de trombólise e de shunts portossistêmicos intra-hepáticos, como pontes para o transplante hepático. Recentemente, técnicas de angioplastia com balão ou stents têm sido relatadas para o tratamento dessa afecção. Neste artigo, é relatado e discutido um caso de síndrome de Budd-Chiari por obstrução membranosa da via de saída da veia supra-hepática com trombose da veia hepática média em uma paciente de 24 anos. O tratamento estabelecido foi a angioplastia transjugular com balão, que obteve resultados satisfatórios e boa evolução clínica.

Abstract The Budd-Chiari syndrome is a rare hepatic venous disease. It is more prevalent in young adults and may present in acute, subacute, or chronic forms, causing portal hypertension. Traditional treatment consists of thrombolysis techniques and transjugular intrahepatic portosystemic shunt, as a bridge to liver transplantation. Recently, use of balloon or stent angioplasty techniques has been reported for treatment of this condition. In this article, we report and discuss a case of BCS by membranous obstruction in the hepatic vein outflow tract, with middle hepatic vein thrombosis, in a 24-year-old patient. The treatment chosen and employed was transjugular balloon angioplasty, which achieved satisfactory results and good clinical evolution.

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.

Caracterización clínica e histopatológica de niños con hepatitis autoinmune en un centro de referencia de Bogotá, Colombia / Clinical and histopathological characterization of children with autoimmune hepatitis at a referral center in Bogotá, Colombia

Resumen La hepatitis autoinmune (HAI) es una enfermedad hepática inflamatoria progresiva poco frecuente en niños y adolescentes, la cual es un reto diagnóstico para clínicos y patólogos. Describimos las características clínicas, bioquímicas e histopatológicas de 21 pacientes pediátricos con HAI diagnosticados en los últimos 14 años. Las biopsias hepáticas se reevaluaron para analizar detalladamente los hallazgos histopatológicos. De los 21 casos evaluados, 12 (57,1%) fueron mujeres, la mediana de edad fue 14 años, y 17 (80,9%) tenían HAI tipo 1. Los signos clínicos más frecuentes fueron ictericia (66,7%) y coluria (44,4%); también hubo evidencia de hipertensión portal con várices esofágicas (47,1%) y esplenomegalia (41,2%). El 11,8% de los pacientes tenía antecedentes de otras enfermedades autoinmunes. El 89,5%, 88,9% y 60,0% de los casos tenía elevación de aminotransferasas, hiperbilirrubinemia y bajos niveles de albúmina sérica, respectivamente. Las biopsias reevaluadas mostraron infiltrado linfoplasmocitario portal (94,4%), hepatitis de interfase (77,8%) y formación de rosetas (50,0%). En el 42,9% de las biopsias se hallaron inclusiones hialinas en las células de Kupffer. Cerca del 33,5% de los casos mostró cirrosis en la biopsia inicial. A pesar del tratamiento inmunosupresor, 4 pacientes requirieron trasplante hepático y 2 están en lista de espera. La HAI en niños puede manifestarse con ictericia y coluria, signos de hipertensión portal, aminotransferasas elevadas, hiperbilirrubinemia y anticuerpos circulantes. Las inclusiones hialinas en las células de Kupffer pueden ser un hallazgo útil en el diagnóstico histopatológico de la HAI en niños.

Abstract Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease. It is uncommon in children and adolescents, and is a diagnostic challenge for clinicians and pathologists. We describe the clinical, biochemical and histopathological characteristics of 21 pediatric patients with AIH diagnosed in the last 14 years. Liver biopsies were reassessed to analyze histopathological findings in detail. Of the 21 cases evaluated, 12 (57.1%) were girls and young women, the median age was 14 years old, and 17 (80.9%) had type 1 AIH. The most frequent clinical signs were jaundice (66.7%), choluria (44.4%), evidence of portal hypertension with esophageal varices (47.1%), and splenomegaly (41.2%). Histories of other autoimmune diseases were found in 11.8% of these patients. Elevated levels of aminotransferases were found in 89.5% of the patients, hyperbilirubinemia was found in 88.9%, and 60.0% of the cases had low levels of serum albumin. Reassessed biopsies showed portal lymphoplasmocytic infiltrate (94.4%), interface hepatitis (77.8%) and rosette formation (50.0%). Hyaline inclusions were found in Kupffer cells in 42.9% of the biopsies. About 33.5% of the cases showed cirrhosis at the initial biopsy. Despite immunosuppressive treatment, four patients required liver transplantation and two are on the waiting list. AIH in children can manifest with jaundice, choluria, signs of portal hypertension, elevated aminotransferases, hyperbilirubinemia and circulating antibodies. Hyaline inclusions in Kupffer cells may be a useful finding in the histopathological diagnosis of AIH in children.

Refractory ascites and graft dysfunction in early renal transplantation / Ascite refratária e disfunção do enxerto no pós-transplante renal precoce

Abstract The occurrence of ascites after Renal Transplant (RT) is infrequent, and may be a consequence of surgical or medical complications. Case report: 61 year-old, male, history of arterial hypertension, tongue carcinoma and alcoholic habits 12-20g/day. He had chronic kidney disease secondary to autosomal dominant polycystic kidney disease, without hepatic polycystic disease. He underwent cadaver donor RT in September 2017. He had delayed graft function by surgically corrected renal artery stenosis. He was admitted in January 2018 for ascites de novo, with no response to diuretics. HE had visible abdominal collateral circulation. Graft dysfunction, adequate tacrolinemia, Innocent urinary sediment, mild anemia, without thrombocytopenia. Serum albumin 4.0g / dL. Normal hepatic biochemistry. Peritoneal fluid with transudate characteristics and serum albumin gradient > 1.1. Ultrasound showed hepatomegaly, permeable vascular axes, without splenomegaly. Mycophenolate mofetil was suspended, with reduced remaining immunosuppression. He maintained refractory ascites: excluded infectious, metabolic, autoimmune and neoplastic etiologies. No nephrotic proteinuria and no heart failure. MRI: micronodules compatible with bile cysts. Upper Digestive Tract Endoscopy did not show gastroesophageal varicose veins. Normal abdominal lymphoscintigraphy. He underwent exploratory laparoscopy with liver biopsy: incomplete septal cirrhosis of probable vascular etiology some dilated bile ducts. He maintained progressive RT dysfunction and restarted hemodialysis. The proposed direct measurement of portal pressure was delayed by ascites resolution. There was further recovery of the graft function. Discussion: Incomplete septal cirrhosis is an uncommon cause of non-cirrhotic portal hypertension. Its definition is not well known, morphological and pathophysiological. We have not found published cases of post-RT ascites secondary to this pathology, described as possibly associated with drugs, immune alterations, infections, hypercoagulability and genetic predisposition.

Resumo A ocorrência de ascite no pós-Transplante Renal (TR) é infrequente, podendo ser consequência de complicações cirúrgicas ou médicas. Caso clínico: 61 anos, masculino, antecedentes de hipertensão arterial, carcinoma da língua e hábitos alcoólicos 12-20g/dia. Doença renal crônica secundária à doença renal poliquística autossômica dominante, sem poliquistose hepática. Submetido a TR de doador cadáver em setembro de 2017. Atraso na função de enxerto por estenose da artéria renal, corrigida cirurgicamente. Internado em janeiro de 2018 por ascite de novo, sem resposta a diuréticos. Circulação colateral abdominal visível. Disfunção do enxerto, tacrolinemia adequada. Sedimento urinário inocente. Anemia ligeira, sem trombocitopenia. Albumina sérica 4,0g/dL. Bioquímica hepática normal. Líquido peritoneal com características de transudado e gradiente sero-ascítico de albumina > 1,1. Ecografia com hepatomegalia, eixos vasculares permeáveis, sem esplenomegalia. Suspendeu micofenolato mofetil, reduziu restante imunossupressão. Manteve ascite refratária: excluídas etiologias infecciosas, metabólicas, autoimunes e neoplásicas. Sem proteinúria nefrótica e sem insuficiência cardíaca. RM: micronódulos compatíveis com quistos biliares. EDA sem varizes gastroesofágicas. Linfocintigrafia abdominal normal. Submetido a laparoscopia exploradora com biópsia hepática: cirrose septal incompleta de provável etiologia vascular, alguns ductos biliares dilatados. Manteve disfunção progressiva do TR, reiniciou hemodiálise. Proposta medição direta da pressão portal, protelada por resolução da ascite. Recuperação posterior da função de enxerto. Discussão: A cirrose septal incompleta é uma causa incomum de hipertensão portal não cirrótica. A sua definição é morfológica e a fisiopatologia, pouco conhecida. Não encontramos publicados casos de ascite pós-TR secundária a esta patologia, descrita como possivelmente associada a fármacos, alterações imunitárias, infecções, hipercoagulabilidade e predisposição genética.

Observational Cohort Study of Hepatic Encephalopathy After Transjugular Intrahepatic Portosystemic Shunt (TIPS)

Abstract: Introduction and aim. Hepatic encephalopathy (HE) is a common complication of transjugular intrahepatic portosystemic shunting (TIPS). It is associated with a reduced quality of life and poor prognosis. The aim of this study was to compare two groups of patients who did and did not develop overt HE after TIPS. We looked for differences between these groups before TIPS. Material and methods. A study of 895 patients was conducted based on a retrospective analysis of clinical data. Data was analyzed using Fisher’s exact test, χ2, Mann Whitney test, unpaired t-test and logistic regression. After the initial analyses, we have looked at a regression models for the factors associated with development of HE after TIPS. Results. 257 (37.9%) patients developed HE after TIPS. Patients’ age, pre-TIPS portal venous pressure, serum creatinine, aspartate transaminase, albumin, presence of diabetes mellitus and etiology of portal hypertension were statistically significantly associated with the occurrence of HE after TIPS (p < 0.01). However, only the age, pre-TIPS portal venous pressure, serum creatinine, presence of diabetes mellitus and etiology of portal hypertension contributed to the regression model. Patients age, serum creatinine, presence of diabetes mellitus and portal vein pressure formed the model describing development of HE after TIPS for a subgroup of patients with refractory ascites. Conclusion. We have identified, using a substantial sample, several factors associated with the development of HE after TIPS. This could be helpful in further research.

Bariatric surgery in individuals with liver cirrhosis: A narrative review / Cirurgia bariátrica em indivíduos com cirrose hepática: uma revisão narrativa

Summary Introduction: Bariatric surgery has become the gold standard treatment for morbid obesity, but there is no consensus regarding its safety and efficacy among individuals with chronic liver diseases. Objective: To critically evaluate the existing evidence on literature about bariatric surgery in individuals with liver cirrhosis. Method: Narrative review performed by means of an online search in the MEDLINE and LILACS databases. Results: Bariatric surgery is safe and effective in individuals with chronic liver disease without clinical decompensation or significant portal hypertension. Individuals with severe liver function impairment present significantly higher surgical morbidity and mortality. Among candidates to liver transplantation, surgery may be performed before, after and even during transplantation, and there is a predominant trend to perform it after. Vertical sleeve gastrectomy seems to be the most adequate technique in this group of subjects. Conclusion: Bariatric surgery is safe and effective in individuals with compensated cirrhosis without significant portal hypertension, but presents higher morbidity. Among candidates to liver transplantation and/or individuals with severe portal hypertension, morbidity and mortality are significantly higher.

Resumo Introdução: A cirurgia bariátrica tornou-se nos últimos anos o tratamento padrão-ouro para a obesidade mórbida; porém, entre obesos portadores de hepatopatia crônica, não existe consenso a respeito de sua segurança e efetividade. Objetivo: Análise crítica da literatura existente sobre a realização de cirurgia bariátrica em portadores de cirrose hepática. Método: Revisão narrativa por meio de pesquisa online nas bases de dados Medline e Lilacs. Resultados: As cirurgias bariátricas em indivíduos cirróticos sem descompensação clínica levam a resultados satisfatórios. Já indivíduos com hepatopatia grave apresentam morbidade perioperatória e mortalidade significativamente maiores do que as observadas na população obesa sem hepatopatia. Em candidatos a transplante hepático, a cirurgia pode ser realizada antes, durante ou após o transplante, havendo uma tendência predominante à realização após o transplante. A gastrectomia vertical parece ser a técnica mais adequada nesse grupo de pacientes. Conclusão: A cirurgia bariátrica é segura e efetiva em portadores de cirrose hepática compensada e sem hipertensão portal; porém, apresenta maior morbidade. Em candidatos a transplante e/ou indivíduos com hipertensão portal significativa, a morbimortalidade é significativamente maior.

Características clínicas y laboratoriales de pacientes hospitalizados con hipertensión portal y complicaciones vasculares pulmonares en el Instituto Nacional de Salud del Niño / Clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño

El síndrome hepatopulmonar (SHP) y la hipertensión portopulmonar (HTPP) son distintas complicaciones vasculares pulmonares de la hipertensión portal (HTP) y se asocian con una mayor morbilidad y mortalidad. Objetivos: Describir las Características Clínicas y Laboratoriales de los pacientes con HTP y complicaciones vasculares pulmonares hospitalizados en el Instituto Nacional de Salud del Niño. Materiales y métodos: se incluyeron los pacientes con HTP hospitalizados desde enero del 2012 hasta junio del 2013 y que durante su evolución cursaron con SHP o HTPP. Para el análisis se les dividió en un primer grupo de pacientes con cirrosis hepática y un segundo grupo con obstrucción extra hepática de vena porta. Resultados: De 22 pacientes con HTP el 45,5% fueron varones y el rango de edad fue entre 1 mes y 17 años. La etiología en el grupo de cirrosis (n=14) fue: hepatitis autoinmune (35,7%), cirrosis criptogénica (35,7%), error innato del metabolismo (14,3%), hepatitis viral crónica por virus C (7,15%) y atresia de vías biliares extra hepática (7,15%). Las complicaciones vasculares pulmonares, se presentaron más frecuentemente en los pacientes con cirrosis hepática (1 caso de síndrome hepatopulmonar y un caso de hipertensión portopulmonar). En ellos se encontró más frecuentemente disnea, astenia, edema, desnutrición, ascitis, hiperesplenismo y hemorragia digestiva por várices esofágicas, además de valores elevados de ALT, fosfatasa alcalina y menores niveles de albúmina sérica. Conclusiones: En niños con HTP, las complicaciones vasculares pulmonares son muy infrecuentes. En la evaluación de estos pacientes debería incluirse la oximetría de pulso para detectar hipoxemia y posteriormente, de ser necesario una ecocardiografía Doppler y de contraste. Ante el hallazgo de hipertensión sistólica pulmonar es necesario realizar un cateterismo cardiaco derecho.

The hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHN) are distinct pulmonary vascular complications of portal hypertension (PHT) and are associated with increased morbidity and mortality. Objectives: To describe the clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño. Materials and methods: We included patients with HTP hospitalized from January 2012 to June 2013 and that during its evolution progressed with SHP or HTPP. For analysis, they were divided into a first group of patients with liver cirrhosis and a second group with extrahepatic portal vein obstruction. Results: Of 22 patients with HPT 45.5% were male and the age range was between 1 month and 17 years. The etiology in the group of cirrhosis (n=14) was: autoimmune hepatitis (35.7%), cryptogenic cirrhosis (35.7%), inborn error of metabolism (14.3%), chronic viral hepatitis C (7.15%) virus and atresia extra-hepatic bile ducts (7.15%). Pulmonary vascular complications more frequently occurred in patients with liver cirrhosis (1 case of HPS and a case of PPHTN). They most often dyspnea, asthenia, edema, malnutrition, ascites, hypersplenism and gastrointestinal bleeding from esophageal varices was found. Also, they had elevated ALT values, alkaline phosphatase and serum albumin values decreased. Conclusions: In children with pulmonary hypertension, pulmonary vascular complications are rare. In the evaluation of these patients pulse oximetry should be included to detect hypoxemia and subsequently a Doppler echocardiography and contrast echocardiography necessary. Dueto the finding of systolic pulmonary hypertension it is necessary to perform right heart catheterization.

Caput Medusae due to portal hypertension in schistosomiasis mansoni / Cabeza de Medusa debida a la hipertensión portal en esquistosomiasis mansoni

A 62-year-old Brazilian man who lived in endemic areas of tropical diseases had an episode of hematemesis associated with portal hypertension. He used to swim in natural ponds during childhood and developed the hepatosplenic form of schistossomiasis with moderate ascites, in addition to the characteristic features of abdominal Caput Medusae. The aim of the report is highlight the role of chronic liver disease and schistossomiasis

Un hombre natural de Brasil de 62 años de edad que vivía en zonas endémicas de enfermedades tropicales presentó un episodio de hematemesis asociada con hipertensión portal. Frecuentemente se bañaba en los estanques naturales durante la infancia y desarrolló la forma hepatosplénica de la esquistosomiasis con ascitis moderada, además de los rasgos abdominales característicos de la Cabeza de Medusa. El objetivo del informe es poner de relieve el papel de la enfermedad hepática crónica y de la esquistosomiasis

Persistencia y repermeabilización del conducto de Cuvier izquierdo / Persistence and restoration of the patency of the left duct of Cuvier

La hepatopatía crónica favorece la redistribución del flujo hacia la circulación sistémica. Si bien es poco frecuente, la repermeabilización de los conductos venosos embrionarios causa el aumento de la presión en la circulación portal. Nuestro objetivo es dar a conocer la persistencia y permeabilidad del conducto venoso de Cuvier izquierdo y su presentación en imágenes

The increased pressure in the portal circulation due to a chronic liver disease favours the redistribution of the flow to the systemic circulation. Although rare, reperfusion of embryonic venous channels may be a possibility. The aim of this article is to determine the persistence and the patency of the ductus venosus, called the left duct of Cuvier, and its presentation in images

Hemorragia digestiva alta varicosa em hospital de emergência e, Recife - PE / Varicel upper gastrointestinal bleeding in emergency hospital in Recife - PE

A hemorragia digestiva alta varicosa (HDAV) é a mais grave complicação da hipertensão portal, com alta taxa de ressangramento e mortalidade. Em Pernambuco, devido à endemicidade da esquistossomose mansônica, há uma elevada frequência deste tipo de sangramento. Objetivos: avaliar a doença hepática de base de pacientes portadores de varizes esofagogástricas (VVEEGG) com HDA, além do seguimento quanto à orientação após alta hospitalar da emergência, taxa de ressangramento e óbito em hospital de emergência. Pacientes e métodos: trata-se de um estudo descritivo, tipo série de casos, em pacientes com VVEEGG atendidos no setor de endoscopia digestiva do Hospital da Restauração em Recife, referência no estado em hemorragia digestiva, de outubro de 2008 a outubro de 2009. Foram coletados dados sobre antecedentes pessoais, aspectos demográficos e clínicos e realizado exame endoscópico para avaliação do sítio de sangramento e propedêutica para controle da hemorragia se necessário. Resultados: foram analisados 178 pacientes portadores de VVEEGG com HDA. A média de idade foi 53,9 anos, sendo 115 (64,6%) do sexo masculino. A faixa etária mais frequente foi de 50 a 59 anos (31,5%) e 67 (37,7%) eram naturais de Zona da Mata. Ao exame físico, 58 (32,6%) pacientes apresentavam ascite. Foi evidenciado que 177 pacientes tinham varizes esofágicas (VVEE) e 78 tinham varizes gástricas. Quanto aos exames solicitados para caracterizar a doença de base, evidenciou-se um valor médio de albumina de 2,8 g/dl, de bilirrubina total 2,2 mg/dl e de INR 1,4. A doença hepática crônica (DHC) foi definida pelo ultrassom (USG) em cirrose (38), esquistossomose (60) e doença mista (22). Após a alta hospitalar, 45/174 pacientes (25,9%) ficaram em uso de propranolol e 57/174 (32,8%) conseguiram acompanhamento em ambulatório especializado. Após 3 meses do episódio de HDA, foi constatado relato de ressangramento em 92/161 (57,1%) pacientes e óbito em 44/161 (27,3%). Houve relação de ressangramento com óbito (p<0,001), com presença de manchas vermelhas nas VVEE (p<0,001), diagnóstico de cirrose hepática e DHC mista pelo USG (p=0,023), presença de ascite (p=0,004) e a não utilização de propranolol (p=0,002). Observou-se a associação de óbito e faixa etária de 50 a 59 anos (p=0,016) e com aspecto hepático pelo USG (p=0,001), assim como da presença de fibrose periportal no USG com a naturalidade (p=0,031). Conclusão: neste estudo, observou-se predomínio de pacientes do sexo masculino e naturais da Zona da Mata. A maioria dos pacientes apresentava fibrose periportal ao USG, mostrando a endemicidade da esquistossomose no estado e houve uma elevada frequência de ressangramento e óbito.

Background: variceal upper gastrointestinal bleeding (VUGB) is the most serious complication of portal hypertension, with high rebleeding and mortality rates. In Pernambuco, where schistosomiasis mansoni is endemic, this type of bleeding occurs frequently. Aim: to evaluate the etiology of esophageal variceal bleeding, as well as the rebleeding and death rates. The study also followed up the patients after hospital discharge, rebleeding and death rates of the patients admitted in an Emergency Hospital. Patients and methods: this is a descriptive study about patients with gastric-esophageal varices and UGB assisted at the digestive endoscopy unit of Restauração hospital in Recife from October 2008 to October 2009. An application form was filled out with demographic data, personal history, clinical aspects and an endoscopy was performed to identify the area of the bleeding, as well as a propaedeutical endoscopy to control the bleeding, when necessary. Results: 178 patients with gastric esophageal varices and UGB were observed. The specific-age rate was 53.9 years old, with 115 (64.6%) males. The most frequent age group was from 50 to 59 years old (31.5%) and 67 (37.7%) were born from the Zona da Mata region. Physical examination revealed ascites in 58 (32.6%) patients. It was seen that 177 patients had esophageal and 78 gastric varices. The blood tests done after the endoscopy to identify the underlying disease showed approximately 2,8g/dl of albumin, total bilirrubin of 2.2mg/dl and INR 1.4. Hepatic chronic disease (HCD) was defined with an ultrasound in cirrhosis (38), schistosomiasis (60) and mixed disease (22). After hospital discharge, 45/174 patients (25.9%) were prescribed propranolol and 57/174 (32.8%) were granted ambulatory care. Three months after the UGB episode, 92/161 (57.1%) patients had rebleeding and 44/161 (27.3%) died. There was a connection between the rebleeding and the deaths (p<0.001), there was presence of red spots on the esophageal varices (p<0.001), diagnose of cirrhosis and mixed hepatic chronic disease by ultrasound (p=0.023), ascitis (p=0.004) and the absence of propranolol use (p=0.002). There was also a relation between death and age 50 to 59 years (p=0.016) and the hepatic aspect detected by ultrasound (p=0.001), as well the presence of periportal fibrosis in the USG with the birthplace (p=0.031). Conclusion: in this study, it was possible to observe that there were predominance of male and patients from the Zona da Mata region. Most of the patients presented periportal fibrosis in the ultrasound, reflecting the high endemicity of schistosomiasis in the state and elevated rebleeding and death rates were observed.

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.

Síndrome de Cruveilhier-Baumgarten: aspectos ultrassonográficos / Cruveilhier-Baugarten syndrome: sonographic aspects

Objetivo: este trabalho analisa as alterações ultrassonográficas que caracterizam a síndrome de Cruveilhier-Baumgarten (SCB), com suas alterações clínicas. A SCB caracteriza-se pela veia paraumbilical proeminente, com dilatação das veias superficiais da parede abdominal de aspecto radiado provenientes do umbigo dos pacientes com hepatopatia crônica e hipertensão portal. Casuística e métodos: em avaliação ultrassonográfica de 60 pacientes com diagnóstico de hepatopatia crônica com hipertensão portal foram encontrados quatro casos com a SCB em que foi realizado o estudo por meio do doppler colorido. Resultados: todos os pacientes avaliados eram do gênero masculino, estavam na terceira década de vida, tinham história de esplenomegalia, varizes na parede abdominal e eram portadores de esquistossomose e aspecto histopatológico de cirrose micronodular. Foram analisados também dois pacientes com varizes do esôfago sem clinica de hematêmese. Ultrassonograficamente, todos os pacientes tinham sinais de hepatopatia crônica com esplenomegalia, com fluxo hepatopetal em todos os segmentos da veia porta e fluxo hepatofugal nas veias que deixam o fígado (veia paraumbilical no ligamento falciforme e nas veias superficiais da parede abdominal anterior). Conclusão: a SCB caracteriza-se pela associação de sinais de hepatopatia crônica com esplenomegalia, veias paraumbilicais com aumento de calibre (hipertensão porta) e com fluxo para as veias superficiais da parede abdominal, que se acham dilatadas na região umbilical e com sopro à ausculta pelo ultrassom e ao exame físico.

Objective: The purpose of this paper is to analyze the sonographic changes that characterize the Cruveilhier-Baumgarten syndrome and its clinical charactheristics. Cruveilhier- Baumgartem syndrome is characterized by a prominent paraumbilical vein, with dilatated superficial veins of the abdominal wall radiating from the umbilicus of patients with chronic liver disease and portal hypertension. Patients and methods: In a sonographic evaluation of 60 patients diagnosed with chronic liver disease with portal hypertension we found 4 cases of Cruveilhier-Baumgarten syndrome, which we studied with color doppler sonography.Results: All patients were male, in their third decade of life, with a clinical history of splenomegaly and varicose abdominal wall veins. Patients had schistosomiasis and micronodular cirrhosis on hystopathological examination. We also evaluated two patients with esophageal varices without hemathemesis. All patients had ultrasound signs of splenomegaly and chronic liver disease, with hepatopetal flow in all segments of the portal vein and hepatofugal flow in the efferent veins of the liver (paraumbilical vein in the falciform ligament and the anterior abdominal wall surface veins). Conclusion: CBS is charactherized by the associationof signs of chronic liver damage with splenomegaly, distention of paraumbilical veins (portal hypertension) and blood flow towards the superficial abdominal wall veins, which are dilated in the umbilical region, associated with an audible bruit in both clinical and sonographic examination.

Preditores não invasivos de varizes esofágicas em crianças e adolescentes com hepatopatia crônica ou obstrução extra-hepática da veia porta / Non-invasive predictors of esophageous varices in children and adolescents with chronic liver disease or extrahepatic portal venous obstruction

OBJETIVO: Identificar preditores não invasivos de varizes esofágicas em crianças e adolescentes com hepatopatia crônica ou obstrução extra-hepática da veia porta. PACIENTES E MÉTODOS: Estudo transversal que incluiu 53 crianças e adolescentes com hepatopatia crônica ou obstrução extra-hepática da veia porta, sem antecedente de hemorragia digestiva ou tratamento de varizes esofágicas, com até 20 anos de idade. Dois grupos foram formados: grupo I (35 pacientes com hepatopatia crônica) e grupo II (18 com obstrução extra-hepática da veia porta). Foram realizados hemograma, razão normalizada internacional, albumina, bilirrubina total, ultrassonografia de abdome e endoscopia digestiva alta. O índice esplênico foi determinado dividindo a dimensão esplênica pelo valor do limite superior da normalidade. As variáveis foram comparadas quanto à presença ou não de varizes esofágicas através de análise univariada (testes qui-quadrado, exato de Fischer e de Wilcoxon) e multivariada (regressão logística). A acurácia foi determinada a partir da área sob a curva ROC. RESULTADOS: As varizes esofágicas foram observadas em 48,5% dos pacientes do grupo I e em 83,3% do grupo II. Plaquetopenia (p = 0,0015), esplenomegalia (p = 0,0003) e a razão plaquetas/índice esplênico (p = 0,0007) se mostraram indicadores preditivos de varizes esofágicas entre os pacientes do grupo I. Após análise multivariada, a plaquetopenia (odds ratio = 21,7) se manteve como um indicador independente da presença de varizes esofágicas entre os pacientes com hepatopatia crônica. CONCLUSÃO: O número de plaquetas, o índice esplênico e a razão plaquetas/índice esplênico se mostraram preditivos de varizes esofágicas em crianças e adolescentes com hepatopatia crônica. Não foram encontrados preditores de varizes esofágicas entre os pacientes com obstrução extra-hepática da veia porta.

OBJECTIVE: To identify non-invasive predictors of esophageal varices in children and adolescents with chronic liver disease or extrahepatic portal venous obstruction (EHPVO). METHODS: 53 patients younger than 20 years with chronic liver disease or EHPVO and no history of bleeding or prophylactic treatment of esophageal varices (EV) were assessed. They were divided into 2 groups: group I (35 with chronic liver disease) and group II (18 with EHPVO). Their blood count, international normalized ratio (INR), albumin, bilirubin, abdominal ultrasonography and upper endoscopy results were taken. A splenic index was determined by dividing the patients' spleen dimension by its uppermost limit according to their age. The variables were compared to EV presence or not. Univariate (chi-square test, Fischer's exact test and Wilcoxon exact test) and multivariate (logistic regression) analyses were performed. A receiver operating characteristic (ROC) curve was constructed and the area under the ROC curve was calculated. RESULTS: EV were observed in 48.5% of group I patients and in 83.3% of group II patients. Low platelet count (p = 0.0015), splenomegaly (p = 0.0003) and splenic index (p = 0.0007) were statistically significant predictors of EV among group I patients. The multivariate analysis showed low platelet count (odds = 21.7) as an independent predictor of EV in patients with chronic liver disease. CONCLUSION: Platelet count, splenic index and platelet-splenic index ratio were predictors of EV in children and adolescents with chronic liver disease. There were no EV predictors among group II patients.

Fístula arterio-venosa intrahepática como causa de hipertensión portal no cirrótica, en paciente portador de hepatitis autoinmune: diagnóstico y tratamiento: caso clínico / Autoimmune hepatitis patient carrier with non cirrhotic portal hypertension caused by an intrahepatic portal arteriovenous fistula: diagnosis and treatment: clinical case

Portal hypertension (PH) is defined as pathological increase of hydrostatic pressure in the portal venous system, usually related to liver cirrhosis. Among the uncommon causes of PH is the arteriovenous intra or extrahepatic fistula (AVF) of traumatic, iatrogenic or congenital origin. Clinical history and ultrasound findings of AVF are very important for the diagnosis. From a therapeutic point of view, there are three alternatives: clinical/imaging follow-up, surgical repair and transcutaneous catheter embolization. A report of a clinical case and a review of the literature are presented. Patient with portal hypertension as a result of intra hepatic AVF, successfully treated by transcutaneous catheter embolization. A 54 year-old female patient, and cholecystectomized and with history of breast cancer, presented altered liver function tests several months after gallbladder surgery. Once biliary disease was ruled out, liver biopsy was performed, which was compatible with autoimmune hepatitis. During follow-up, intrahepatic AVF was observed by means of ultrasound. Underlying disease was successfully managed with Prednisone and Azathioprine. Nine years later, she experienced an episode of confusion and disorientation compatible with hepatic encephalopathy (HE) and esophageal varices diagnosed by upper endoscopy. Laboratory tests and imaging did not show progression baseline liver disease. Angiographic procedures confirmed an intra hepatic AVF and selective embolization was carried out. There was clinical remission of HE and esophageal varices. We concluded, that transcutaneous catheter embolization is a valid alternative for the treatment of intra hepatic AVF, which accounted for the successful result for this particular patient.

La hipertensión portal (HP) se define como el aumento patológico de la presión hidrostática en el sistema venoso portal, habitualmente relacionada con cirrosis hepática. Entre las causas infrecuentes de HP está la fístula arterio-venosa (FAV) intra o extrahepática de origen traumáticas, iatrogénicas o congénitas entre otras. En el diagnóstico son importantes los antecedentes clínicos y hallazgos ecográficos que demuestran FAV. Desde el punto de vista terapéutico, existen tres alternativas: seguimiento clínico y de imágenes, reparación quirúrgica y embolización con catéter transcutáneo. Se presenta caso clínico de paciente con (HP) a consecuencia de una FAV intrahepática, tratada satisfactoriamente mediante embolización con catéter transcutáneo y revisión de la literatura pertinente. Se trata de una paciente de 54 años con antecedentes de cáncer mamario y colecistectomía en quien se constatan alteraciones de pruebas hepáticas varios meses con posterioridad a cirugía vesicular. Una vez descartada patología biliar, se realizó biopsia hepática la que fue compatible con hepatitis autoinmune. Durante el seguimiento se pesquisó FAV intrahepática como hallazgo ecográfi co. Su enfermedad de base se trató satisfactoriamente con Prednisona y Azatioprina. Nueve años más tarde, consulta por episodio de confusión y desorientación compatible con encefalopatía hepática (EH) y presencia de várices esofágicas a la endoscopia. Tanto el laboratorio como imágenes no mostraron progresión de enfermedad hepática de base. Es sometida a procedimiento angiográfico, que confirmó FAV intrahepática, procediendo a embolización selectiva. Hubo remisión del cuadro clínico de EH y regresión de las várices esofágicas. Se concluye que la embolización con catéter transcutáneo, es una alternativa válida en el tratamiento de FAV intrahepáticas, terapia que constituyó la solución definitiva del cuadro clínico reportado.

Trombose de veia porta em crianças e adolescentes: revisão de literatura / Portal vein thrombosis in children and adolescents: literature review

Trombose de veia porta (TVPO) refere-se à obstrução total ou parcial do fluxo sanguíneo nessa localização, secundária à formação de trombos, Essa entidade se mostra importante na faixa etária pediátrica, por ser causa importante de hipertensão porta, com elevadas taxas de morbidade devido à sua principal complicação - a hemorragia digestiva alta (HDA). Aproximadamente 79% das crianças com diagnóstico de trombo- se de veia porta apresentarão ao menos um episódio de HDA. A etiologia da trombose é variada e, na maioria dos casos, há associação de fatores locais e sistêmicos, sendo o cateterismo umbilical o principal fator etiológico encontrado em crianças. A importância dos distúrbios de coagulação entre esses fatores está se tornando mais acentuada à medida que novos estudos e métodos diagnósticos são disponibilizados. O tratamento varia de acordo com a forma de apresentação - aguda ou crônica - e com a existência ou não de complicações associadas à TVPO, No quadro agudo a anticoagulação está indicada na maioria dos casos, enquanto no quadro crônico sua indicação acontece geralmente quando há trombofilias associadas, Na evolução crônica, o tratamento se direciona às complicações decorrentes da hipertensão porta secundária à TVPO.

Portal Vein Thrombosis (PVT) refers to a total or partial obstruction of the blood flow in this location, secondary to a thrombus tormation.': 2 PVT is important in the pediatric age group because it is one of lhe most frequent causes of portal hypertension, with high morbidity rates due to its main complicalion - the upper gastrointestinal bleeding. Approximately 79% of lhe children diagnosed with PVT will show at least one episode of upper gastrointestinal bleeding during their lives. The etiology of portal vein lhrombosis is varied and, in most cases, there is an associalion of local and systemic tactors, and lhe umbilical calheterization is lhe main etiological tactor found in children. The importance of coagulation disorders among these factors is becoming greater as new studies and diagnostic methods are available. Treatment varies according to the form of presentation - acute or chronic - and the presence or absence of complications associated with PVT. In acute thrombosis, anticoagulation is indicated in most cases, while in the chronic condition is usually indicated when the patient has thrombophilia.In chronic disease, treatment is directed at complications of portal hypertension secondary to PVT.

Profilaxis primaria de várices esofágicas en pediatría / Primary prophylactic treatment of esophageal varices in pediatrics

Children with chronic liver disease or portal vein thrombosis frequently develop portal hypertension. The main complication of the portal hypertension are the development of esophageal varices who are at risk of variceal bleeding, which is associated with significant morbidity and mortality. Guidelines for adults with portal hypertension recommend performing esophagogastroduodenoscopy to identify those with varices that may benefit from prophylactic therapy, however there are no consensus in the pediatric age. This revision includes the incidence, diagnostic and the evidence for primary prophylactic treatment of the esophageal varices in children.

Pacientes pediátricos con daño hepático crónico o con trombosis de la vena porta frecuentemente desarrollan hipertensión portal. La principal complicación de la hipertensión portal es el desarrollo de várices esofágicas que conlleva riesgo de hemorragia variceal, la cual se asocia a significativa morbimortalidad. Las guías clínicas para adultos recomiendan realizar, en pacientes cirróticos, una endoscopia como screening para el diagnóstico de várices esofágicas y su tratamiento profiláctico, sin embargo, en pacientes pediátricos no existe consenso. En esta revisión se actualiza la incidencia, diagnóstico y evidencia del tratamiento profiláctico primario de várices esofágicas en pediatría.