Perforación espontánea de la vía biliar en un neonato: un hallazgo inesperado: caso clínico / Spontaneous perforation of the bile duct in a neonate: an unexpected finding: case report

La perforación espontánea de la vía biliar extrahepática es rara en recién nacidos. Es una causa quirúrgica de ictericia en este período y la presentación aguda es inusual. OBJETIVO: presentar un caso de perforación espontanea de la vía biliar en un recién nacido por sus graves complicaciones si no se realiza un diagnóstico temprano y oportuno. CASO CLÍNICO: Recién nacido de término de 10 días de vida que consultó por cuadro de rechazo alimentario, fiebre y distensión abdominal de 2 días de evolución, sin ictericia, acolia, ni coluria. En los exámenes de laboratorio se encontró leucopenia, trombocitosis y elevación de la proteína C reactiva, con función hepática normal. La radiografía de abdomen demostró neumoperitoneo, sospechándose enterocolitis necrosante, se realizó laparotomía, encontrándose perforación espontánea de la vía biliar extrahepática y peritonitis biliar. La colangiografía intraoperatoria demostró la vía biliar proximal rescatable y conducto cístico dilatado. Se realizó hepático-yeyunostomía con Y de Roux transmesocolónica y colecistectomía. En el estudio postoperatorio se encontró trombosis portal, por lo que recibió tratamiento anticoagulante. En el control a los ocho meses de edad, la paciente tenía buena tolerancia oral y adecuado incremento ponderal. CONCLUSIONES: La perforación biliar es una entidad rara y más en el período neonatal, condición que la vuelve un reto diagnóstico y terapéutico. El pronóstico dependerá de la intervención temprana y los hallazgos intraoperatorios.

Spontaneous extrahepatic bile duct perforation is rare in newborns. It is a surgical cause of jaundice in this period and the acute presentation is unusual. OBJECTIVE: To report a case of spontaneous bile duct perforation in a newborn due to its serious complications if an early and timely diagnosis is not performed. CLINICAL CASE: A 10-day-old newborn who developed food rejection, fever and abdominal distension without jaundice, acolia, or coluria two days prior of admission. The laboratory tests showed leukopenia, thrombocytosis, increased C-reactive protein, and normal hepatic function. The abdominal x-ray showed pneumoperitoneum, and the diagnosis of necrotizing enterocolitis was made. Laparotomy was performed; extrahepatic bile duct perforation and biliary peritonitis were noted. Intraoperative cholangiography demonstrated rescatable proximal bile duct and dilated cystic duct. Hepatic-jejunostomy was performed with Roux-en-Y and cholecystectomy. In the postoperative study portal thrombosis was found, so he received anticoagulant treatment. At 8 months of age, the patient had enteral feeding tolerance and adequate weight gain. CONCLUSIONS: Biliary perforation is a rare entity and more in the neonatal period, a condition that makes it a diagnostic and therapeutic challenge. The prognosis will depend on early intervention and intraoperative findings.

Novedades en enfermedades colestásicas / Latest advances in cholestatic diseases

Cholestatic liver diseases arise from impaired hepatobiliary production and excretion of bile. They have some common clinical manifestations and pathogenic features, but at the same time differences that require a special approach. Intrahepatic cholestasis of pregnancy and drug induced liver cholestasis are the most common reversible intrahepatic cholestasis. Primary biliary cirrhosis and primary sclerosing cholangitis are chronic cholestatic diseases. This review will be focused on these two types, in particular their clinical and therapeutic management and complications. While in recent years there has not been much change in the basic clinical approach of these diseases, every day we receive more information from both the basic and clinical science studies, which has enabled to develop new therapeutic lines and reject others that have not confirmed effectiveness.

Las enfermedades colestásicas se caracterizan por la disminución de la formación o excreción del flujo de bilis. Ellas tienen ciertas manifestaciones clínicas y mecanismos patogénicos comunes, pero a su vez diferencias que requieren un enfrentamiento no siempre similar. La colestasia intrahepática del embarazo(CIE) y la colestasia secundaria a fármacos son las más frecuentes dentro de las colestasias intrahepáticas reversibles. La cirrosis biliar primaria (CBP) y la colangitis esclerosante primaria (CEP), son las enfermedades colestásicas crónicas en que centramos esta revisión, en particular en los aspectos clínicos y de manejo terapéutico tanto de éstas, como de sus complicaciones. Si bien en los últimos años no ha habido un cambio significativo en el manejo fundamental de estas enfermedades, cada vez tenemos más información tanto en el área de ciencias básicas como en aspectos clínicos, lo que ha permitido ir desarrollando nuevas líneas terapéuticas y descartando otras que no han confirmado efectividad.

A Case of Hepatic Peribiliary Cysts in a Patient with Alcoholic Liver Cirrhosis / 대한소화기학회지

Hepatic peribiliary cysts (HPCs) are characterized by cystic dilatations of the peribiliary glands located throughout the branches of the biliary systems. Specifically, they are mainly located along the hepatic hilum and major portal tracts. The natural history and prognosis of HPCs are uncertain. In fact, almost all HPCs have been discovered incidentally during radiological examination or autopsy, and they are considered to be clinically harmless. Recently, several cases of HPCs associated with obstructive jaundice or liver failure were reported in patients with pre-existing liver disease in several studies. However, until now there have been no reports of such a case in Korea. Herein, we report a case of HPCs that show a disease course with a poor prognosis. These HPCs developed in a 47-year-old man with progressive alcoholic liver cirrhosis.

Portal biliopathy.

A young male with portal venous thrombosis presented with obstructive jaundice, due to common bile duct stricture secondary to portal biliopathy.

Hepatic hydatid cysts with biliary, and peritoneal rupture and transdiaphragmatic migration.

Hydatid disease is a parasitic infection that most commonly involves the liver. Imaging plays a vital role in the diagnosis of this disease. Rupture of the cyst can give rise to a wide spectrum of complications. We describe a case of hepatic hydatid cyst with rupture into the biliary tree, right pleural cavity and dissemination into the peritoneal cavity, with associated splenic hydatid cysts. MRI may be a useful non-invasive diagnostic tool in such disseminated cases to define the complete extent of the disease.

Obstructive Jaundice due to Biliary Cast Syndrome Followed by Orthotopic Liver Transplantation / 대한소화기학회지

Biliary complication occurs in 6-34% of all liver transplant patients. Although bile leaks and strictures are relatively common, other biliary complications such as T-tube leak, choledocholithiasis, and biliary cast syndrome can also be observed. The biliary cast syndrome describes the presence of casts causing obstruction with its resultant sequelae of biliary infection, hepatocyte damage secondary to bile stasis and ductal damage, all contributing to cholangiopathy. Because the exact timing of cast formation after orthotopic liver transplantation is not consistent, it is difficult to define the true incidence of biliary cast syndrome without long-term follow-up data. Proposed etiological mechanisms include acute cellular rejection, prolongation of cold ischemic time, infection, biliary drainage tubes, and biliary obstruction. The diagnosis of biliary cast syndrome is usually confirmed by endoscopic retrograde cholangiopancreatography. There have been few published articles about biliary casts in Korea. Herein, we report a case of biliary cast syndrome followed by orthotopic liver transplantation.

Spontaneous Resolution of Vanishing Bile Duct Syndrome in Hodgkin's Lymphoma / 대한간학회지

Cholestasis in a patient with Hodgkin's disease is uncommon, and the causes of cholestasis are mainly direct tumor involvement of the liver, hepatotoxic effects of drugs, viral hepatitis, sepsis and opportunistic infections. Vanishing bile duct syndrome (VBDS) represents a very rare cause for cholestasis in this disease. We report here on a case of a 45-year-old man who developed VBDS during the complete remission stage of Hodgkin's lymphoma. There was no history of hepatitis or intravenous drug abuse, and the patient had negative results for hepatitis A virus, hepatitis B virus, hepatitis C virus, cytomegalovirus, and human immunodeficiency virus. The serological studies for antinuclear antibodies, anti-mitochondrial antibodies and anti-smooth muscle antibodies were also negative. Liver biopsy disclosed the absence of interlobular bile ducts in 9 of 10 portal tracts without any active lymphocyte infiltration and there were no Reed-Sternberg cell in the liver. The patient's cholestasis was in remission and the serum bililrubin level was normalized after two months without treatment, but tumor recurrence was noted at multiple sites of the abdominal lymph nodes on follow-up abdomino-pelvic computed tomogram.

Validade da amilasemia e da lipasemia no diagnóstico diferencial entre pancreatite aguda/crônica agudizada e outras causas de dor abdominal aguda / Validity of serum amylase and lipase for the differential diagnosis between acute/acutized chronic pancreatitis and other causes of acute abdominal pain

RACIONAL: Várias doenças abdominais podem cursar com aumento de amilasemia e lipasemia. OBJETIVO: Avaliar a validade da amilasemia e lipasemia para os diagnósticos diferenciais entre pancreatite aguda/pancreatite crônica agudizada, doenças das vias biliares, úlcera gastroduodenal perfurada e apendicite aguda. PACIENTES E MÉTODOS: Foram avaliados, prospectivamente, 38 pacientes com pancreatite aguda/pancreatite crônica agudizada, 35 com doenças das vias biliares, 17 com úlcera gastroduodenal perfurada e 44 com apendicite aguda, com idade média (desvio padrão) de 42,4 ± 17,7, 46,7 ± 18,3, 47,8 ± 12 e 33,7 ± 17,8 anos, respectivamente. A amilasemia e a lipasemia foram determinadas à admissão no pronto-socorro. RESULTADOS: Para o diagnóstico de pancreatite aguda/pancreatite crônica agudizada, quando o nível de corte da amilasemia variou entre o limite superior de referência e 5 vezes este limite, a sensibilidade diminuiu de 92 por cento para 74 por cento, a especificidade aumentou de 85 por cento para 99 por cento, o valor preditivo positivo aumentou de 71 por cento para 97 por cento e o valor preditivo negativo diminuiu de 96 por cento para 91 por cento. Para a lipasemia valores semelhantes foram obtidos para sensibilidade e valor preditivo negativo, mas a especificidade e o valor preditivo positivo foram mais baixos. Quando se considerou amilasemia ou lipasemia elevadas, houve pequeno aumento na sensibilidade e no valor preditivo negativo. CONCLUSÕES: Para o diagnóstico de pancreatite aguda/pancreatite crônica agudizada, 1) o melhor nível de corte para ambos os testes foi o de duas vezes o limite superior de referência; 2) as sensibilidades da amilasemia e da lipasemia foram semelhantes; 3) a especificidade e o valor preditivo positivo da amilasemia foram ligeiramente maiores do que as da lipasemia; 4) a sensibilidade, mas não a especificidade, aumentou quando pelo menos uma das enzimas estava elevada.

Obstructive jaundice due to compression of the common hepatic duct by right hepatic artery: a case associated with the absence of the lateral segment of the left hepatic lobe

Various benign and malignant conditions can cause biliary obstruction. We present a rare case of obstructive jaundice due to the compression of the common hepatic duct by the anteriorly overriding right hepatic artery. This case was also associated with the absence of the lateral segment of the left hepatic lobe. The patient was a 39 year-old housewife with a 4-day history of jaundice and occasional febrile sensation. An abdominal computed tomography showed absence of the lateral segment of the left hepatic lobe and a percutaneous transhepatic cholangiography showed a band-like filling defect of 2 mm width at the level of the upper common hepatic duct. The anteriorly overriding right hepatic artery compressing the common hepatic duct and the absence of the lateral segment of the left hepatic lobe were confirmed by operation.

Punción aspirativa intraoperatoria del liquido biliar: informe preliminar

Los autores presentan los resultados preliminares de un estudio doble ciego prospectivo y randomizado realizado durante seis meses (de noviembre de 1988 a abril de 1989), en treinta pacientes con indicación de cirugía biliar, a fin de determinar la presencia o no de contaminación en vías biliares. Se practicó punción intraoperatoria de las vías biliares y el material obtenido fué sometido a examen directo, coloración de GRAM, cultivo en aerobiosis y anaerobiosis, y prueba de sensibilidad a los antibióticos y quimioterápicos. Se buscó determinar la necesidad o no del uso rutinario de antibióticos en pacientes sometidos a cirugía de vías biliares, utilizando como parámetro la presencia de bacterias en el material así obtenido. Los resultados indican que en el 61% de los casos la bilis no estaba contaminada siendo innecesaria la utilización rutinaria de antibióticos, práctica habitual en nuestro medio. Y que en pacientes mayores de 50 años sí debe hacerse profilaxis con antibióticos dado el alto índice de contaminación encontrado en estos pacientes

Hemobilia: consideraçöes sobre dois casos

É apresentado um estudo clínico e patológico da hemobilia e enfatizada sua raridade. Dois casos säo descritos: no primeiro, a hemobilia foi complicaçäo de uma colecistie aguda hemorrágica; no segundo, foi decorrente de uma ulceraçäo da vesícula biliar. Ambos foram tratados pela colecistectomia, com excelentes resultados