Cavernoma portal complicada con biliopatía secundario a enfermedad de Gaucher tipo 1: reporte de un caso peruano / Cavernoma complicated with biliopatia secondary to type 1 Gaucher disease: report of a Peruvian case

La enfermedad de Gaucher, es un trastorno autosómico recesivo de depósito lisosomal que se caracteriza por deficiencia de la beta-glucocerebrosidasa que lleva a la acumulación de glucosilceramida principalmente en células del sistema fagocítico mononuclear causando afectaciones sistémicas. Se presenta paciente varón de 20 años que cursa con dolor crónico en hipocondrio izquierdo con episodios de sangrados desde hace 3 años y sensación de alza térmica, al examen físico se identificó ictericia y esplenomegalia masiva, sin afectación neurológica. Como apoyo al diagnóstico se mostró osteoporosis severa, pancitopenia y como hallazgo inesperado la presencia de trombosis de vena porta con transformación cavernomatosa complicada con biliopatía portal simulando un tumor de klatskin, los estudios de médula y enzimáticos eran compatibles con enfermedad de Gaucher, por lo cual recibió tratamiento con imiglucerasa realizando seguimiento. Es un caso poco frecuente, de gran interés, heterogeneidad en sus manifestaciones clínicas e inéditas por su complicación, constituyendo un desafío llegar a su diagnóstico de esta enfermedad huérfana.

Gaucher disease is an autosomal recessive lysosomal storage disorder characterized by deficiency of beta-glucosidase that would lead to the accumulation of glucosylceramide mainly in cells of the mononuclear phagocytic system causing systemic effectations. We present a patient of twenty years who is suffering from chronic pain in the left hypochondrium with episodes of bleeding for 3 years and sensation of thermal rise, physical examination revealed jaundice and massive splenomegaly, without neurological involvement. Severe osteoporosis, pancytopenia, and the presence of portal vein thrombosis with cavernomatous transformation complicated by portal biliopathy simulating a klatskin tumor, marrow and enzymatic studies were compatible with Gaucher disease, were shown as unexpected findings. he received treatment with imiglucerase, following up. It is a rare case, of great interest, heterogeneity in its clinical manifestations and unpublished by its complication, constituting a challenge to reach its diagnosis of this orphan disease.

La vía biliar intrahepática: clasificación anátomo-quirúrgica con base colangiográfica / Intrahepatic biliary ducts: anatomic and surgical classification after cholangiographic findings

Las variaciones en la constitución de la vía biliar son muy frecuentes. Su conocimiento adquiere importancia en distintos procedimientos quirúrgicos, como la colocación de un drenaje en la vía biliar o una colecistectomía. Sin embargo, el cirujano a menudo la visualiza por primera vez durante el acto quirúrgico, y debe lidiar con clasificaciones complejas para poder comprenderlas. El objetivo de este trabajo es presentar una clasificación sencilla y de rápida interpretación. Se analizaron 100 estudios colangiográficos y se realizaron 10 disecciones cadavéricas, como apoyo al estudio colangiográfico. A partir de los resultados obtenidos, se propuso la siguiente clasificación: Conducto hepático derecho "típico", cuando éste reúne la bilis de toda la porción hepática derecha, o "dividido", cuando sus ramas desembocan separadamente en la vía biliar principal. Conducto hepático izquierdo "típico" o "dividido", siguiendo el mismo criterio. Conducto hepático "central", cuando las secciones parasagitales de ambas porciones hepáticas, derecha e izquierda, se reúnen en un solo conducto. Respecto a la confluencia de los conductos biliares, puede clasificarse en "típica" precisamente cuando ambos conductos hepáticos también lo son, "triple confluencia" cuando uno o ambos conductos hepáticos se encuentran divididos y todos ellos confluyen en un punto, o "escalonada" cuando uno de ellos se encuentra dividido y la confluencia se realiza a distinta altura, en ocasiones con algún conducto segmentario. Creemos que esta clasificación resulta de fácil aplicación por su sencillez, permitiendo identificar todas las estructuras de la vía biliar rápidamente aún sin contar con estudios previos, colaborando de esta manera en una cirugía más segura.

Variations in intrahepatic biliary ducts are frequent. Its knowledge is of great importance when facing certain procedures such as drainage or a simple cholecystectomy to avoid iatrogenic lesion or incomplete drainage of the biliary tract. Nevertheless, it is during surgery that the surgeon attempts to see it for the first time, and must deal with complex classifications in order to recognize the ducts. This paper aims to suggest an easy and quick way to interpret and simple classification. 100 cholangiograms were studied and 10 cadaveric specimens were analyzed to support radiologic findings. As a result, we propose the following classification: Right "typical" biliary duct, when all the bile produced in the right hemiliver is drained by a single duct, or "divided" when sectional ducts reach separately the main bile duct. The same applies to the left hepatic ducts, "typical" or "divided". When both paramedian sections are drained by the same duct, there is a "Central" hepatic duct The biliary confluence may be "typical", when both hepatic ducts are also typical, "triple confluence" when one or both hepatic duct are divided and reach the main bile duct in the same place, or "staggered (selved) confluence" (etagée) when one of the ducts is divided and reaches the main bile duct separately from the others. This name is even proper if a segmentary duct reaches the main bile duct. We think this classification is easy to use due to its simplicity, allowing the surgeon to quickly identify each biliary duct and get through the surgery safely.

Asymptomatic Accessory Bile Duct Draining into the Stomach / 대한소화기학회지

No abstract available.

A Case of Autoimmune Pancreatitis Combined with Extensive Involvement of Biliary Tract / 대한소화기학회지

Autoimmune pancreatitis is a distinct disease characterized by the presence of autoantibodies and hypergammaglobulinemia, inflammation of the pancreatic parenchyma, and irregular stricture of the pancreatic duct. The involvement of distal common bile duct is frequently observed, but intrahepatic bile duct involvement is very rare, which seem to have similar feature to primary sclerosing cholangitis. We report a case of the patient with autoimmune pancreatitis combined with extensive involvement of extrahepatic and intrahepatic bile duct, which had a favorable response to steroid therapy.

Spontaneous rupture of intrahepatic biliary ducts with biliary peritonitis.

Spontaneous rupture of intrahepatic biliary ducts is a rare cause of acute abdomen due to biliary peritonitis. We report a 92-year-old woman with 48-h history of upper abdominal pain, nausea and vomiting and peritoneal signs. CT scan showed free fluid in the abdomen and mild dilatation of the common bile duct. Exploratory laparotomy showed bile in the abdominal cavity with leak-age from a ruptured bile duct radicle in segment 3, as confirmed on intraoperative cholangiography. She underwent cholecystectomy, choledochotomy with removal of gallstones, repair of the perforation with primary suture and placement of a T-tube. She had an uneventful recovery.

A Case of Infected Biloma due to Spontaneous Intrahepatic Biliary Rupture

A "biloma" is a loculated collection of bile located outside of the biliary tree. It can be caused by traumatic, iatrogenic or spontaneous rupture of the biliary tree. Prior reports have documented an association of biloma with abdominal trauma, surgery and other primary causes, but spontaneous bile leakage has rarely been reported. A spontaneous infected biloma, without any underlying disease, is a very rare finding. We recently diagnosed a spontaneous infected biloma by abdominal computed tomography and sonographically guided percutaneous aspiration. The patient was successfully managed with percutaneous drainage and intravenous antibiotics. We report here a case of infected biloma caused by spontaneous rupture of the intrahepatic duct, and review the relevant medical literature.

Applications of Multidetector-row CT for the Imaging Diagnosis of Liver Disease / 대한소화기학회지

Owing to the development of multidetector-row CT (MDCT), capability in the imaging diagnosis of liver disease has increased surprisingly. First, a marked decrease in scan duration of the liver enables us to select the accurate scan time optionally. Secondly, a marked decrease in slice thickness enables us to render isotropic and three-dimensional images completely. In this pictorial review, we described the optimal contrast enhancement of the liver on MDCT and its significance as well as the applications of isotropic and three-dimensional images obtained by MDCT.

Cholangiocarcinoma presenting with recurrent venous thrombosis.

A 38 years female presented with three episodes of venous thrombosis over one year--first in left femoral vein, next in splenic vein causing haematemesis and malena and lastly in inferior vena cava causing Budd-Chiari syndrome. During third episode, endoscopic cholangiopancreatography and guided biopsy established a diagnosis of cholangiocarcinoma at the mid portion of common bile duct. The disease was far advanced with cervical lymph node metastasis.

Modified single step ultrasound guided percutaneous transhepatic biliary drainage (MSSUGPTBD): experience with 102 patients.

Modified single step ultrasound guided percutaneous transheptic biliary drainage was performed in 102 patients between 1993 and 1998 at the National Cancer Institute, Bangkok with successfully placed drainage tubes in the dilated bile ducts. The advantages of this technique are single step puncture without major complication or bleeding, reduction of radiation exposure, capability for bile duct selection and time saving.

Intrahepatic biliary lithiasis.

It has been known that intrahepatic biliary lithiasis (IHBL) is prevalent in East Asia including Japan, South Korea, Taiwan, Malaysia, Hong Kong, and Singapore. In contrast, the entity has drawn little attention in Europe and the United States where only scattered reports appear. IHBL can be placed in the category of the benign disease. Its distinctive clinical picture is an intractable course necessitating multiple surgical interventions because recurrence is usual, rather than exceptional. This is in distinct contrast to ordinal stones which originate in the gallbladder. Patients with IHBL do not rarely die of progressive hepatic damage resulting from longstanding obstructive jaundice, cholangitis, liver abscess, septicemia, and so forth.