Hemobilia como forma inusual de presentación en la enfermedad de Caroli / Hemobile as an unusual presentation form of the Caroli´s disease

La enfermedad de Caroli, o ectasia comunicante de las vías biliares intrahepáticas, es una entidad congénita, caracterizada por múltiples dilataciones saculares o quísticas de las vías biliares intrahepáticas. En este trabajo se hace presentación de un nuevo caso portador de enfermedad de Caroli. Se trata de una paciente de raza blanca, de 26 años de edad, con antecedentes de buena salud anterior, que ingresa por fiebre elevada de 39 ºC, hepatomegalia dolorosa e imágenes hipoecoicas en el hígado, detectadas en la ultrasonografia, con aminotransferasas hepáticas ligeramente elevadas. En el segundo ingreso sufre complicaciones febriles y de sangramiento digestivo alto por hemobilia importante, que compromete la hemodinamia y junto a la sepsis dan al traste con su vida. La paciente ingresa por crisis de colangitis y presentó un hemobilia importante que la llevó a complicaciones hemodinámicas, colangitis ascendente, y finalmente a sepsis generalizada como causa de muerte.

Caroli´s disease, or communicant ectasia of the intrahepatic biliary ducts, is a congenital entity, characterized by multiple saccular or cystic dilatations of the intrahepatic biliary ducts. In this work we present a new case of a carrier of Caroli’s disease. It deals with a female patient, aged 26 years, with antecedents of good health status, who entered the hospital with high fever of 39oC, painful hepatomegaly and hyper echoic images in the liver, detected in the ultrasonography, with slightly raised hepatic aminotranferases. During the second staying in the hospital she suffered fever complications and high digestive bleeding caused by important hemobile that compromised the hemodynamics and together with sepsis caused her to die. The patient entered the hospital because of cholangitis and presented an important hemobile leading her to hemodynamic complications, ascendant cholangitis, and finally a generalized sepsis as death cause.

Hemobilia como forma inusual de presentación en la enfermedad de Caroli / Hemobile as an unusual presentation form of the Caroli´s disease

La enfermedad de Caroli, o ectasia comunicante de las vías biliares intrahepáticas, es una entidad congénita, caracterizada por múltiples dilataciones saculares o quísticas de las vías biliares intrahepáticas. En este trabajo se hace presentación de un nuevo caso portador de enfermedad de Caroli. Se trata de una paciente de raza blanca, de 26 años de edad, con antecedentes de buena salud anterior, que ingresa por fiebre elevada de 39 ºC, hepatomegalia dolorosa e imágenes hipoecoicas en el hígado, detectadas en la ultrasonografia, con aminotransferasas hepáticas ligeramente elevadas. En el segundo ingreso sufre complicaciones febriles y de sangramiento digestivo alto por hemobilia importante, que compromete la hemodinamia y junto a la sepsis dan al traste con su vida. La paciente ingresa por crisis de colangitis y presentó un hemobilia importante que la llevó a complicaciones hemodinámicas, colangitis ascendente, y finalmente a sepsis generalizada como causa de muerte(AU)

Caroli´s disease, or communicant ectasia of the intrahepatic biliary ducts, is a congenital entity, characterized by multiple saccular or cystic dilatations of the intrahepatic biliary ducts. In this work we present a new case of a carrier of Carolis disease. It deals with a female patient, aged 26 years, with antecedents of good health status, who entered the hospital with high fever of 39oC, painful hepatomegaly and hyper echoic images in the liver, detected in the ultrasonography, with slightly raised hepatic aminotranferases. During the second staying in the hospital she suffered fever complications and high digestive bleeding caused by important hemobile that compromised the hemodynamics and together with sepsis caused her to die. The patient entered the hospital because of cholangitis and presented an important hemobile leading her to hemodynamic complications, ascendant cholangitis, and finally a generalized sepsis as death cause(AU)

Findings of the UK national audit evaluating image-guided or image-assisted liver biopsy. Part II. Minor and major complications and procedure-related mortality.

PURPOSE: To determine the frequency of complications and death following image-guided and/or image-assisted liver biopsy and to identify significant variables associated with an increased risk of complications or death. MATERIALS AND METHODS: Institutional review board approval for this type of study is not required in the United Kingdom. United Kingdom radiology departments with a department leader for audit registered with the Royal College of Radiologists were invited to participate. The first 50 consecutive patients who underwent liver biopsy in 2008 were included. Audit standards were developed for minor pain (<30%), severe pain (<3%), vasovagal hypotension (<3%), significant hemorrhage (<0.5%), hemobilia (<0.1%), puncture of another organ (<0.1%), and death (<0.1%). Organizational, clinical, and coagulation variables were investigated statistically for their association with complications and/or death. RESULTS: Data were obtained from 87 of 210 departments (41%). Audit standards were met for pain, hypotension, hemorrhage, hemobilia, and puncture of another organ. There were four hemorrhage-related deaths, and this target was narrowly missed (rate achieved in practice, 0.11% [four of 3486 patients]). Fifteen additional patients experienced at least one major complication. The international normalized ratio (INR) was absent in 3% of cases (97 of 2951 patients), the platelet count was absent in 1% (32 of 2986 patients), the INR was more than 1 week old in 8% (229 of 2888 patients), and the platelet count was more than 1 week old in 10% (291 of 2955 patients). CONCLUSION: Results of this audit confirm that image-guided and image-assisted biopsy is performed safely in United Kingdom radiology departments, with complication rates within expected parameters. Preprocedural clotting assessment was inadequate in some cases and would merit repeat audit. SUPPLEMENTAL MATERIAL: http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.12120224/-/DC1.

[Causes of death in liver cirrhosis patients seen on an outpatient basis]. / Prichini za smurtta pri bolni s chernodrobna tsiroza, nabliudavani pri dispanserni usloviia.

The immediate cause of death were studied in 122 patients with cirrhosis of liver, out of 236 patients with cirrhosis of liver under follow-up care observation in the course of 12 years. The average age of the deceased is 53,8 years, with a ratio males: females--2,3:1. The deceased were grouped according to the type of liver cirrhosis as follows: micronodular--63,1%, macronodular--15,6%, mixed--9,8%, primary biliary--6,6%, hemochromatosis--, 4,1% and the disease of Wilson--Konovalov--0,81%. Decompensated stage of cirrhosis was established in 77,9% from the deceased at the last examination and compensated--21,1%. Immediate cause for the death in the summed up group cirrhoses is: hepatic coma in 42,2%, acute hemorrhage with or without coma--32,3%, other causes--18,9%. Degeneration of liver cirrhosis into cancer was established in 9,01%. Cirrhosis degenerated more frequently in males. Hemochromatosis and micronodular cirrhosis are the ones that most frequently degenerated.

[Endoscopic sphincterotomy. 362 cases (author's transl)]. / Sphinctérotomies oddiennes endoscopiques. 362 cas.

In addition to its diagnostic value, endoscopic catheterisation of the papilla may be used for the therapeutic purpose of diathermy incision of the papilla and of the intraduodenal portion of the common bile duct. Endoscopic sphincterotomy of this type has been essentially used in choletithiasis and in particular for residual lithiasis. These indications may be widened to include benign stenosis of the sphincter of Oddi, tumours of the ampulla of Vater or even acute pancreatitis. In a total of 362 cases, sphincterotomy was successful in 347 patients, it being possible to remove the calculi present in 279 of the 309 patients treated with success for lithiasis of the common bile duct. Complications occur in approximately 10% of cases, being fatal in 1.4% of cases. These are essentially related to bleeding. Retrospective comparison of mortality after surgical operation and after endoscopic sphincterotomy is in favour of the latter, provided the technique employed is perfect and the indication clearly defined. Performed without general anaesthesia, endoscopic sphincterotomy is above all indicated in the elderly and/or those with other major pathology who can be successfully treated with reduced risk in 93% of cases, as shown by the 140 personal cases of the authors cured between 6 and 36 months ago.