Ansa pancreática: desafio no diagnóstico etiológico em paciente jovem com pancreatite grave / Ansa pancreatica: etiological diagnosis challenge in a young patient with severe pancreatitis

A ansa pancreática é uma variação anatômica rara dos ductos pancreáticos. Consiste numa comunicação entre o ducto pancreático principal (Wirsung) e o ducto pancreático acessório (Santorini). Recentemente, estudos têm demonstrado estar essa variação anatômica implicada como fator predisponente e significativamente associada a episódios recorrentes de pancreatite aguda. A pancreatite é uma entidade clínica pouco frequente na infância. Diferente dos adultos, as causas mais comuns incluem infecções virais, por ascaris, medicamentosas, traumas e anomalias estruturais. O objetivo deste estudo foi relatar um caso de pancreatite aguda grave não alcoólica e não biliar, em um paciente jovem de 15 anos, em cuja propedêutica imagenológica evidenciou-se alça, comunicando com os ductos pancreáticos ventral e dorsal, compatível com ansa pancreática.

Ansa pancreatica is a rare anatomical variation of the pancreatic ducts. It consists of communication between the main pancreatic duct (Wirsung) and the accessory pancreatic duct (Santorini). Recently, studies have shown that this anatomical variation is implicated as a predisposing factor and significantly associated with recurrent episodes of acute pancreatitis. Pancreatitis is a rare clinical entity in childhood. Different from that in the adults, the most common causes include viral and ascaris infections, drugs, traumas, and structural abnormalities. The objective of this study was to report a case of a severe non-alcoholic and non-biliary acute pancreatitis in a 15-year-old patient, whose propedeutic imaging showed a loop communicating with the ventral and dorsal pancreatic ducts, consistent with ansa pancreatica.

Intraductal Ultrasonography without Radiocontrast Cholangiogram in Patients with Extrahepatic Biliary Disease

BACKGROUND/AIMS: Intraductal ultrasonography (IDUS) has been performed as an adjunct to endoscopic retrograde cholangiography (ERC) during radiocontrast cholangiography (RC). Radiation exposure during RC poses a health risk to both patients and examiners. We evaluated the feasibility of IDUS without RC in various extrahepatic biliary diseases. METHODS: IDUS was performed with the insertion of an IDUS probe from the papilla of Vater to the confluent portion of the common hepatic duct without fluoroscopy. The technical success rate and procedure-related complications were evaluated retrospectively. RESULTS: Wire-guided IDUS without RC was performed in 105 patients. The mean age was 66.5 years, and 50 (47.6%) were male. The IDUS diagnoses included choledocholithiasis (73, 69.5%), benign biliary stricture (11, 10.5%), choledocholithiasis with biliary pancreatitis (9, 8.6%), bile duct cancer (5, 4.8%), pancreatic cancer (1, 0.9%), and others (6, 5.7%). After IDUS, 66 (62.8%) underwent stone removal, 19 (18.1%) underwent biliary drainage, and 7 (6.6%) underwent brush cytology and biopsy. No significant complications such as perforation or severe pancreatitis occurred. CONCLUSIONS: IDUS without RC was a feasible and safe approach in patients with various extrahepatic biliary diseases. We anticipate a potentially important role of IDUS in various ERC procedures because it lacks the hazards of RC.

Ictericia obstructiva secundaria a tumor de células granulares de la vía biliar extrahepática / Obstuctive jaundice secondary to extrahepatic bile ducts granular cell tumor

Bile ducts granular cell tumor is a rare entity. Of neural origin, mostly benign, may, however, present mimicking malignancy. We report a 32 years old female presenting with painless jaundice and extrahepatic bile ducts stenosis confirmed with MRC. Extrahepatic bile ducts resection is performed. Reconstruction involves four independent ducts to a Roux en Y enteric loop. She has a good postoperative outcome, with no evidence of complications nor recurrence at 17 months of follow up.

El tumor de células granulares en la vía biliar es una neoplasia rara de origen neural, en su mayoría benigna y cuya presentación puede sugerir patología maligna. Objetivo: Se presenta el caso clínico, características anatomopatológicas, manejo y evolución de una paciente joven que se presenta con ictericia obstructiva por estenosis subcarinal biliar. Paciente y Método: Paciente 32 años, sexo femenino, con ictericia, coluria y prurito. Diagnóstico de estenosis biliar y dilatación de vía biliar intrahepática se confirma con colangiorresonancia magnética. Se realiza resección de vía biliar extrahepática desde supracarinal que incluye vía biliar distal. Reconstitución bilioentérica a Y de Roux que involucra cuatro conductos intrahepáticos. Evoluciona en forma satisfactoria en el postoperatorio. El seguimiento alejado a 17 meses revela una satisfactoria condición de la paciente, sin signos de complicación o recidiva. Conclusión: El manejo por un equipo de experiencia multidisciplinario nos permitió ayudar a una paciente con rara patología, benigna en lo histológico, pero que puede representar un gran desafío técnico.

Influence of glutamine on morphological and functional changes of liver in the presence of extrahepatic biliary obstruction in rats / Influência da glutamina em alterações funcionais e morfológicas do fígado na vigência de obstrução biliar extra-hepática em ratos

PURPOSE: To study the influence of glutamine on functional and morphological changes of liver in the extrahepatic biliary obstruction through an experimental model in rats. METHODS: Seventy Wistar rats were divided into four groups: control (group C) fictitious operation, (group FO), submitted to laparotomy with handling of bile ducts, but without hepatic duct ligation, (group EBO) submitted to laparotomy and hepatic duct ligation, one of them submitted to supplementation with glutamine 2 percent (group G). The control group consisted of 6 animals. The animals from groups FO, EBO and G were divided into three groups consisting of 6 animals each, being sacrificed at 7, 14 and 21 days after operation, respectively. Blood samples were collected for biochemical analysis and a fragment of liver tissue was collected from the middle lobe for histological analysis. RESULTS: Both for biochemical analysis (BT, aspartate and alanine aminotransferase AST, ALT and alkaline phosphatase FAL) and for histopathological changes (fibrosis, portal inflammation, parenchymal inflammation, hepatocytic changes and duct proliferation), no statistical difference between groups submitted to extrahepatic biliary obstruction (EBO) with and without treatment with glutamine (G) was observed. CONCLUSION: Glutamine supplementation did not alter the prognosis of liver enzymes and histopathological changes in animals submitted to extrahepatic biliary obstruction.

OBJETIVO: Estudar a influência da glutamina em alterações funcionais e morfológicas do fígado na obstrução biliar extra-hepática por meio de um modelo experimental desenvolvido em ratos. MÉTODOS: Setenta ratos Wistar distribuídos em quatro grupos: controle (grupo C); operação fictícia (grupo OF), submetido à laparotomia com manuseio das vias biliares, mas sem ligadura do ducto hepático; (grupo OBE), submetido à laparotomia exploradora e ligadura do ducto hepático, sendo um deles submetido à suplementação com glutamina a 2 por cento (grupo G). O grupo controle era composto por seis animais. Os animais dos grupos OF, OBE e G foram divididos em três grupos compostos por seis animais cada e que foram sacrificados no 7°, 14° e 21° dias após a operação, respectivamente. Foi colhido sangue para análise bioquímica e um fragmento de tecido hepático do lobo médio para estudo histológico. RESULTADOS: Tanto em relação à analise bioquímica (BT, aspartate and alanine aminotransferase AST, ALT e FAL) quanto em relação às alterações histopatológicas (fibrose, inflamação portal, inflamação parenquimatosa, alterações hepatocíticas e proliferação de ducto), não houve diferença estatística entre os grupos submetido a obstrução biliar extra-hepática sem (OBE) e com tratamento com glutamina (G). CONCLUSÃO: A suplementação com glutamina não alterou o prognóstico em relação às enzimas hepáticas e alterações histopatológicas nos animais submetidos à obstrução biliar extra-hepática.

Small Cell Carcinoma of Extahepatic Bile Duct Presenting with Hemobilia / 대한소화기학회지

We report a case of small cell carcinoma of extrahepatic bile duct presenting with jaundice and hemobilia. A 59-year-old woman was admitted due to right upper quadrant pain and jaundice. An abdominal computed tomography revealed a 2 cm sized mass in the extrahepatic bile duct. Endoscopic retrograde cholangiopancreatography revealed bloody discharge coming out of the papillary orifice in endoscopic view and a dilated extrahepatic bile duct with multiple irregular filling defects in cholangiogram. A coronal T2-weighted image revealed a hyperintense mass at extrahepatic bile duct. Laparotomy was performed, and pathologic examination of resected specimen showed tumor cells having round to oval nuclei with coarsely granular chromatin and scanty cytoplasm, which were immunoreactive for synaptophysin and chromogranin A, compatible with the diagnosis of small cell carcinoma. The small cell carcinoma of bile duct, despite its rarity, should be considered in differential diagnosis of the causes for obstructive jaundice and hemobilia.

A Case of Biliary Cast Syndrome after Cadaveric Liver Transplantation / 대한소화기학회지

We experienced one fatal case of biliary cast syndrome after cadaveric liver transplantation involving both intrahepatic ducts. A 58-year-old man underwent cadaveric liver transplantation because of hepatitis B virus related liver cirrhosis and concomitant hepatocellular carcinoma. Five weeks after the liver transplantation, postoperative course was complicated by development of acute cholangitis. Subsequent endoscopic retrograde cholangiography revealed diffuse intrahepatic bile duct strictures without filling defects. Percutaneous liver biopsy, which was done to exclude rejection, revealed biliary cast. Successful endoscopic removal was precluded due to its diffuse involvement. Because of the deterioration of patient's condition by refractory biliary obstruction and cholangitis, retransplantation from cadaveric donor was performed. Debridement of the biliary tree after graft removal yielded a near-complete cast of the intrahepatic ductal system. Biliary cast syndrome should be suspected when jaundice or cholangitis is associated with dilated ducts on abdominal imaging studies in cadaveric liver transplantation recipients. Initial therapeutic options include removal of biliary cast after endoscopic or percutaneous cholangiography. Although endoscopic retrieval of biliary cast by endoscopic retrograde cholangiopancreatography could be employed as a first-line management, other modalities such as endoscopic nasobiliary drainage, percutaneous transhepatic drainage, or retransplantation should be considered when complete removal is not feasible and the condition of the recipient deteriorates.

Hepatobiliary cystadenoma with mesenchymal stroma--a case report.

Hepatobiliary cystadenoma with mesenchymal stroma (HCMS) is a rare lesion characterized by multiloculated cyst lined by columnar epithelium ad mesenchymal stroma. It occurs exclusively in females and is intrahepatic in location with only 17% cases arising in extrahepatic biliary tree. Exact histogenesis is not known, but it is hypothesized the HCMS arises from ectopic embryonic tissue destined to form the adult gall bladder. HCMS has got a malignant potential and requires radical excision.

Forme fruste choledochal cyst.

Two girls presenting with features of pancreatitis were diagnosed to have minimal dilatation of extra hepatic biliary duct (EHBD) associated with pancreatico-biliary maljunction (PBMJ). A high degree of suspicion is required to diagnose this condition that has been termed Forme fruste choledochal cyst (FFCC). Both did well with pancreatico-biliary disconnection procedure and reconstruction of biliary channel using enteric conduit.

Extrahepatic Biliary Atresia / 대한간학회지

No abstract available.

Hamartoma de la vía biliar. Reporte de un caso / Bile duct hamartoma. Report of a case

Antecedentes: las obstrucciones de la vía biliar en general son provocadas por cálculos y neoplasias. Existen otras alteraciones mucho menos frecuentes que pueden provocar obstrucción, entre ellas se encuentran los hamartomas de la vía biliar. Se presenta el caso de una paciente de 34 años diabética, que inicia su padecimiento con dolor abdominal e ictericia. El estudio inicia con ultrasonido de vesícula y vías biliares, que reporta colelitiasis y dilatación de vías biliares. Se realiza CPRE y se descubre una lesión ocupativa del árbol biliar. Se somete a colecistectomía y exploración de la vía biliar. La evolución de la paciente fue satisfactoria. La revisión de patología reporta un hamartoma de la vía biliar extrahepática. Los hamartomas de la vía biliar son neoplasias benignas poco frecuentes, sin embargo, debemos de tenerlas en mente como parte del diagnóstico diferencial.

Non-syndromic paucity of interlobular bile ducts.

A 3 1/2-month-old male infant presented with cholestatic jaundice. Peroperative cholangiogram showed a gall bladder and small but patent extrahepatic bile ducts. Liver histology showed paucity of interlobular bile ducts. The child is being treated medically for his symptoms.

Endoscopic and laparoscopic treatment of mirizzi syndrome

Endoscopic followed by laparoscopic treatment of Mirizzi syndrome has been shown to be safe and effective. This technique was applied in 15 patients with Mirizzi syndrome. Ten patients had a single large stone and five had multiple stones impacted in the cystic duct. Nasobiliary drainage tube [NBDT] or stent was applied in patients for preoperative drainage of biliary tree and to prevent acute cholangitis by endoscopic retrograde cholangiopancreatography [ERCP]. Laparoscopic cholecystectomy was tried in all patients within 3 days after endoscopic intervention. In 6 cases, conversion was done from laparoscopic to open cholecystectomy. Conversion was due to fistula in three cases, injury to common bile duct in two cases and bleeding from slipped cystic artery in one case. Mortality was zero. Preoperative endoscopic drainage has many advantages including abortion of endotoxaemia in acute cholangitis, lowering the elevated serum bilirubin, improving the clinical condition of the patient, allowing easy identification of the anatomy of the bile ducts intraoperatively and immediate detection of any injury to bile ducts. The presence of stented bile duct allows for primary closure of the injury

Paraganglionoma of extrahepatic biliary tract causing obstructive jaundice.

We report a young woman with paraganglionoma arising from the extrahepatic bile duct presenting with acute obstructive jaundice. The patient underwent excision of the gall bladder and extrahepatic bile duct with the tumor, and Roux-en-Y hepaticojejunostomy. She is asymptomatic 9 months later, with normal biochemical investigations and imaging.

Carcinoma de vesícula biliar: una neoplasia subestimada / Gallbladder carcinoma: an underestimate neoplasia

El carcinoma de vesícula biliar es una neoplasia subestimada en nuestro país, principalmente por tres razones: elevada frecuencia de error diagnóstico, epidemiología poco conocida y decremento en la práctica de autopsias que confirmen la causa de muerte. Hicimos un estudio retrospectivo de 4,769 autopsias consecutivas para comparar la frecuencia del carcinoma de vesícula biliar en el resto de carcinomas de tubo digestivo. Nuestros resultados indican que este tumor es muy frecuente. Ocupó el primer lugar como causa de muerte sobre el resto de neoplasias malignas del tubo digestivo: en general (25.8 por ciento del total), y en mujeres (34.6 por ciento). El diagnóstico clínico fue incorrecto en el 69 por ciento de los casos. La autopsia es obligada si deseamos tener una idea real sobre su frecuencia. Es un error considerar al carcinoma de vías biliares extrahépaticas y al carcinoma de vesícula biliar como una misma entidad, pues tienen diferencias clinicoepidemiológicas considerables. Aunque es muy probable que existe susceptibilidad genética para el carcinoma de vesícula biliar en la población indígena y mestiza del país, la etiopatogenia de esta neoplasia continúa siendo un misterio

Primary cystic duct carcinoma case report and literature review

Primary cystic duct carcinoma is rare. To date only 27 cases have been reported in the English literature, and 17 cases in the Japanese literature. This paper reports another case of primary cystic duct carcinoma. A 70-year-old male presented with signs and symptoms of acute calcular cholecystitis and dilated extrahepatic bile ducts. An ERCP failed technically. An ultrasound-guided aspiration was performed to drain an obstructed gall bladder and an adjacent pyogenic liver abscess. A cholecystogram showed an obstructed cystic duct. On table a tumour was suspected at the junction of the cystic duct and common hepatic duct [CHD]. En masse excision of the gall bladder, cystic duct, tumour, part of the CHD and the supraduodenal part of the common bile duct was performed with a Roux-en-Y hepatico-jejunostomy. Histopathologically the tumour fulfills all of Farrar's criteria for primary cystic duct carcinoma. Our case is of interest since it is the 2nd case to present with Mirrizzi-like syndrome

Colangiocarcinoma / Cholangiocarcinoma

El colangiocarcinoma es uno de los cánceres más raros, siendo el menos frecuente de los del hígado y el tracto biliar. Puede originarse en cualquier nivel del árbol biliar. Los pacientes se presentan clásicamente con una ictericia indolora y prurito, la colangitis no es común. Para su estudio y tratamiento, se clasifican en tres grandes grupos: los carcinomas intrahepáticos, los parahiliares y los distales. Existen también tumores difusos, pero son sumamente raros. Los carcinomas perihiliares son los más comunes. Esta clasificación es útil en el monitoreo de resultados, ya que los tumores intrahepáticos usualmente son manejados como carcinomas hepatocelulares con resección de hígado, mientras que los tumores distales son tratados como cánceres de la cabeza del páncreas, con una pancreatoduodenectomía. Las lesiones perihiliares pueden ser tratadas con una variedad de procedimientos quirúrgicos o no quirúrgicos. La cirugía es el único procedimiento potencialmente curativo. La resecabilidad y el pronóstico depende de la localización del tumor, a través del árbol biliar, la extensión o no al parénquima hepático y la invasión del tumor a los vasos sanguíneos mayores

Primary extranodal non-Hodgkin lymphoma of the extrahepatic bile duct mimmicking Klatskin tumor

Relatamos um caso de linfoma nao-Hodgkin primário das vias biliares extra-hepaticas, cujo sintoma inicial foi ictericia. A colangiografia demonstrou estenose do ducto biliar, de aspecto semelhante a um tumor de Klatskin tipo II. O tratamento cirurgico permitiu completa ressecçäo do tumor e a análise histopatologica mostrou tratar-se de um linfoma näo-Hodgkin folicular centrocitico-centroblastico. No seguimento pos-operatório de 48 meses, a paciente permaneceu assintomatica. O diagnóstico e as operaçöes terapeuticas säo discutidos