Coledocolitiasis múltiples y colangitis en paciente con quiste de colédoco: a propósito de un caso / Multiple choledocholithiasis and cholangitis in a patient with choledochal cyst: a case report

INTRODUCCIÓN: El quiste de colédoco (QC) es una patología infrecuente, caracterizada por una dilatación de vía biliar intra o extrahepática. Constituye una lesión congénita, representado 1% de las lesiones biliares benignas. Tiene una incidencia de 1 en 100.000 a 150.000 habitantes. Es más frecuente en mujeres, y su etiología es desconocida. En adultos los síntomas son inespecíficos; predominando dolor abdominal e ictericia. PRESENTACIÓN DEL CASO: Mujer de 61 años con cólico abdominal en hipocondrio derecho de tres días, vómitos e ictericia. Al ingreso hospitalario presentaba leucocitosis, hiperbilirrubinemia, aumento de fosfatasa alcalina, transaminasas y amilasa. Se plantearon los diagnósticos de ictericia obstructiva, pancreatitis y quiste hidatídico complicado, por lo que se realiza tomografía computada (TC) de abdomen evidenciando dilatación sacular intra y extrahepática, compatible con QC tipo IV-a. Se realizó colecistectomía y coledocostomía con sonda T de urgencia por evolución a colangitis con resultados favorables. DISCUSIÓN: Los QC son una causa rara de ictericia obstructiva. En Chile existen escasos datos estadísticos al respecto. Se manifiesta con una sintomatología inespecífica, sobretodo en adultos. El diagnóstico se realiza con hallazgos de laboratorio concordantes con ictericia colestásica, donde los estudios imagenológicos como ultrasonido y TC tienen un rol importante, pese a que en algunas ocasiones pueden pasar inadvertido. Es primordial un alto índice de sospecha para el diagnóstico y un tratamiento oportuno debido a su importante riesgo de progresión a colangiocarcinoma

INTRODUCTION: Choledochal cysts (CCs) is a rare disease characterized by dilatation of the intrahepatic or extrahepatic bile duct, which is about 1% of all benign biliary lesions. Its incidence is 1:100,000 to 150,000 habitants. It is more common in females, and its etiology is unknown. In adults the symptoms are nonspecific, predominantly abdominal pain and jaundice. CASE REPORT: 61 year old female patient with three days of severe abdominal colic in the right upper quadrant, whit both vomiting and jaundice. On admission, she presents leukocytosis, hyperbilirubinemia, and increased levels of alkaline phosphatase, transaminases and amylase. Diagnosis of obstructive jaundice, pancreatitis and complicated hydatid cyst arising. The abdominal CT Scan reveals intra and extrahepatic saccular dilatations, compatible with a type IV-a CCs. Both cholecystectomy and T-tube choledochotomy were done by evolution to cholangitis with favorable results and satisfactory postoperative. DISCUSSION: CCs is a rare cause of obstructive jaundice, and in this regard, there are few data described in Chile, Its diagnosis requires a high index of suspicion because of its nonspecific symptoms found mostly in adults. Despite this, the diagnosis is determined with laboratory findings consistent with cholestatic jaundice and support diagnostic imaging such as ultrasound, CT Scan, among others. Although the imaging findings, it may not be detected. A correct diagnosis and appropriate treatment is essential because of its high risk of progression to cholangiocarcinoma. Currently the patient is waiting for resection of extrahepatic bile duct and Roux-en-Y hepatic jejunostomy which is the optimal treatment.

Cisto de colédoco com apresentação em idade adulta / Choledochal cyst presenting in adulthood

Cistos biliares são ectasias ductais congênitas envolvendo um segmento ou toda árvore biliar intra- e/ou extra-hepática, comuns na infância, mas de diagnóstico raro na idade adulta. O objetivo do presente trabalho é de relatar o caso de um paciente adulto diagnosticado com cisto gigante de colédoco durante investigação para dor abdominal (AU)

Biliary cysts are congenital ductal ectasias involving a segment or the entire intra- and/or extrahepatic biliary tree, common in childhood, but of uncommon diagnosis in adulthood. The aim of this study is to report the case of an adult patient diagnosed with giant choledochal cyst during investigation for abdominal pain (AU)

A Case of Intrahepatic Cholangiocarcinoma Associated with Type IV Choledochal Cyst / 대한소화기학회지

Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.

Ictericia colestásica neonatal: por quiste de colédoco en hospitaltipo III / Neonatal cholestatic jaundice: choledochal cyst in a type III hospital

La ictericia colestásica neonatal representa un grupo de desórdenes hepatobiliares, constituyendo urgencia médica. Un diagnóstico a descartar es el quiste coledociano. Su tratamiento es quirúrgico por asociación a colangiocarcinoma, existiendo otras complicaciones: daño hepático, pancreatitis aguda. Se presenta el primer caso clínico de quiste coledociano en el hospital de Parral, recinto tipo III. Lactante menor femenino con disminución de 10 por ciento del peso de nacimiento, ictericia leve hasta región inguinal, masa abdominal derecha. lmageneología muestra quiste coledociano 9x9x8cm, derivándose a centro terciario para abordaje quirúrgico, evolucionando en buenas condiciones. Se enfatiza conducta activa ante hiperbilirrubinemia neonatal por riesgo de etiología obstructiva.

Quistes de colédoco en adultos / Choledochal cysts in adults

Introducción: Los quistes de colédoco en adulto son muy raros ya que la mayoría se diagnostica antes de los 10 años de edad. Los síntomas más frecuentes son dolor abdominal e ictericia, principalmente en mujeres. El objetivo de esta investigación fue informar la experiencia en el manejo de quistes de colédoco en adultos en un hospital de tercer nivel. Material y métodos: Estudio retrospectivo, descriptivo, observacional y transversal de la evolución posoperatoria de pacientes adultos con diagnóstico de quistes de colédoco tratados en un servicio de cirugía general en un periodo de 17 años. Resultados: Fueron en total 23 pacientes, de los cuales 16 fueron mujeres, con una mediana de edad de 26 años. Los síntomas principales fueron dolor abdominal (87 %) e ictericia (57 %). Solo cuatro pacientes (17 %) tuvieron además de estos síntomas tumor abdominal. El método diagnóstico más utilizado fue ultrasonido (100 %) seguido de colangiografía endoscópica (78 %) y tomografía computarizada de abdomen (74 %). De acuerdo con la clasificación de Todani, el tipo de quiste fue I en 17 pacientes, II en dos pacientes, III en tres pacientes y IV-A en un paciente. Todos fueron intervenidos quirúrgicamente, a 18 se les realizó resección del quiste y hepatoyeyuno anastomosis, a dos resección del quiste y a tres esfinteroplastia transduodenal. Hubo complicaciones posoperatorias en siete pacientes (35 %) y no hubo mortalidad operatoria. Solo en uno se informó colangiocarcinoma. Conclusiones: La cirugía de elección para el manejo de los quistes de colédoco es la resección del mismo, con morbilidad y mortalidad aceptables.

BACKGROUND: Choledochal cysts are usually diagnosed during childhood. They mainly affect females. Abdominal pain and jaundice are the two most common symptoms. Our objective was to report the experience of a third-level referral center in the management of choledochal cysts in adults. METHODS: We performed a 17-year retrospective review of patients with choledochal cysts who underwent surgical interventions in a gastrointestinal surgery department. RESULTS: There were 23 patients with a median age of 26 years; 16 were females. The most frequent symptoms were abdominal pain and jaundice. Only four patients had an abdominal mass. The most frequently used diagnostic studies were abdominal sonography and endoscopic retrograde cholangiopancreatography. According to the classification of Todani, most cysts were type I and were found in 17 patients, two patients had type II, three patients had type III (choledocele) and one patient had type IVA. Eighteen patients underwent complete removal of the cyst with a concomitant hepatic-jejunostomy, three patients had transduodenal sphincteroplasty and two patients had only cystectomy. Seven patients developed postoperative complications, representing a morbidity rate of 35%. None of the patients died during the first 30 postoperative days. Cholangiocarcinoma was found in the resected specimen in only one case. CONCLUSIONS: The surgery of choice for choledochal cysts in the adult is hepatojejunostomy, which has a low surgical morbidity and mortality.

Pancreas divisum and choledochal cyst.

Pancreas divisum (PD) is the most common congenital variant of the pancreas, affecting 5% to 14% of the population. The ventral duct only drains the ventral pancreas through the major papilla, whereas the majority of the pancreas drains via the dorsal duct through the minor papilla. We report the case of a 21-year-old woman with recurrent acute pancreatitis who presented with the rare finding of choledochal cyst and pancreas divisum (PD). She underwent minor papilla sphincterotomy and pancreatic duct stenting. Comparable literature findings of PD and choledochal cyst are discussed with regard to the presented case.

Spontaneous perforation of the extrahepatic biliary tract.

Spontaneous perforation of the extra-hepatic biliary tract presenting with pseudocyst is rare. We report the case of a two-month old infant who presented to us with increasing jaundice and progressive loss of weight. Ultrasonography, magnetic resonance cholangiopancreatography and hepatobiliary iminodiacetic acid scan revealed features of obstructed choledochal cyst. Laparotomy revealed a small, walled-offcollection of bile near the confluence of the cystic and common bile ducts. Distal obstruction was excluded and biliary tract drainage was successful in treating the infant.

Spontaneous perforation of choledochal cyst and hyperemesis gravidarum.

A 28-year-old primigravida at seven weeks gestation presented to the Accident & Emergency Department complaining of vomiting for five days. She was admitted and managed as a case of hyperemesis gravidarum. Two days later she developed abdominal distension. Ultrasound scan revealed ascites and common bile duct dilatation. The ascitic fluid was bile stained. At emergency laparotomy spontaneous perforation of the supraduodenal part of the common bile duct was seen. This was closed around a T-tube. She then underwent medical termination of pregnancy. The post-operative T-tube cholangiogram was suggestive of a type I choledochal cyst. Three months later this was excised and biliary enteric continuity restored by performing a hepaticojejunostomy. To the best of our knowledge, spontaneous rupture of a choledochal cyst in a patient of hyperemesis gravidarum has not been reported before. In this article, we discuss treatment options for choledochal cyst during pregnancy.

Perforated choledochal cyst in a Jehovah's Witness patient.

Perforation is a rare presentation of a choledochal cyst. The case reported is a 9-month-old female baby with a perforated choledochal cyst. Being a Jehovah's Witness, blood tranfusion was refused. Apart from highlighting this social dilemma, the suitability of a cystojejunostomy as a temporary measure in the above scenario is evaluated and discussed.

Quiste de colédoco en la gestación y puerperio: reporte de dos casos y revisión de la literatura / Choledochal cyst in the pregnancy and puerperium: report of two cases and revision of literature

El quiste de colédoco consiste en la dilatación quística de la vía biliar intrahepática o extrahepática. Según la teoría mas aceptada, es causado por una anormalidad de la unión biliopancreática. Sus complicaciones más importantes son colangiocarcinoma, litiasis biliar, y pancreatitis, y su terapia es la resección quirúrgica. Sólo 20 por ciento a 30 por ciento de los casos se diagnostican en la vida adulta. Se reportan dos casos de quiste de colédoco en pacientes femeninas adultas, una de ellas al final de la gestación, y la otra puérpera. El diagnóstico de quiste de colédoco en la gestación y puerperio es un evento inusual que tiene consideraciones propias en cuanto a presentación y manejo.

A choledochal cyst is a cystic dilation of the intrahepatic or extrahepatic biliary tract. According to the most accepted theory, it is caused by an anomalous pancreatobiliary junction. The most important complications are cholangiocarcinoma, lithiasis, andpancreatitis. Current therapy is surgical resection. Only 20 per cent to 30 per cent of cases are diagnosed in adult life. Two cases of choledocal cysts are reported in female adult patients, one of them in late pregnancy and the other in puerperium. Diagnosis of choledochal cyst in pregnancy and puerperium is an uncommon event, entailing particular considerations regarding symptoms and treatment.

Spontaneous infantile choledochal cyst perforation.

Spontaneous perforation is a rare complication of infantile choledochal cysts. A 23-weeks female with acute biliary peritonitis is described and differentiation from a spontaneous perforation of the extrahepatic bile duct highlighted.

Quiste de colédoco en el adulto / Choledochal cyst in adults

Antecedentes: El quiste de colédoco en el adulto es una entidad poco frecuente. Existe una asociación significativa entre quiste de colédoco y neoplasias biliares. Objetivo: El objetivo del trabajo es evaluar métodos diagnósticos y el tratamiento de pacientes adultos con quiste de colédoco. Lugar de aplicación: Centro público terciario de referencia. Diseño: Estudio observacional descriptivo retrospectivo de cohorte transversal. Población: Cinco pacientes adultos intervenidos por quiste de colédoco. Método: Fueron utilizados para el diagnóstico ecografía, colangiografía, tomografía axial computada (TAC) y resonancia nuclear magnética. Los quistes fueron agrupados según la clasificación de Todani. Los tratamientos efectuados fueron una gastroenteroanastomosis y 4 resecciones de la vía biliar extrahepática con hepaticoyeyunoanastomosis en Y de Roux. Resultados: Cuatro pacientes tenían quiste tipo I y la última un quiste tipo IV b. No se constató morbilidad ni mortalidad operatoria. El seguimiento se efectuó hasta la actualidad. La enferma del caso n° 1 falleció por progresión de su enfermedad neoplásica. Las 4 restantes evolucionan favorablemente. Conclusiones: El potencial carcinogenético justifica la conducta quirúrgica de resección. La cirugía de elección en los quistes tipo I y IV es la resección completa de la vía biliar extrahepática con hepático yeyuno anastomosis en Y de Roux

Management Strategy for Congenital Choledochal Cyst with Co-existing Intrahepatic Dilation and Aberrant Bile Duct As Well As Other Complicated Biliary Anomalies

The purpose of this study was to investigate and discuss imaging methods and management strategies for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies. In this study we reviewed and analyzed 72 patients with congenital choledochal cyst, ranging in age from 15 days to 12 years old and who were seen at our hospital during the past 12 years, from January 1993 to October 2005. The image manifestation and clinical significance of patients with co- xisting intrahepatic biliary dilation and aberrant bile duct were carefully examined during operation via MRCP, cholangiography and choledochoscope. Twenty-two cases (30.1%) presented with intrahepatic bile duct dilation and 12 of these were of the cystic type. That is, the orifice of the dilated intrahepatic tract that converged into the common hepatic duct showed membrane or septum-like stenosis. In 10 cases the dilation tapered off from the porta hepatis to the initiating terminals of the intra-hepatic bile ducts and was not accompanied by stenosis. An aberrant bile duct was observed in 2 of the cases. In 3 cases, the right and left hepatic ducts converged at the choledochal cyst. In conclusion, the imaging methods for intrahepatic bile duct dilation possess important clinical significance. Further, for hepatojejunostomy with radical excision of a choledochal cyst, additional operative procedures for intrahepatic stenosis, possible bile duct malformation and pancreaticobiliary common duct calculi can potentially reduce postoperative complications.

Management Strategy for Congenital Choledochal Cyst with Co-existing Intrahepatic Dilation and Aberrant Bile Duct As Well As Other Complicated Biliary Anomalies

The purpose of this study was to investigate and discuss imaging methods and management strategies for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies. In this study we reviewed and analyzed 72 patients with congenital choledochal cyst, ranging in age from 15 days to 12 years old and who were seen at our hospital during the past 12 years, from January 1993 to October 2005. The image manifestation and clinical significance of patients with co- xisting intrahepatic biliary dilation and aberrant bile duct were carefully examined during operation via MRCP, cholangiography and choledochoscope. Twenty-two cases (30.1%) presented with intrahepatic bile duct dilation and 12 of these were of the cystic type. That is, the orifice of the dilated intrahepatic tract that converged into the common hepatic duct showed membrane or septum-like stenosis. In 10 cases the dilation tapered off from the porta hepatis to the initiating terminals of the intra-hepatic bile ducts and was not accompanied by stenosis. An aberrant bile duct was observed in 2 of the cases. In 3 cases, the right and left hepatic ducts converged at the choledochal cyst. In conclusion, the imaging methods for intrahepatic bile duct dilation possess important clinical significance. Further, for hepatojejunostomy with radical excision of a choledochal cyst, additional operative procedures for intrahepatic stenosis, possible bile duct malformation and pancreaticobiliary common duct calculi can potentially reduce postoperative complications.

Pancreatitis aguda: manifestaciones clínica de quiste del colédoco / Acute pancreatitis: manifestation clinic of choledochal cyst

Los quistes del colédoco son dilataciones saculares o fusiformes, locales o generalizadas del árbol biliar. Son la segunda malformación de vías biliares después de la atresia de vías biliares; siendo más frecuente en el sexo femenino. El 80 por ciento de los casos se diagnóstican en la primera década de la vida. En el neonato los quistes del colédoco por lo general se manifiestan por ictericia persistente, a menudo con una masa abdominal palpable en hipocondrio derecho. En niños mayores y adultos se puede presentar como manifestación de una de sus complicaciones: colangitis, pancreatitis, hipertensión portal y peritonitis biliar por ruptura del quiste. Sólo 30 por ciento de los pacientes desarrollan la tríada clásica de ictericia, dolor abdominal y masa en hipocondrio derecho. La ecografía es el método diagnóstico de elección,otros estudios como la tomografía, colangioresonancia, la colangiopancreatografía retrógrada endoscópica son útiles para confirmar el diagnóstico o cuando se desea descartar afección intrahepática concomitante. Presentamos a continuación a una preescolar de 2 años de edad, la cual debuta con una pancreatitis aguda de evolución tórpida como complicación de quiste del colédoco

Quiste del colédoco y carcinoma adenoescamoso / Cyst of the choledochal cyst and carcinoma adenosguamous

La frecuencia de la malignidad en el quiste de colédoco es una rara condición y debe ser sospechada en paciente con síntomas de colangitis, pérdida de peso y anemia. El pronóstico de esta entidad es pobre, su incidencia aumenta con la edad, y el diagnóstico casi siempre es tardío. Se han establecido algunas teorías con base a su oncogénesis. El tipo histológico más frecuente es el adenocarcinoma y muy raro la variedad escamosa. Presentamos el caso de una paciente a quien a los 49 años se le diagnosticó esta afección en la pared posterior de un quiste del colédoco tipo 1 según la clasificación de Todani

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.

Colangiorresonancia magnética: experiencia inicial / MR cholangiography: experience in 186 patients

La colangiorresonancia magnética (CRM) es una técnica especial de utilización creciente, realizada con RM de cuerpo, que permite el estudio de la vía biliar en forma incruenta, especialmente en casos de ictericia obstructiva. Presentamos nuestra experiencia inicial con 2D fast Spin Echo con cortes finos (3mm). Se describirá la técnica y se analizarán los resultados obtenidos, con respecto al brusco y beneficioso cambio producido en el manejo actual del paciente ictérico