Guía de práctica clínica en dilatación congénita de las vías biliares en la edad pediátrica / Clinical practice guidelines in congenital dilation of biliary tract in pediatric ages

Las dilataciones congénitas de los conductos biliares, son poco frecuentes y se pueden descubrir en cualquier etapa de la vida, incluido el período prenatal. Es una afección que puede ser asintomáticas, ocasionar síntomas escasos o producir graves complicaciones, razones por las cuales, la actitud actual del especialista se enfoca hacia el diagnóstico temprano y el tratamiento correcto de las diferentes variantes de dilatación congénita de la vía biliar antes que aparezcan las complicaciones mencionadas. El objetivo de publicar esta guía responde a la necesidad de contar con herramientas actualizadas en el manejo de estas afecciones que permitan sistematizar la conducta y mejorar la calidad de la asistencia médica, la docencia y las investigaciones y está aprobada por la Sociedad Cubana de Cirugía Pediátrica. Se realizó una extensa revisión de la literatura científica basada en la mejor evidencia publicada mundialmente sobre el tema. Se presentan el concepto de esta afección, su epidemiología, clasificación, etiología, cuadro clínico, formas de presentación, diagnóstico y conducta a seguir con los diferentes tipos, así como las medidas posoperatorias y de seguimiento del paciente. Si todos los procedimientos se realizan de forma correcta y en el momento oportuno, el pronóstico es favorable en la mayoría de los casos(AU)

Congenital dilation in the biliary duct is not common and can be found in any stage of life, including the prenatal period. It is a condition that can be asymptomatic, causing scarse symptoms or producing severe complications; therefore, the current performance of specialists must be focused towards the early diagnosis and the accurate treatment of the different types of congenital dilation of the biliary tract before the above mentioned complications appear. The aim of publishing this guidelines has to do with the need of having updated tools for the management of these conditions which allow to systematize the behaviours and to improve the quality of medical care, teaching and researches, and also because it is approved by the Cuban Society of Pediatric Surgery. It was made an extensive review of the scientific literature based in the best evidences published worldwide on the topic. It is presented the concept of this condition, its epidemiology, classification, etiology, clinical picture, ways of presentation, diagnosis and behaviour to follow with the different types of it, as well as the patient's post-operative and follow-up measures. If all the procedures are made in a proper way and in the opportune moment, the prognosis is favorable in most of the cases(AU)

Choledochal cysts in children: How to Diagnose and Operate on

OBJECTIVE: To identify the best mode for diagnosing and treating the patients with choledochal cysts. METHODS: A retrospective study was performed with medical records of patients diagnosed with choledochal cysts from January 1994 to December 2017. In all cases, the diagnosis was based on ultrasound examination. All the patients underwent cyst resection and were divided in two groups: bile enteric anastomosis in the high portion of the common hepatic duct or in the dilated lower portion. RESULTS: Eighty-one cases were studied. The age of presentation was 4 y 2 mo ± 4 y 1 mo, and the age for the surgical treatment was 5 y 5 mo ± 4 y 6 mo. In 61 cases, US was the only image examination performed. There were 67 cases of Todani type I (82.7%), 13 cases of type IV (16.0%) and one case of type III (1.2%). Nine patients (29.0%) in the first period and 2 patients (4.0%) in the second period presented with postoperative complications (p=0.016). CONCLUSION: In patients with choledochal cysts, US is the only necessary diagnostic imaging. Performing the bile enteric anastomosis in the lower portion of the common hepatic duct is safer and has a lower risk of complications.

Quiste gigante del colédoco en Pediatría / Giant bile duct cyst in Pediatrics

Introducción: los quistes del colédoco son infrecuentes en nuestro medio. Su etiología es desconocida, y su diagnóstico se realiza usualmente en los primeros años de vida. Presentación del caso: se reporta el caso de un niño de 5 años que presenta dolor abdominal de 6 meses de evolución, asociado a vómitos y masa abdominal. Conclusiones: los estudios imagenólogicos concluyen que se trata de quiste de colédoco tipo I(AU)

Introduction: bile duct cysts are uncommon in Pediatrics. Its etiology is unknown, and its diagnosis is usually made in the first years of life. Presentation of the case: the case of a 5-year-old boy with abdominal pain of 6 months' evolution, associated with vomiting and abdominal mass is reported. Conclusions: imaging studies conclude that it is a type I choledochal cyst(AU)

Choledochal cyst in the pediatric population: experience of 13 laparoscopic procedures in two years at a single institution / Cisto de colédoco na população pediátrica: experiência de 13 procedimentos laparoscópicos em dois anos de uma única instituição

ABSTRACT Objective: to describe the first 13 cases of laparoscopic correction of common bile duct cyst in the Pequeno Príncipe Hospital, Curitiba, Paraná, Brazil. Methods: we performed a retrospective analysis of medical records of cases of choledochal cyst operated by laparoscopy between March 2014 and September 2016. Results: of the 13 patients, eight were female and the mean age at surgery was 7.8 years. The most common symptom was abdominal pain. The hepaticoduodenal anastomosis was the most used reconstruction technique, in 84.6% of the cases. There was no conversion to laparotomy or intraoperative complications. Only one patient presented anastomotic fistula and was reoperated by laparotomy. All patients were followed up in an outpatient clinic, were asymptomatic and had no episode of cholangitis after surgery, with a mean follow-up of 16 months. Conclusion: laparoscopy is a safe method to correct choledochal cysts, even in younger children, with low rates of complications and low rates of conversion to open surgery when performed by well trained surgeons.

RESUMO Objetivo: descrever os primeiros 13 casos de correção laparoscópica de cisto do ducto biliar comum no Hospital Pequeno Príncipe, Curitiba, Paraná, Brasil. Métodos: análise retrospectiva dos registros médicos em prontuário dos casos de cisto de colédoco operados por via laparoscópica entre março de 2014 e setembro de 2016. Resultados: dos 13 pacientes, oito eram do sexo feminino e a média de idade na ocasião da cirurgia foi de 7,8 anos. O sintoma mais comum foi dor abdominal. A anastomose hepático-duodenal foi a técnica de reconstrução mais utilizada, em 84,6% dos casos. Não houve conversão para laparotomia ou complicações intraoperatórias. Apenas um paciente apresentou fístula da anastomose e foi reoperado por laparotomia. Todos permanecem em acompanhamento ambulatorial, com tempo de seguimento médio de 16 meses, assintomáticos e não apresentaram episódio de colangite após a cirurgia. Conclusão: a laparoscopia é um método seguro para correção dos cistos de colédoco, mesmo em crianças mais jovens, com baixas taxas de complicações e baixas taxas de conversão para cirurgia aberta quando realizada por cirurgiões com bom treinamento.

Resección y hepaticoyeyunostomía laparoscópicas en quiste de colédoco tipo 1 / Laparoscopic resection and hepaticojejunostomy for type 1 choledochal cyst

Los quistes del colédoco son dilataciones congénitas de la vía biliar que afectan fundamentalmente a la población pediátrica, de ellas, el tipo 1 es el más frecuente. Pueden producir graves complicaciones y el tratamiento quirúrgico de elección es la resección total de la lesión seguida de hepaticoenterostomía. Actualmente, este proceder se realiza por vía laparoscópica con buenos resultados. El objetivo del trabajo es reportar el tratamiento laparoscópico de un quiste de colédoco tipo 1 en un paciente de 5 años. No hubo complicaciones posoperatorias y los resultados estéticos y funcionales al año de seguimiento son excelentes. Se concluye que la resección laparoscópica en quistes de colédoco tipo 1 es posible y se sugiere continuar con su implementación(AU)

Choledochal cysts are congenital dilatation of the bile ducts that typically affect the pediatric population. Cases of its type 1 are the more frequent. Serious complications may occur and the surgical treatment of election is the lesions' total resection followed by hepaticoenterostomy. Today this procedure is carried out by laparoscopic way with good outcomes. The objective of this paper is to report a choledochal cyst type 1´s laparoscopic treatment in a 5-year-old patient. There were not postoperative complications and the aesthetic and functional results after one-year follow-up are excellent. The choledochal cysts´ laparoscopic resection is concluded to be possible and its implementation is suggested to follow(AU)

Caso Clínico: Quiste de Colédoco Tipo IA, Resolución Quirúrgica / Case Report: Type IA Choledochal Cyst, Surgical Resolution

INTRODUCCIÓN: El quiste del colédoco es una malformación congénita de etiología desconocida, caracterizada por diversos grados de dilatación del sistema biliar. El examen inicial para la valoración de la vía biliar debe ser una ecografía abdominal,sin embargo, la resonancia magnética con contraste es el estudio de elección para valorar estas estructuras. El tratamiento quirúrgico final depende del grado de dilatación quística y generalmente está basado en la escisión completa de la vía biliar extrahepática. CASO CLÍNICO: Paciente de sexo femenino de 27 años con dolor abdominal tipo cólico a nivel de epigastrio e hipocondrio derecho de 7 días de evolución. Se realizaron exámenes de Imagenología (ecografía­ TAC ­colangio RM)que reportaron colédoco con marcada dilatación fusiforme en toda su extensión, en relación a quiste de colédoco tipo IA. EVOLUCIÓN: Se planificó la exéresis del quiste de colédoco, anastomosis bilio-digestiva en «Y de Roux¼ y colecistectomía. El procedimiento quirúrgico no tuvo complicaciones. La paciente permaneció hospitalizada por 5 días con adecuada evolución y tolerancia a dieta. CONCLUSIÓN: El quiste de colédoco es una patología congénita infrecuente en la edad adulta generalmente diagnosticada como un hallazgo incidental. El diagnóstico y tratamiento oportuno aseguran el éxito del procedimiento quirúrgico y evita la aparición de complicaciones graves (colangiocarcinoma); con la escisión completa el pronóstico es excelente, la mortalidad posoperatoria es mínima. (au)

BACKGROUND: The choledochal cyst is a congenital malformation of unknown etiology characterized by different grades of dilatation of the biliary system. The first exam to evaluate the bile duct should be the abdominal ultrasound, which suggests the diagnosis of choledochal cyst. However, the magnetic resonance imagin with contrast is the gold standard to assess these structures. The final surgery treatment depends of the grade of cyst dilatation, the main procedure is based on the complete excision of the extrahepatic bile duct. CASE REPORT: A female 27-years old patient who reffered colic abdominal pain localized in epigastrium and right hypochondrium for 7 days. Imaging exams were performed (US­CT Scan­ choledochal MRI) which reported the bile duct with a severe fusiform dilatation, in complete extension related to type IA choledochal cyst. EVOLUTION: Choledocal cyst exeresis, «Roux-en-Y¼ bilio digestive anastomosis and cholecystectomy were planned. Surgical procedure had no complications. Patient was hospitalized for 5 days with proper evolution and oral diet tolerance. CONCLUSION: The choledochal cyst is a rare congenital disease in adult hood mostly diagnosed as an incidental finding. Early diagnosis and treatment ensure the success of the surgical procedure and prevents appearance of serious complications (cholangiocarcinoma).The prognosis after complete excision is excellent with minimal postoperative mortality(au)

Diagnóstico y tratamiento del quiste del colédoco neonatal. Reporte de casos / Diagnosis and treatment strategy of neonatal choledochal cyst: Cases Report

Se reportan 2 casos de quiste de colédoco neonatal sintomáticos, uno de ellos con diagnóstico prenatal, que fueron llevados a tratamiento quirúrgico, realizando la resección de quiste del colédoco, derivación bilioentérica tipo hepático-yeyuno anastomosis en Y de Roux y colocación de drenaje de Penrose. En seguimiento de 20 meses en promedio con adecuada evolución.

We report two cases of symptomatc neonatal choledochal cysts, one of them prenatally diagnosed, who had surgical treatment with choledochal cyst resecton and Roux en Y hepato-jejunal anastomosis and Penrose drain. Follow up at 20 months (average) with good outcomes.

Doenças císticas das vias biliares / Cystic diseases of the bile ducts

JUSTIFICATIVA E OBJETIVOS: Os ductos biliares podemapresentar dilatações congênitas ou adquiridas. As lesões congênitaspodem ser hipoplásticas (atresias) e císticas. O primeiroestudo descrito na literatura foi realizado por Vater and Ezler in1723. Os cistos biliares são isolados ou múltiplos, e aparecemem qualquer idade, mas 75% deles são antes dos 20 anos. Elespodem ocorrer em toda árvore biliar, sendo, as intra-hepáticasrestritas, conhecida como doença de Caroli. O objetivo desseestudo foi fazer uma revisão sobre as Doenças císticas das viasbiliares. CONTEÚDO: As dilatações císticas extra-hepáticaatingem diversos tamanhos e são classificadas segundo sua formae localização. São elas: fusiforme, divertículos e dilataçõessaculares. Háuma incidência variada dessas lesões com câncer,entre 2,5% e 17,5%. O quadro clínico mais comum éa colestaseneonatal, correspondendo a 80% dos pacientes. Na idadeadulta, podem ser assintomáticos ou apresentar apenas sintomasinespecíficos como náuseas,vômitos. A dificuldade de drenagemda colestase obstrutiva pode lavar a cirrose biliar secundária.CONCLUSÃO: O ultrassom de abdome pode confirmar odiagnóstico. No tocante ao laboratório, observa-se uma hiperbilirrubinemiae elevação de enzimas canaliculares. A terapêuticaempregada na maioria dos casos écirúrgica, com excisão do cistoe reconstrução da drenagem biliar, devendo-se evitar a drenagemisolada, pois tais pacientes tendem a evoluir com complicaçõescomo fístulas, estenose, colangite e carcinoma.(AU)

BACKGROUND AND OBJECTIVES: The bile duct dilatationmay have congenital or acquired. Congenital lesions can behipoplásticas (atresia) and cystic. The first study described in theliterature was performed by Vater and Ezler in 1723. Biliary cystsare isolated or multiple and appear at any age, but 75% of themare before age 20. They can occur throughout the biliary tree,and the intrahepatic restricted, known as Caroli disease. Theaim of this study was to review about the cystic biliary diseases.CONTENTS: Dilated cystic extrahepatic reach various sizesand are classified according to their shape and location. Theyare: fusiform, saccular dilatation and diverticula. There is anincidence of these lesions with cancer varied between 2.5% and17.5%. The most common symptom is a neonatal cholestasis,corresponding to 80% of patients. In adulthood, may beasymptomatic or show only nonspecific symptoms such as nausea,vomiting. The difficulty of draining obstructive cholestasis canwash the secondary biliary cirrhosis. CONCLUSION: Theabdominal ultrasound can confirm the diagnosis. Regarding thelaboratory, there is a hyperbilirubinemia and elevated GGT. Thetherapy used in most cases is surgical excision of the cyst andreconstruction of biliary drainage, should be avoided drainagealone because these patients tend to develop complications suchas fistula, stricture, cholangitis and carcinoma.(AU)

Optimización en la estrategia diagnóstica y terapéutica en el quiste de colédoco / Optimization in diagnostic and therapeutic strategy of choledochal cyst

Objectives: The choledochal cyst has an incidence of 1 in 100.000-150.000. The purpose of this study was to analyze the variables introduced for the optimization in the diagnosis and treatment of choledochal cyst. Material and Methods: Retrospective study of patients treated in our center by hepatic-jejunostomy and Roux-Y, from September 1988 to November 2012. We analyzed 40 variables including age, symptoms, type of cysts, diagnostic tests, changes in surgical technique, complications and outcomes. Results: Eighteen patients (66.6 percent female) were grouped according to the age of presentation: Prenatal (< 1 month), Early (124), Delayed (> 24 months). The most common symptoms were jaundice and abdominal pain (for early-onset and late-onset respectively). The 83.3 percent presented choledochal cysts type I, the ultrasound was sufficient for diagnosis in 94.4 percent. Since 2004 we modified the surgical technique, performing laparoscopic dissection of the bile duct and cyst, adding a mini-laparotomy (3-5 cm) for hepatic-jejunostomy with 40 cm intestinal loop using polypropylene suture. One complication was observed since 2004, one case of partial dehiscence of the anastomosis resolved with conservative treatment and a cholangitis in 1 patient with hepatic and renal polycystic. In 2012, 83.3 percent are asymptomatic. Conclusions: The diagnostic have been simplified, in more than 90 percent of cases was done by ultrasound; advances in minimally invasive surgery and creation of descending loop of 40 cm, have helped to improve the prognosis of choledochal cyst.

Objetivos: El quiste de colédoco presenta una incidencia de 1 en 100.000-150.000. El propósito de este estudio fue analizar las variables introducidas para la optimización en el diagnóstico y tratamiento del quiste de colédoco. Material y Métodos: Estudio retrospectivo de pacientes intervenidos en nuestro centro de quiste de colédoco mediante hepático-yeyunostomía en Y de Roux, desde septiembre de 1988 a noviembre de 2012. Se analizan 40 variables incluyendo edad, sintomatología, tipo de quiste, pruebas diagnósticas, cambios en la técnica quirúrgica, complicaciones y evolución. Resultados: 18 pacientes (66,6 por ciento mujeres) fueron agrupados de acuerdo a la edad de presentación: Prenatal (< 1 mes); Precoz (1-24 meses); Tardía (> 24 meses). Los síntomas más frecuentes fueron ictericia y dolor abdominal (para los de inicio precoz y tardío respectivamente). El 83,3 por ciento presentaban quistes de colédoco tipo I; siendo suficiente la ecografía para el diagnóstico en el 94,4 por ciento. A partir de 2004 modificamos la técnica quirúrgica, realizando por vía laparoscópica la disección de la vía biliar y mediante laparotomía mínima (3-5 cm) hepático-yeyunostomía con asa descendente de 40 cm, empleando sutura de polipropileno, observando desde el 2004, sólo 1 dehiscencia parcial de la anastomosis, resuelta con tratamiento conservador y 1 colangitis en paciente con poliquistosis hepática y renal. En el año 2012 el 83,3 por ciento están asintomáticos. Conclusiones: El diagnóstico se ha simplificado, en más de 90 por ciento de los casos se realiza sólo con ecografía; los avances en cirugía mínimamente invasiva y la creación de asa descendente de 40 cm, han logrado optimizar el pronóstico del quiste de colédoco.

Quiste de colédoco gigante / Giant choledochal cyst

La dilatación quística del colédoco es una entidad rara. Tiene mayor incidencia en los países orientales que en los occidentales y predomina en el sexo femenino. Se diagnostica habitualmente en la infancia y se han postulado varias hipótesis para su origen entre las que se destacan las anomalías en la unión biliopancreática. Se presenta el caso de una paciente de 19 años de edad con un quiste de colédoco tipo IV de Todani con varios años de evolución. Finalmente recibió, una derivación como tratamiento definitivo; la evolución fue inmediata y satisfactoria(AU)

Choledochal cysts are rare and has higher incidence rate in the Eastern countries than in the Western nations. It is predominant in females. It is regularly diagnosed in the childhood and several hypotheses have been formulated about their origin, being one of them the biliopancreatic junction anomalies. This is the case of a 19 years-old patient with a Todani´s type IV choledochal cyst with several years of progression. He was finally performed a shunt as definitive treatment and his recovery was immediate and satisfactory(AU)

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.

Retrospective Assessment of the Validity of Robotic Surgery in Comparison to Open Surgery for Pediatric Choledochal Cyst

PURPOSE: We evaluated the validity of robotic surgery (RS) for pediatric choledochal cyst (CC) in comparison to open surgery (OS). MATERIALS AND METHODS: From January 2009 to April 2013, clinical data from 79 consecutive pediatric patients with CC, who underwent RS (n=36) or OS (n=43) performed by a single pediatric surgeon, were analyzed retrospectively. RESULTS: In the RS group, the age of the patients was significantly older, compared to the OS group. Operation and anesthesia times were significantly longer in the RS group than the OS group. Fluid input rates to maintain the same urine output were significantly smaller in the RS group than the OS group. The American Society of Anesthesiologists (ASA) physical status, length of postoperative hospital stay, and the incidence of surgical complications did not differ significantly between the two groups. CONCLUSION: Although early complications could not be avoided during the development of robotic surgical techniques, RS for pediatric CC showed results comparable to those for OS. We believe that RS may be a valid and alternative surgery for pediatric CC. After further development of robotic surgical systems and advancement of surgical techniques therewith, future prospective studies may reveal more positive results.

Choledochal cyst in childhood: review of 30 cases / Cisto de colédoco na infância: revisão de 30 casos

Objective: To analyze and discuss the clinical data, diagnosis and treatment of a number of patients with cystic dilatation of the common bile duct of a Brazilian pediatric hospital. Methods: We analyzed 30 patients treated at the Martagão Gesteira Institute of Pediatrics and Child Care of the Federal University of Rio de Janeiro for 23 years ,with statistical analysis of epidemiological data, clinical manifestations, diagnosis, treatment and postoperative outcome. Results: We observed a marked female predominance (73.4% of cases), the diagnosis being made in the first decade of life in 90% of patients. The most prevalent clinical manifestation was jaundice (70% of cases) and the classic triad of choledochal cyst was not observed. Abdominal ultrasound was the first imaging examination performed, with a sensitivity of 56.6%, with diagnostic definition in 17 children. Two patients (6.6%) had prenatal diagnosis. All patients underwent surgical treatment, cyst resection with Roux-en-Y hepaticojejunostomy being performed in 80% of cases. The incidence of postoperative complications was 13.3% and the mortality rate was 6.6%, ie two patients were diagnosed with Caroli's disease. Conclusion: The non-observance of the classic triad of choledochal cyst suggests that its incidence is lower than that reported in the medical literature. The surgical treatment of choledochal cysts, with resection and bilioenteric anastomosis, is safe even for small children. .

Objetivo: analisar e discutir os dados clínicos, o diagnóstico e tratamento de uma série de pacientes portadores de dilatações císticas do colédoco de um hospital pediátrico brasileiro. Métodos: foram analisados 30 pacientes tratados no Instituto de Pediatria e Puericultura Martagão Gesteira da Universidade Federal do Rio de Janeiro durante 23 anos, com análise estatística de dados epidemiológicos, manifestações clínicas, diagnóstico, tratamento e evolução pós-operatória. Resultados: foi observada marcada predominância do sexo feminino (73,4% dos casos), sendo o diagnóstico feito na primeira década de vida em 90% dos pacientes. A manifestação clínica mais prevalente foi a icterícia (70% dos casos) e a tríade clássica do cisto de colédoco não foi observada. A ultrassonografia abdominal foi o primeiro exame de imagem realizado, demonstrando sensibilidade de 56,6%, com definição diagnóstica em 17 crianças. Dois pacientes (6,6%) tiveram diagnóstico pré-natal. Todos os pacientes foram submetidos a tratamento cirúrgico, sendo a ressecção do cisto com hepaticojejunostomia com Y de Roux realizada em 80% dos casos. A incidência de complicações pós-operatórias foi 13,3% e a taxa de mortalidade foi 6,6%, ou seja, dois pacientes com diagnóstico de doença de Caroli. Conclusão: a não observação da tríade clássica do cisto de colédoco nos pacientes avaliados sugere que a sua incidência seja menor que a relatada na literatura médica mundial. O tratamento cirúrgico dos cistos de colédoco, com sua ressecção e anastomose bíleodigestiva é seguro, mesmo em crianças pequenas. .

unusual cause of pain in the right hypochondrium

Choledochal cyst is an uncommon anomaly of the biliary system. It manifests by cystic dilation of the extra or intrahepatic biliary tract or both. Three cases of choledochal cysts are reported, who had presented with pain in right hypochondrium. All the three patients presented in one Surgical Unit of a Teaching Hospital within a span of three months. The first patient was initially managed as obstructive jaundice, second as acute pancreatitis and third patient as a case of pancreatic pseudocyst. However, after investigations, the final diagnosis in all three of them was the same i.e. choledochal cyst which was managed by Roux-en-Y hepatico-jejunostomy

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)

Quistes de colédoco: un diagnóstico inadvertido. Serie de casos / Choledochal cysts: an inadvertent diagnosis. Case series

Las lesiones quísticas de vías biliares, constituyen una entidad, que rara vez se observa o diagnostica en el adulto. El motivo de este trabajo es mostrar la experiencia de un centro de referencia biliopancreática en el diagnóstico y tratamiento de quistes de colédoco. Usamos la clasificación de Todani para la descripción de los mismos. Fue un estudio Descriptivo y Retrospectivo con 502 colangiopancreatografías retrógradas endoscópicas y de ellas 20 casos de quistes de colédoco identificados mediante colangiografías de alta calidad.

Cystic lesions of the bile ducts constitute an entity that is rarely seen or diagnosed in adults. The purpose of this study is to show the experience of a biliopancreatic referral center in the diagnosis and treatment of choledochus cysts. The Todani classification was used to describe them. It was a retrospective, descriptive study of 502 endoscopic retrograde cholangiopancreatographies and of these 20 cases of choledochal cysts were identified by high-quality cholangiographies.

Quiste del colédoco: presentación de quince casos / Choledochal cyst: presentation of fifteen cases

El quiste del colédoco es una rara anomalia congénita que ocurre en los países occidentales con una frecuencia de 1 por cada 2 millones de nacidos vivos. De predominio en el sexo femenino en una relación 4:1. La colangiografia permite clasificarlos en cinco tipos anatómicos y de acuerdo a la unión pancreático biliar en seis tipos. Su etiología no está todavía bien definida. Entre las hipótesis que tratan de explicar su patología están la debilidad de la pared del conducto biliar, la obstrucción distal del colédoco y el reflujo de enzimas pancreáticas al conducto biliar principal. En este informe se presentan quince casos tratados con éxito en el servicio de cirugía ifantil del Hopsital Unversitario de Maracaibo entre 1988 y 2008, todos del tipo 1 en hembras, con un poco más de la mitad entre 1 y 5 años de edad. En 14 casos se practicó la extirpación total con anastomosis hepato-yeyunal en "Y" de Roux y extirpación parcial en uno. Todos sobrevivieron. Entre las complicaciones se citan una dehiscencia de la anastomosis y una eventración posoperatoria en el mismo paciente.

Choledochal cysts are a rare congenital anomaly in occidental countries with a frequences of 1 x 2 million of live births. Predominant in females with a relation 4:1. By colangiography they are classify in five types and according to the pancreatic bile union in six types. Aetiology is still unknown. Among the hypothesis to explain pathogeny we have weak bile un conduct wall, distal choledochal obstruction and pancreatic enzymes reflux into the principal bile duct. In his report we present 15 treated cases with success between 1988 and 2008 in the surgical service for children of the Maracaibo university hospital, all of them type I in female patients with a little more tham half between I and 5 years old. In 14 cases we practice total extirpation with hepato-yeyunal anastomosis in Roux "Y" and partial extirpation in one. All of them survive. As complications they were one anastomosis dehiscence and one postoperative eventration in the same patient.

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.