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

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          Abstract

          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.

          Translated abstract

          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.

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          Choledochal cysts: age of presentation, symptoms, and late complications related to Todani's classification.

          The aim of this study was to compare presentation, complications, diagnosis, and treatment of choledochal cysts in pediatric and adult patients. Forty-two patients were analyzed after subdivision into 3 groups: group A, less than 2 years (n = 10); group B, 2 to 16 years (n = 11); group C, greater than 16 years (n = 21). The cysts were classified as extrahepatic (n = 33), intrahepatic (n = 5), and combined (n = 4). Seventy-six percent of patients presented with abdominal pain, (20 of 21 group C), and 57% with jaundice, (10 of 10 group A). Cholangiocarcinoma occurred in 6 patients, 4 of whom had previously undergone internal drainage procedures. Excision of the extrahepatic cyst was performed in 27 of 37 patients. Five patients, of whom, 4 had cholangiocarcinoma, were beyond curative treatment at the time of diagnosis. Six patients had died at the closure of this study, 5 of them had carcinoma. Presenting symptoms are age dependent with jaundice prevailing in children and abdominal pain in adults. In view of the high risk of cholangiocarcinoma, early resection and not internal drainage is the appropriate treatment of extrahepatic cysts. Patients who had undergone internal drainage in the past still should undergo resection of the cyst. Copyright 2002, Elsevier Science (USA). All rights reserved.
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            Management of choledochal cyst: 30 years of experience and results in a single center.

            Choledochal cyst is usually diagnosed in childhood. Early treatment can prevent further complication. We report on our series of patients over the past 30 years.
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              Risk factors for cholangiocarcinoma.

              Cholangiocarcinoma occurs with a varying frequency in different areas of the world. Some of the variations in incidence rates can be explained by the distribution of risk factors in different geographic regions and ethnic groups. Several accepted risk factors for cholangiocarcinoma include infestation with liver flukes, primary sclerosing cholangitis, hepatolithiasis, choledochal cysts, cirrhosis, and infusion of certain chemical agents. Approximately, 90% of patients diagnosed with cholangiocarcinoma do not have a recognized risk factor for the malignancy. The study by Ahrens et al. [16] finds that obesity and gallstones are risk factors for developing extrahepatic cholangiocarcinoma in men patients. Obesity was found to have a 'dose-effect' relationship with the strength of statistical association. No significant association was reported for tobacco or alcohol use, hepatitis, cirrhosis, diabetes, or inflammatory bowel disease. Although the author's definition of extrahepatic cholangiocarcinoma was unusual, the association of obesity with the risk of developing cholangiocarcinoma persisted for all anatomic subsites.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                rcbc
                Revista do Colégio Brasileiro de Cirurgiões
                Rev. Col. Bras. Cir.
                Colégio Brasileiro de Cirurgiões (Rio de Janeiro )
                1809-4546
                October 2014
                : 41
                : 5
                : 331-335
                Affiliations
                [1 ] Universidade Federal do Rio de Janeiro Brazil
                [2 ] Universidade Federal do Rio de Janeiro Brazil
                [3 ] Universidade Federal do Rio de Janeiro Brazil
                Article
                S0100-69912014000500331
                10.1590/0100-69912014005006
                25467097
                06c6800f-4911-457f-97e9-9434b1009982

                http://creativecommons.org/licenses/by/4.0/

                History
                Product

                SciELO Brazil

                Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=0100-6991&lng=en
                Categories
                SURGERY

                Surgery
                Choledochal Cyst,Jaundice,Bile duct,Anastomosis, Roux-en-Y,Cholangiocarcinoma,Child,Cisto do Colédoco,Icterícia,Ducto Biliar,Anastomose em Y de Roux,Colangiocarcinoma,Criança

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