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.

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.

Quistes de colédoco, una causa inusual de ictericia en pediatría: presentación de serie de casos / Bile duct cysts; an unusual cause of jaundice in paediatrics: presentation of a case series

Introducción Los quistes de la vía biliar o quistes de colédoco (QC) son una patología rara en nuestro medio. La etiología es desconocida, siendo la hipótesis más aceptada las anomalías en la unión biliopancreática. Objetivo Analizar los datos clínicos, diagnóstico y tratamiento de una serie de pacientes diagnosticados de QC y realizar una actualización sobre el tema. Metodo Se revisaron retrospectivamente los diagnósticos de QC en 20 años en un hospital terciario. Casos clínicos Se identificaron 4 casos, con predominio del sexo femenino. Rango de edad 16 meses a 4 años. Los signos y síntomas fueron ictericia y coluria (100%), vómitos (75%), dolor abdominal y acolia (50%). Ninguno tuvo masa palpable. La ecografía abdominal orientó el diagnóstico que se confirmó con colangio-resonancia magnética (colangio-RM). Se clasificaron como QC tipo I tres de los casos y uno como tipo IVa. El tratamiento fue quirúrgico, ningún paciente presentó complicaciones hasta la fecha. Conclusiones Los quistes de las vías biliares son de baja prevalencia. El tratamiento de elección es quirúrgico, requiriendo seguimiento estrecho, dado el riesgo de colangiocarcinoma.

Introduction Cysts of the bile duct or choledochal cysts are rare diseases in our area. The aetiology is unknown, with the most accepted hypothesis being a pancreatobiliary maljunction anomaly. Objective To analyse the clinical data, diagnosis and treatment of a number of patients with choledochal cyst, as well as presenting an update on this condition. Method A retrospective descriptive study was performed on paediatric patients diagnosed with choledochal cyst in the last 20 years in a tertiary hospital. Case reports A total of 4 choledochal cyst cases in childhood, predominantly female, are pre- sented. The most frequent reason for consultation was vomiting, and presenting with jaundice and choluria in all cases. Patients with choledochal cyst were classified as type I in 3 cases, and one case of type IVa. In all cases surgical treatment was performed; any patient had complications to date. Conclusions Cysts of the bile ducts have a low prevalence. The treatment of choice is surgical, requiring close monitoring due to the risk of cholangiocarcinoma.

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.

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. .

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)

Avaliação por imagem das lesões císticas congênitas das vias biliares / Imaging evaluation of congenital cystic lesions of the biliary tract

Utilizando a classificação dos cistos congênitos de colédoco, imagens de vários tipos de cistos foram selecionadas para compor um ensaio iconográfico sobre esta doença pouco frequente na prática clínica, mas que faz parte do diagnóstico diferencial das lesões mais comuns da via biliar. Os cistos de colédoco possuem clínica inespecífica e baixa prevalência. O diagnóstico correto por meio dos exames de imagem é uma ferramenta importante para evitar as complicações e auxiliar na conduta e tratamento definitivo.

Based on the classification of congenital choledochal cysts, images of different cyst types were selected to compose a pictorial essay about this disease, which is not frequently observed in the medical practice, but is included in the differential diagnosis of most common lesions of the biliary tract. Choledochal cysts present nonspecific symptoms and low prevalence. The correct diagnosis based on imaging findings is mandatory to avoid complications, and to aid in the approach and definite treatment.

Disección mucosal endoscópica de cáncer gástrico en paciente con poliposis colonica / Endoscopic mucosal dissection of gastric cancer in patient with colonic polyposis

La disección mucosal endoscópica (DME), surge a partir de la resección mucosal endoscópica (RME), permitiendo resecar lesiones malignas en estadio precoz, logrando tasas de curación de hasta el 99%, disminuyendo la morbi-mortalidad de la cirugía tradicional. Es importante la obtención de la pieza de forma completa, ya que de esta manera, el patólogo podrá informar los márgenes de la lesión en extensión y profundidad. Caso clínico: Se trata de paciente masculino de 40 años de edad, valorado por nuestro servicio (agosto 2009) por clínica de 4 meses de evolución, dado por cambios en el patrón evacuatorio (evacuaciones diarreicas) y rectorragia. Consulta a especialista, el cual realiza colonoscopia con hallazgo de tumor en recto medio, con biopsia positiva para Adenocarcinoma, por lo que refieren a nuestro centro. Antecedentes personales y familiares: no contributorios. Se realizó gastroscopia la cual reporta: Lesión en cara anterior de antro, elevada, fondo ulcerado, deprimido, amputación de pliegues; se concluye: Ca. Gástrico Precoz IIc. Ultrasonido Endoscópico: Engrosamiento de la mucosa y muscular de la mucosa en antro. Cara posterior. Biopsia: Sin atipias. Colonoscopia: Tu recto bajo. Poliposis colonica. Ultrasonido endoscópico transrectal: Tu. Recto bajo uT2N1. Biopsia: Adenocarcinoma Moderadamente diferenciado. En vista de las características de la lesión gástrica, se decide realizar disección mucosal endoscópica, cuya biopsia que reporta: Carcinoma intramucoso precoz, bordes de resección libres de lesión. Paciente recibe tratamiento oncológico neo-adyuvante y posteriormente se realiza ileoprocto anastomosis. Evolución actual satisfactoria. Discusión: La apariencia endoscópica de las lesiones juega un papel importante en la conducta a seguir por el médico y es clave al momento de plantearse la realización de DME, ya que es un procedimiento que conlleva riesgos y tiene indicaciones especificas, para obtener así los resultados planteados...

Endoscopic mucosal dissection (DME), arises from endoscopic mucosal resection (EMR), allowing resection malignant lesions in early stage, achieving cure rates of up to 99%, decreasing morbidity and mortality of traditional surgery. It is important to obtain the piece completely, as this way, the pathologist may report the margins of the lesion extent and depth. Case report: This male patient aged 40 years, valued for our service (August 2009) by clinical evolution of 4 months, changes in the pattern of voiding (bowel diarrhea) and bleeding rectal. Consulting specialist, performed colonoscopy finding means is rectal tumor, with positive biopsy for adenocarcinoma, so refer to our center. Personal and family history: no contributory. Gastroscopy was performed which reported: Injury anterior antrum, high background ulcerated, depressed, amputation of folds, it is concluded: IIc early gastric Ca. Endoscopic Ultrasound: Thickening of the mucosa and muscle of the antral mucosa. Posterior. Biopsy: No atypia. Colonoscopy: tu. lower rectum. Colonic polyposis. Transrectal endoscopic ultrasound: Tu. UT2N1 lower rectum. Biopsy: moderately differentiated adenocarcinoma. Given the characteristics of gastric injury is endoscopic submucosal dissection dedide perform, with biopsy reports: early intramucosal carcinoma, resection margins free of injury. patient receives Neoadjuvant cancer treatment and subsequently coloproctoanastomosis. Current developments satisfactory. Discussion: The endoscopic appearance of lesions plays an important role in the conduct to be followed by the doctor and is key to consider when conducting DME because it is a procedure that carries risks and has specific indications for and results obertener raised...

Dilema en el diagnóstico de quiste de colédoco: reporte de un caso / Dilemma in the diagnosis of choledochal cyst: report of a case

El quiste de colédoco es una malformación congénita de las vías biliares poco común, que suele presentarse en la infancia. La triada clásica de ictericia, masa y dolor abdominal no siempre esta presente. Caso clínico: escolar femenina de 9 años, con Pancreatitis Aguda de evolución tórpida, ecosonograma abdominal inicial sin alteraciones, y diagnóstico no concluyente de Colecistitis crónica o Colangitis alitiásica por tomografía abdominal y colangioresonancia, respectivamente. Referida para colangiopancreatografía retrograda endoscópica, estudio que fue omitido por ecosonograma abdominal control sugestivo de Quiste de colédoco. Se solicita nueva colangioresonancia, que no visualiza quiste y reporta dilatación de hepatocoledoco y colédoco terminal de aspecto afinado. Se mantiene conducta expectante. La paciente permanece asintomática, con reevaluación 3 meses después. El ecosonograma abdominal revelo: dilatación fusiforme del colédoco en toda su extensión, hallazgo compatible con quiste de colédoco tipo I. Se realiza resección del quiste, con buena evolución clínica. Se concluye que el uso de estudios por imágenes no invasivos son de utilidad en la evaluación hepatobiliar en niños, y el ultrasonido abdominal continua como método de elección para esta patología.

Choledochal cyst is a congenital malformation of the bile ducts rare, usually seen in childhood. The classic triad of jaundice, abdominal mass and pain is not always present. Case report: A school girl of nine years, with Acute Pancreatitis torpid, unaltered initial abdominal ultrasound, and no conclusive diagnosis of chronic cholecystitis or acalculous cholangitis abdominal and from magnetic resonance tomography, respectively. Referred for endoscopic retrograde cholangiopancreatography, a study that was omitted from control abdominal ultrasound suggestive of choledochal cyst. New requested from magnetic resonance, which displayed no reports cyst and bile duct dilation and terminal hepatocoledoco related aspects. Expectant management is maintained. The patient remains asymptomatic, with reassessment 3 months later. The abdominal ultrasound revealed: fusiform dilatation of the bile duct in its entirety, a finding compatible with type I choledochal cyst Resection was performed, the cyst, cholecystectomy and reconstruction of the bile duct through a hepato-jejunostomy Roux en Y no complications. Liver biopsy reported normal tissue. We conclude that the use of noninvasive imaging tests such as ultrasound is the method of choice due to its sensitivity to determine the presence of cyst and to demonstrate the anatomy of the pancreaticduct.

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.

[Report of 23 cases of choledochal cyst from two hospitals, Mofid and Taleghani in Tehran [1986-2004]]

Choledochal cyst is a congenital disorder of biliary tract. It is rare in western but more common in Asian countries. Most studies have been case reports but in this study for assessment of surgical method of treatment and late complications, patients were followed for 18 years. This retrospective study was done based on 23 patients profiles with diagnosis of choledochal cyst who have been admitted in two hospitals, Mofid and Taleghani in Tehran during 1986-2004. Diagnosis was based on imaging and intraoperative findings variables for assessment were age, sex, cyst type, clinical picture and surgical method of treatment. 70% of cases were female. Type I [sacular and fusiform, 74%] was the most common type, 70% of cases were between 1-5 years old, sonography was the most common imaging technic, that used for diagnosis [100% of cases], Icter was the most common clinical picture found in 70% of cases, surgical procedures and method in 70% of cases was standard and 30% of patients underwent palliative procedures. Although this study shows similarity in age, sex, common type, diagnostic and treatment methods with another centers and countries, but it shows necessity of surgeon's encouragement to use standard surgical method for decreasing late complications of disease

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.

[Case report Surgical treatment of choledochal cyst]

Choledochal cysts are congenital cystic dilatation of the extrahepatic and / or intrahepatic billiary tree. Treatment of choice is surgery which varies according to the type of choledochal cysts. The present case was a woman with abdominal pain, jaundice, nausea, vomiting and pruritus, underwent surgery at Shahid Rajai Hospital in Qazvin. Intraoperative finding was choledochal cyst [Type V]. The extrahepatic cysts were excised and Roux- en-y hepaticojejunostomy, end to side jejunojejunostomy and cholecystectomy was performed which was accompanying with a good response

Diagnóstico prenatal de quiste de colédoco: presentación de un caso / Prenatal diagnosis of the choledochal cyst: case report

Clasificación de los quistes, etiología, diagnóstico y tratamiento. En el caso que se presenta se diagnosticó un quiste Tipo Ic en la semana 29 de gestación, y se confirmó el diagnóstico luego de una cesárea de urgencia en la semana 40.

Fetal Choledochal Cyst Diagnosed at 22 Weeks of Gestation by Three-Dimensional Ultrasonography: A Case Report

Choledochal cyst is a cystic or fusiform dilatation of the extra- or intrahepatic bile duct that has rarely been reported in prenatal cases. Here we report a fetus with choledochal cyst diagnosed prenatally by three-dimensional (3-D) ultrasonography at 22 weeks of gestation. We demonstrated an image of choledochal cyst by using a new ultrasound technique, a 3-D multislice view. After close intrauterine followup, surgery was successfully performed and postoperative course was uneventful.

Choledochal cysts in infancy and childhood.

OBJECTIVE: To study the clinical presentation, management and results of treatment of 41 consecutive cases of choledochal cyst (CC) managed from 1999 to 2006. METHODS: The age of the patients ranged from 20 days to 11 years. Eleven cases were 1 year or less in age (infantile group) and 30 patients were more than 1 year old (classical pediatric group). Children less than 1 year old presented with jaundice (72%), hepatomegaly (54%) and clay-colored stools (63%); whereas those above one year in age presented with pain (83.3%) and jaundice (47.6%). Three cases had biliary perforation with localized or generalized biliary peritonitis. Ultrasonography (US) diagnosed/suggested CC in all 41 patients, however, contrast-enhanced computed tomographic (CECT) scan (n=12), radionuclide DISIDA scan (n=5) and magnetic resonance cholangiopancreatogram (MRCP) (n=3) also contributed to the diagnosis. 19 patients, including 4 from the 'infantile' group, had significant dilatation of intrahepatic biliary ducts. RESULTS: Nine patients from the 'infantile' group had cystic type I CC (type Ic), while 2 patients had fusiform dilatation of the common bile duct (type If disease). In contrast, 21 of the older patients had type If disease while 9 had type Ic disease. Operative management included primary complete excision of the cyst with a Roux-en-Y hepaticodochojejunostomy (HDJ) (n=32), Lilly's modification of submucosal resection with a HDJ (n=8) and cyst excision with a hepaticodochoduodenostomy (HDD) (n=1). All 3 patients with biliary perforation had primary excision of the CC with a HDJ. Unusual operative findings included accessory hepatic ducts (n=2), anteriorly placed common or right hepatic arteries (n=3) and partially or completely blocked proximal extrahepatic bile ducts (n=3). There were 2 postoperative deaths, one had cirrhotic liver disease with portal hypertension, the other had deep icterus with poor general condition. Both patients succumbed to overwhelming sepsis. One patient required reexploration and refashioning of the HDJ for biliary leak. Short-term follow-up was satisfactory in all 39 patients. CONCLUSION: Infants with CC constitute a distinct group with regard to clinical presentation and the pathological should be kept in mind while evaluating neonates and infants with cholestatic jaundice and older children with recurrent abdominal pain. Primary excision of the cyst with a HDJ provides satisfactory results in the management of the vast majority of cases of CC including those in infants, massive CC and in the presence of biliary perforation and peritonitis.

Embryonal rhabdomyosarcoma of the biliary tree mimicking a choledochal cyst.

Embryonal rhabdomyosarcoma (ERMS) of biliary tree is a rare type of mesenchymal neoplasm diagnosed at surgery or by preoperative liver biopsy. We present a one year eight months old child who mimicked a choledochal cyst and was eventually treated with surgery, chemotherapy with IRS IV protocol and adjuvant postoperative radiotherapy to surgical bed with 6 MV photons to a dose of 5040 cGy in 28 fractions.