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.

MRI Findings of Intrinsic and Extrinsic Duodenal Abnormalities and Variations

This pictorial review aims to illustrate the magnetic resonance imaging (MRI) findings and presentation patterns of anatomical variations and various benign and malignant pathologies of the duodenum, including sphincter contraction, major papilla variation, prominent papilla, diverticulum, annular pancreas, duplication cysts, choledochocele, duodenal wall thickening secondary to acute pancreatitis, postbulbar stenosis, celiac disease, fistula, choledochoduodenostomy, external compression, polyps, Peutz-Jeghers syndrome, ampullary carcinoma and adenocarcinoma. MRI is a useful imaging tool for demonstrating duodenal pathology and its anatomic relationships with adjacent organs, which is critical for establishing correct diagnosis and planning appropriate treatment, especially for surgery.

Is Preoperative Subclassification of Type I Choledochal Cyst Necessary?

OBJECTIVE: The aim of this study was to evaluate the frequency of postoperative biliary stricture and its risk factors in patients undergoing surgery for type I choledochal cyst. MATERIALS AND METHODS: A total of 35 patients with type I choledochal cyst underwent laparoscopic cyst excision and Roux-en-Y hepaticojejunostomy between August 2004 and August 2011. Their medical records and radiologic images (including endoscopic retrograde cholangiopancreatography, magnetic resonance cholangiopancreatography, pancreatobiliary computed tomography, or ultrasound) were retrospectively analyzed to evaluate the frequency of postoperative biliary stricture and its risk factors. RESULTS: Postoperative biliary stricture was found in 10 (28.6%) of 35 patients. It developed more frequently in patients with type Ia choledochal cyst (53.8%, 7 of 13 patients) than in patients with type Ic choledochal cyst (13.6%, 3 of 22 patients), which was statistically significant (p = 0.011). There were no significant associations between other factors and postoperative biliary stricture. CONCLUSION: Type Ia is a risk factor of postoperative anastomotic stricture. Therefore, preoperative radiologic subclassification of type Ia and Ic may be useful in predicting postoperative outcomes of choledochal cysts.

Is Preoperative Subclassification of Type I Choledochal Cyst Necessary?

OBJECTIVE: The aim of this study was to evaluate the frequency of postoperative biliary stricture and its risk factors in patients undergoing surgery for type I choledochal cyst. MATERIALS AND METHODS: A total of 35 patients with type I choledochal cyst underwent laparoscopic cyst excision and Roux-en-Y hepaticojejunostomy between August 2004 and August 2011. Their medical records and radiologic images (including endoscopic retrograde cholangiopancreatography, magnetic resonance cholangiopancreatography, pancreatobiliary computed tomography, or ultrasound) were retrospectively analyzed to evaluate the frequency of postoperative biliary stricture and its risk factors. RESULTS: Postoperative biliary stricture was found in 10 (28.6%) of 35 patients. It developed more frequently in patients with type Ia choledochal cyst (53.8%, 7 of 13 patients) than in patients with type Ic choledochal cyst (13.6%, 3 of 22 patients), which was statistically significant (p = 0.011). There were no significant associations between other factors and postoperative biliary stricture. CONCLUSION: Type Ia is a risk factor of postoperative anastomotic stricture. Therefore, preoperative radiologic subclassification of type Ia and Ic may be useful in predicting postoperative outcomes of choledochal cysts.

Choledochal cyst.

Choledochal cyst, a congenital anomaly, is a premalignant condition. Progressive damage to the bile ducts and to the liver parenchyma may also have association with the condition. Subsequent risk of developing cholangiocarcinoma may be lessen by early diagnosis and prompt definitive surgical treatment, that may even permanently correct the condition. A young lady was admitted into Mymensingh Medical College Hospital and was presented with pain in the upper abdomen associated with intermittent vomiting for several months. Her per abdominal examination revealed an ill defined mass in the epigastrium and right hypogastrium with diffuse tenderness. Patients may also present with attacks of jaundice of obstructive type, but this was absent in this particular case. Diagnosis of choledochal cyst was confirmed by the ultrasound. On exploration, the cyst was excised and Hepatico-jejunostomy and jejuno-jejunostomy was done by Roux-en-Y method. Her post operative period was uneventful and followed up till to date. Purpose of this presentation is to emphasize early diagnosis and definitive treatment of this developmental anomaly that may predispose to malignancy.

The liver in choledochal cyst.

BACKGROUND/PURPOSE: Hepatic changes due to choledochal cyst have not been given due emphasis in the published literature. In this study the gross and microscopic appearance of the liver in patients with choledochal cyst have been correlated with clinical features, liver function tests and postoperative complications. METHODS: A retrospective evaluation of patients treated for choledochal cysts between January 1989-December 1998 was undertaken. The case records were reviewed and liver biopsies taken at surgery were analysed. RESULTS: The case records of 22 patients were reviewed (16 girls, 6 boys; mean age 4.6 years, range 1 month-10 years). The presenting features included jaundice (59%; 85.7% in < 1 year), abdominal pain (59%; 86.7% in > 1 years) and fever in 40.9% cases. A palpable abdominal mass and hepatomegaly was present in 32% cases. A type IV cyst was present in 1 case; all others had type I cysts (14 cystic and 7 fusiform). The liver looked grossly 'normal' in 16 and 'cirrhotic' in 6 cases. Liver biopsies were available for review from 5 of the 'cirrhotic' and 7 of the 'normal' looking livers. All the liver biopsies showed varying degrees of bile duct proliferation, cholestasis, parenchymal damage, inflammatory cell infiltration and pericentral fibrosis. Histological features of cirrhosis were evident in 6 cases (4 'cirrhotic' and 2 'normal' looking livers), 4 of these cases were infants. Liver function tests were deranged in 5 cases with histological features of cirrhosis and hepato-biliary scintigraphy showed obstructive features in 3 of these cases. There was an increased risk of postoperative complications in the cases with histological features of cirrhosis, 2 died from hepatic insufficiency and one each had transient ascitic and biliary leak. CONCLUSION: Liver histology showed significant changes in all the cases of choledochal cyst in whom it was studied; even normal looking livers showed evidence of significant changes. Presence of cirrhosis, more common in infants, correlated with jaundice, deranged liver function tests, obstructive features on hepatobiliary scintigraphy and a greater risk of postoperative complications.

Colangiopancreatografia por resonancia magnetica (CPRM) en el estudio de la patologia biliar / MR cholangiopancrea tography in Biliar pathology study's

En el presente estudio se pretende evaluar nuestra experiencia con una técnica de colangiopacreatografía por Resonancia Magnética (CPRM), de tipo "disparo único" Half- Fourier FastAdvanced Spin Echo (FASE), 20 y 3D en el estudio de diferentes patologías de la vía biliar y del conducto pancreático. Se incluyeron 73 pacientes con CPRM, con diversas patologías, cuyo diagnóstico se hubiera confirmado por otras modalidades de imágenes, por cirugía o por estudios histopatológicos. La sensibilidad de la CPRM fue del 92.86 por ciento , la especificidad fue del 82.2 por ciento , el valor predictivo (+) fue del 96.3 por ciento , el valor predictivo (-) fue del 78-9 por ciento , y la exactitud diagnóstica del 91.8 por ciento . La razón de probabilidad (+) (positive likelihood ratio) de la CPRM fue de 7.93, y la razón de probabilidad negativa (negativo likelihood ratio) fue de 0.008. Estos resultados permiten concluir que la CPRM con secuencias de pulso Half-Fourier FASE, es una excelente herramienta diagnóstica, no invasiva, para ser utilizada como método de tamizaje en la patología obstructiva biliar y del conducto pancreático

Choledochal cyst associated the with anomalous union of pancreaticobiliary duct (AUPBD) has a more grave clinical course than choledochal cyst alone

OBJECTIVE: Since choledochal cyst is frequently associated with the anomalous union of pancreaticobiliary duct (AUPBD), AUPBD has been regarded to be the etiologic factor of choledochal cyst. However, the clinical significance of AUPBD an patients with choledochal cyst has not been clearly defined. Therefore, to clarify the significance of AUPBD in choledochal cyst patients, we compared the clinical features of patients with choledochal cyst according to the presence or absence of AUPBD. METHODS: Among 52 cases which were diagnosed as choledochal cyst out of 5,037 ERCP referrals between August 1990 and December 1996, we selected 44 cases, in which the pancreaticobiliary junction was clearly visualized on cholangio-pancreaticography. These cases were divided into AUPBD-present group (n = 28) and AUPBD-absent group (n = 16). Clinical features were compared between the two groups. Furthermore, in AUPBD-present group, clinical data were also analyzed according to Kimura's classification of AUPBD. RESULTS: In our study, AUPBD was associated with choledochal cyst in 28 (64%) cases. AUPBD was found only in type I and IV according to Todani's classification of choledochal cyst. There were no significant differences between the AUPBD-present group and the AUPBD-absent group in the incidence of gallstone disease, while the incidence of acute inflammation was 93% (26/28) in the AUPBD-absent group (p < 0.01). Carcinoma developed only in the AUOBD-present group (9/28, 32%) (p < 0.05). Pancreatic disorders (i.e. pancreatic stone, pancreatitis or pancreatic cancer) occurred in 12 of 28 cases in the AUPBD-present group (43%), while only in 1 of 16 cases in the AUPBD-absent group (6%) (p < 0.05). CONCLUSION: AUPBD associated with choledochal cyst may have implications not only as a possible etiologic factor but also as an important factor that may affect the clinical course, surgical planning and prognosis. In cases with choledochal cyst, we should make an effort to evaluate the presence of AUPBD.

Cisto de colédoco / Choledochal cyst

O cisto de colédoco, apesar de sua raridade, é a anomalia de vias billares mais frequente depois da atresia de vias biliares. Sua incidência nos EUA é em torno de 1:30.000 nascido vivos, na Austrália em torno de 1:15.000, porém, a maior incidência mundial ocorre no Extremo Oriente, tendo o Japão o maior número de casos relatados. Vinte e cinco por cento dos casos são diagnosticados no primeiro ano de vida e entre 50 e 75 por cento dos casos até 10 anos de idade, sendo o sexo feminino o mais acometido (aproximadamente 80 por cento dos casos). O cisto de colédoco é responsável por 2 por cento das icterícias obstrutivas do lactente e pode estar associados a outras anomalias congênitas, mais frequentemente a doença policística do rim. Os autores relatam um caso de cisto de colédoco em uma criança de 7 anos de idade, do sexo feminino, admitida no serviço de cirurgia pediátrica do HULW-UFPB

Adult intrahepatic solitary choledochal cyst.

A 20-year-old woman presented with dull ache in the abdomen. Ultrasonography and CT scan showed a solitary liver cyst. Roux-en-Y cystojejunostomy was done since there was bile in the cyst. Histology of the cyst wall revealed it to be a choledochal cyst. Solitary intrahepatic choledochal cyst and presentation in adult life are rare.

Quiste gigante de colédoco en la infancia. Informe de un caso / Gigantic choledochal cyst in infancy. Case report

Se informa el caso de una paciente de seis años de edad, atendida en el Hospital General de Zona con Medicina Familiar Núm. 1 en Pachuca Hidalgo, por una gran tumoración abdominal en el cuadrante superior derecho. Despúes de diversos exámenes de gabinete, se diagnosticó quiste gigante de colédoco tipo I de la clasificación de Alonso Lej y Todani. Esta patología debe ser tratada mediante resección total del quiste para prevenir su malignización, y la reconstrucción con hepatoyeyunoanastomosis en y de Roux. No se presentaron complicaciones y la paciente evolucionó satisfactoriamente. Se analizan en la literatura mundial la etiología, fisiopatología y pronóstico. El diagnóstico se basó en la tomografía computarizada y en el estudio patológico

Dilataciones quísticas de la vía biliar / Cystic dilatation of bile ducts

Se presentan 4 casos de dilataciones quísticas de la vía biliar extrahepática y se hace una revisión del tema. Se concluye que el tratamiento es el quístico y que en los casos más frecuentes, los llamados quistes del colédoco, el tratamiento ideal es la resección completa del quiste y la derivación biliodigestiva. Las derivaciones internas son una mala solución por la alta incidencia de complicaciones que obligan a reoperaciones y porque no evitan el desarrollo de cánceres, complicación evolutiva frecuente de esta patología

Quiste de coledoco: reporte de un caso / Cholidochal cyst: report of case

Presentamos el caso de un paciente con diagnóstico de quiste del colédoco, el cual fue intervenido quirúrgicamente realizándosele una cistoyeyunoanastomosis. Posteriormente presentó crisis repetidas de ictericia y congalitis, motivo por el cual le fue practicada excisión del quiste y hepatoyeyunoanastomosis en "Y" de Roux. Se hace una revisión bibliográfica evidenciando frecuentes complicaciones en los casos en los cuales se realiza derivaciones, concluyéndose que el mejor procedimiento es la excisión del quiste

Cisto de colédoco em crianças / Choledochal cyst in children

Cisto de colédoco é uma anomalia rara em crianças. De 1983 a 1991 quatro pacientes (dois meninos e duas meninas), com idade s variando entre 15 meses e 11 anos, foram tratados no Serviço de Cirurgia Pediaétrica do nosso hospital. As manifestaçöes clínicas mais freqüentes foram icterícia, febre, dor e tumoraçäo abdominal palpável. Em todos os casos o diagnóstico foi firmado por ultra-sonografia abdominal e confirmado durante a cirurgia, que constou de uma completa ressecçäo intracapsular do cisto. Três cistos eram do tipo 1 e um do tipo 4. O quadro clínico, métodos de diagnóstico, classificaçäo, tratamento atual e resultados terapêuticos desta incomum anomalia säo discutidos