Excepcional variación anatómica del confluente biliar superior / Exceptional anatomical variation of the upper biliary confluence / Variação anatômica excepcional da confluência biliar superior

Las variaciones de la formación del confluente biliar superior y su frecuencia deben ser conocidas para asegurar óptimos resultados en la cirugía hepática y evitar lesiones quirúrgicas biliares.

Variante anatómica de la vía biliar durante la colecistectomía laparoscópica / Anatomical variant of the bile duct during laparoscopic cholecystectomy / Variante anatômica do ducto biliar durante colecistectomia laparoscópica

Se presenta la colangiografía intraoperatoria obtenida durante la colecistectomía laparoscópica de una paciente de 58 años con el diagnóstico de colecistitis aguda.

Tumor de Klatskin y metástasis cerebral como presentación inusual en cáncer de mama / Klatskin tumor and brain metastasis in a breast cancer patient: Case report

Introducción: El cáncer de mama es la principal causa de muerte en mujeres a nivel mundial, se los clasifica en base a la expresión del receptor de estrógenos, de progesterona y de ERBB2. El tumor de Klatskin es infrecuente y se origina en la bifurcación del conducto biliar extrahepático. Presentación del caso: Paciente femenina de 60 años con diagnóstico de cáncer de mama estadío IIIB, que presenta hemiplejía derecha secundaria a lesión cerebral metastásica, inicialmente catalogada como neurocisticercosis. Después de 1 año 7 meses desarrolla ictericia colestásica cuya evaluación clínica permite evidenciar tumor de Klatskin mediante colangioresonancia. Discusión: El presente caso muestra una paciente con múltiples metástasis secundarias a cáncer de mama. Incluyendo, de igual manera, un tumor de Klatskin, el cual fue descubierto posterior a un síndrome colestásico. Son pocos los casos de la literatura que registran procesos metastásicos hacia conductos biliares con origen en un carcinoma ductal infiltrante. Conclusiones: Pocos casos en la literatura registran procesos metastásicos hacia las vías biliares con origen en infiltración ductal carcinoma; sin embargo, es fundamental informar para la evidencia clínica y el estudio de la literatura.

Introduction: Breast cancer is the principal cause of death in women worldwide, classi ed based on the expression of estrogen receptor, progesterone, and ERBB2. Klatskin tumor is rare and originates in the bifurcation of the extrahepatic bile duct. Case presentation: A 60 years old female patient diagnosed with stage IIIB breast cancer presents right hemiplegia secondary to metastatic brain injury, initially classi ed as neurocysticercosis. After one year and seven months, she developed cholestatic jaundice, whose clinical evaluation allows Klatskin tumor evidence through cholangioresonance. Discussion: The present case is about a patient with multiple metastases secondary to breast cancer. Including, in the same way, a Klatskin tumor, which was discovered after a cholestatic syndrome. Conclusions: Few cases in the literature register metastatic processes towards bile ducts originating from in ltrating ductal carcinoma; however, it is essential to report for clinical evidence and study of the literature.

Gene expression profiling reveals upregulated FUT1 and MYBPC1 in children with pancreaticobiliary maljunction

Pancreaticobiliary maljunction (PBM) is associated with high risk of epithelial atypical growth and malignant transformation of the bile duct or gallbladder. However, overall changes in genetic expression have not been examined in children with PBM. Genome-wide expression was analyzed using peripheral blood samples from 10 children with PBM and 15 pediatric controls. Differentially expressed genes (DEGs) were identified using microarray. Bioinformatics analysis was conducted using Gene Ontology and KEGG analyses. The top 5 in the up-regulated genes in PBM were verified with qRT-PCR. Receiver operator characteristic curve analysis was conducted to evaluate the predictive accuracy of selected genes for PBM. The microarray experiments identified a total of 876 DEGs in PBM, among which 530 were up-regulated and the remaining 346 were down-regulated. Verification of the top 5 up-regulated genes (TYMS, MYBPC1, FUT1, XAGE2, and GREB1L) by qRT-PCR confirmed the up-regulation of MYBPC1 and FUT1. Receiver operating characteristic curve analysis suggested that FUT1 and MYBPC1 up-regulation could be used to predict PBM, with the area under the curve of 0.873 (95%CI=0.735−1.000) and 0.960 (95%CI=0.891−1.000), respectively. FUT1 and MYBPC1 were up-regulated in children with PBM, and could be used as potential biomarkers for PBM.

Casuística. Vesícula biliar siniestra (sinistraposición): Caso clínico / Left-sided Gallbladder (Sinistraposition of the gallbladder)

A rare case of left-sided gallbladder (sinistraposition) is reported with review of the literature. Left-sided gallbladder is very unusual, with a frequency of 0.3% of the cases, being generally associated to situs inversus. The aim of this invesigation was to establish the association between left-sided gallbladder and right-sided round ligaments. Left-sided gallbladder is a rare anomaly and has been classified into two situations: 1) gallbladder migration to the left side, and 2) development of a second gallbladder with atrophy of the original one. Left-sided gallbladder were reported to be associated with right-sided round ligaments.

Recurrent acute pancreatitis caused by pancreatic biliary maljunction due to long common channel / Pancreatitis aguda recurrente causada por deficiente unión pancreático-biliar debido a un colédoco largo

The pancreatic biliary maljunction is a rare anomaly that affects mainly females, defined as an anatomical maljunction of the pancreatic duct and the biliary duct confluence, and may be a rare cause of recurrent acute pancreatitis. In order to early diagnosis and prompt treatment, ERCP has an important role in it

La Malformación de la unión biliopancreática es una afección rara y ocurre más en mujeres jóvenes. Es una causa de pancreatitis aguda de causa no conocida. CPRE es una herramienta eficiente para el diagnóstico y también para ser de la terapéutica

Variedades anatómicas del árbol biliar: implicancia quirúrgica / Anatomy variations of biliary tree: surgical implicance

La anatomía de las vías biliares presenta frecuentes variaciones. La anatomía "normal" se describe en aproximadamente la mitad de los casos. Las variedades de las vías biliares deben ser conocidas por los médicos especialistas en diagnóstico por imágenes y cirujanos hepatobiliares, a fin de optimizar su desempeño en sus respectivas áreas. Con ese fin fueron estudiadas en forma prospectiva 690 colangiografías (538 intra-operatorias; 90 resonancias; y 62 trans-catéteres biliares). Se utilizó la clasificación de Blumgart y los resultados fueron: tipo A: 284 (41,16 por ciento); B: 178 (25,80 por ciento). C1: 110 (15,94 por ciento); C2: 38 (5,51 por ciento).; D1: 8 (1,16 por ciento); D2: 23 (3,33 por ciento); E1: 18 (2,61 por ciento); E2: 27 (3,91 por ciento); F: 4 (0,58 por ciento). Como conclusiones, es importante señalar que: a) la colangiografía intra-operatoria debe ser sistemática, por un lado, para descartar litiasis coledociana; y por el otro, para detectar variaciones anatómicas y predecirlas antes de las cirugías resectivas; y b) la colangiorresonancia es ideal como estudio preoperatorio en posibles donantes vivos de órganos.

The anatomy of the biliary tract have frequent variations. Normal anatomy is described in about half of cases. The varieties of the bile ducts should be known by physicians and hepatobiliary surgeons specializing in diagnostic imaging, in order to optimize performance in their respective areas. To this end 690 cholangiographies (538 intra-operative, 90 resonances, and 62 trans-catheter bile) were prospectively studied. We used the classification of Blumgart and the results were as follows: type A: 284 (41.16 percent), B: 178 (25.80 percent). C1: 110 (15.94 percent), C2: 38 (5.51 percent)., D1: 8 (1.16 percent), D2: 23 (3.33 percent), E1: 18 (2.61 percent) , E2: 27 (3.91 percent), F: 4 (0.58 percent). As conclusion, it is important to note that: a) intra-operative cholangiography should be systematic, on the one hand, to exclude cholelithiasis and, on the other hand, to detect and predict anatomical changes before resective surgery and b) cholangioresonance is an ideal preoperative study in potential living donors of organs.

Incremento del factor de crecimiento transformante B en las lesiones de vías biliares luego de la colecistectomía / Hepatic and systemic transforming growth factor B expression in post-cholecystectomy bile duct injury

Introducción: La lesión de vías biliares es un problema clínico importante, se manifiesta por activación de moléculas inflamatorias y fibrogénicas. Objetivo: Evaluar la expresión hepática y sistémica del factor de crecimiento transformante beta (TGF-ß) en la lesión de vías biliares luego de la colecistectomía. Pacientes y métodos: Grupo 1 (control), 3 sujetos sanos; grupo 2, 37 pacientes con lesión de vías biliares poscolecistectomía. Al grupo 2 se le realizó reconstrucción biliodigestiva a 8 semanas de la lesión. Se midió en suero la expresión de TGF-ß por ELISA. La biopsia hepática se sometió a análisis histopatológico e inmuhistoquímica con el anticuerpo anti-TGF-ß, se cuantificó la expresión mediante soporte digital AxioVision 4.6.3. Resultados: El promedio de los niveles séricos de TGF-ß del grupo 1 fue 904.49 ± 227.24 pg/ml contra el grupo 2 con 44 365.71 ± 20 260.61 pg/ml, 48 veces más expresión (p < 0.0001, t de Student). La actividad inflamatoria hepática fue diversa en el grupo 2, mostró grado 4 en 4 (11%); de 3, 15 (40%); de 2, 18 (49%). En el grupo 1 la actividad inflamatoria fue de grado 0. La expresión hepática del TGF-ß en el grupo 1, es de un promedio de 537.15 ± 330.96 μm2; en el grupo 2, 1 768.09 ± 1 024.50 μm2, 2.3 más expresión (p = 0.0473, t de Student). Conclusión: La expresión hepática y sistémica de TGF-ß se encontró aumentada en los pacientes con lesión de vías biliares poscolecistectomía.

Correlação interobservador das alterações morfológicas das vias biliares em pacientes com esquistossomose mansoni pela colangiorressonância magnética / Evaluation of morphological changes of the biliary tree by magnetic resonance cholangiography in patients with schistosomiasis mansoni: interobserver agreement

OBJETIVO: Descrever as alterações das vias biliares pela colangiografia por ressonância magnética (CPRM) na esquistossomose hepatoesplênica e avaliar a concordância interobservador da CPRM na detecção de colangiopatia esquistossomótica. MATERIAIS E MÉTODOS: Estudo prospectivo e transversal em 24 pacientes com a forma hepatoesplênica da esquistossomose mansoni e em 6 pacientes sadios, como grupo controle, com avaliação da via biliar pela CPRM. As alterações da via biliar consideradas foram: distorção, afilamento, estenose, dilatação e irregularidade. Foi calculada a concordância interobsevador para alteração da via biliar com o teste de McNemar e o índice kappa (κ). RESULTADOS: A concordância interobservador na caracterização de distorção e afilamento da via biliar foi quase perfeita (κ = 0,867; intervalo de confiança [IC] 95 por cento [0,512-1,0] e κ = 0,865; IC 95 por cento [0,51-1,0], respectivamente). A concordância foi substancial para a estenose (κ = 0,78; IC 95 por cento [0,424-1,0]), moderada para dilatação (κ = 0,595; IC 95 por cento [0,247-0,942]) e regular para afilamento (κ = 0,229; IC 95 por cento [0,095-0,552]). CONCLUSÃO: As alterações observadas nas vias biliares foram, em ordem decrescente de ocorrência: distorção, afilamento, estenose, dilatação e irregularidade. A concordância interobservador para sinais de colangiopatia esquistossomótica foi quase perfeita para distorção e afilamento e substancial para estenose.

OBJECTIVE: To describe changes of the biliary tree demonstrated by magnetic resonance cholangiography (MRC) in patients with the hepatosplenic presentation of schistosomiasis mansoni, and evaluating the interobserver agreement in the detection of schistosomal cholangiopathy. MATERIALS AND METHODS: Prospective, cross-sectional study involving 24 patients with hepatosplenic schistosomiasis and 6 healthy patients (control group) submitted to biliary tree evaluation by MRC. The following changes of the biliary tree were considered: distortion, thinning, stenosis, dilation and irregularity. The interobserver agreement in the detection of biliary tree changes was calculated with the McNemar's test and the kappa index of agreement (κ). RESULTS: The interobserver agreement in the detection of distortion and thinning of the biliary tree was almost perfect (κ = 0.867; confidence interval [CI] 95 percent [0.512-1.0] and κ = 0.865; CI 95 percent [0.51-1.0], respectively). There was a substantial agreement for stenosis (κ = 0.78; CI 95 percent [0.424-1.0]), moderate agreement for dilation (κ = 0.595; CI 95 percent [0.247-0.942]) and mild agreement for thinning (κ = 0.229; CI 95 percent [0.095-0.552]). CONCLUSION: In a decreasing order of frequency, the changes of the biliary tree were observed: distortion, thinning, stenosis, dilation and irregularity. The interobserver agreement for signs of schistosomal cholangiopathy was almost perfect for distortion and thinning, and substantial for stenosis.

Cholelithiasis and biliary sludge in Down’s syndrome patients

CONTEXT AND OBJECTIVE: Although studies have demonstrated increased frequency of gallbladder abnormalities among Down’s syndrome (DS) patients in some countries, there is only one paper on this subject in the Brazilian literature. The aim of this study was to demonstrate the prevalence, clinical characteristics and evolution of lithiasis and biliary sludge among DS patients in a maternity and children’s hospital in Rio de Janeiro. DESIGN AND SETTING: This was a cross-sectional study followed by a retrospective cohort study on all individuals with an ultrasound diagnosis of gallbladder abnormalities. METHODS: 547 DS patients (53.2 percent male, 46.8 percent female) attending the Instituto Fernandes Figueira in 2001 underwent abdominal ultrasound examination at ages of between one day and three years (mean: five months). Clinical and ultrasound data were analyzed. RESULTS: In 50 patients (9.1 percent), the ultrasound demonstrated gallbladder abnormalities (6.9 percent lithiasis and 2.1 percent biliary sludge). Spontaneous resolution was observed in 66.7 percent of the patients with biliary sludge and 28.9 percent with lithiasis. Cholecystectomy was carried out on 26.3 percent of the patients with gallstones. CONCLUSION: The results from this study and comparison with the literature suggest that DS patients are at risk of developing lithiasis and biliary sludge and should be monitored throughout the neonatal period, even if there are no known risk factors for gallstone formation. Most frequently, these gallbladder abnormalities occur without symptoms and spontaneously resolve in most non-symptomatic patients. DS patients should be monitored with serial abdominal ultrasound, and cholecystectomy is indicated for symptomatic cases or when cholecystitis is present.

OBJETIVO: Demonstrar prevalência, características clínicas e evolução de litíase e lama biliar em pacientes com síndrome de Down (SD) num hospital materno-infantil no Rio de Janeiro. Apesar de estudos revelarem aumento das anormalidades biliares em pacientes com SD em alguns países, no Brasil existe apenas um trabalho abordando o tema. TIPO DE ESTUDO E LOCAL: Estudo transversal seguido por um estudo de coorte retrospectivo de todos os indivíduos com diagnóstico ultra-sonográfico de anormalidades da vesícula biliar. MÉTODOS: Foram selecionados 547 pacientes com SD (53,2 por cento sexo masculino, 46,8 por cento feminino) atendidos no Instituto Fernandes Figueira, Fundação Instituto Oswaldo Cruz (IFF-Fiocruz) em 2001. Todos os pacientes incluídos neste estudo foram submetidos a ultra-sonografia abdominal quando tinham idades variando entre um dia e três anos (mediana cinco meses). Dados clínicos e ultra-sonográficos foram avaliados. RESULTADOS: Em 50 (9,1 por cento) crianças, a ultra-sonografia demonstrou alteração da vesícula biliar (6,9 por cento litíase e 2,2 por cento lama biliar). Houve resolução espontânea em 66,7 por cento dos pacientes com lama biliar e em 28,9 por cento dos pacientes com litíase. A colecistectomia foi realizada em 26,3 por cento dos pacientes com cálculo biliar. CONCLUSÃO: Resultados deste estudo e a comparação com a literatura sugerem que a SD deve ser considerada como fator de risco para desenvolvimento de litíase e lama biliar em crianças, sobretudo no período neonatal, sem que existam outros fatores predisponentes para formação de cálculo biliar. Na maioria das vezes, estas alterações são assintomáticas e, freqüentemente, têm evolução favorável, permanecendo desta forma ou tendo resolução espontânea. Pacientes devem ser acompanhados com ultra-sonografias seriadas. Tratamento cirúrgico está indicado para casos sintomáticos ou na presença de colecistite.

Variantes anatômicas e anomalias congênitas das vias biliares e pancreáticas: análise através da colangiopancreatografia por ressonância magnética / Anatomic variants and congenital abnormalities of the biliary tree seen on magnetic resonance cholangiopancreatography

Com o objetivo de ilustrar, de maneira sistematizada, as principais variantes anatômicas e anomalias congênitas observadas em exames de colangiopancreatografia por ressonância magnética (CPRM), realizamos estudo retrospectivo em exames de CPRM com as mais diversas indicações, procurando por variantes anatômicas e anomalias congênitas das vias biliares e pancraáticas. Todos os exames foram realizados em equipamento de alto campo (1,0 T ou 1,5 T), obtendo-se imagens no plano axial ponderadas em T1 (FFE) e T2 (TSE), e no plano coronal, com técnica de CPRM. Apresentamos variantes anatômicas relacionadas à posição da vesícula biliar, à distribuição e posição das vias biliares intra-hepáticas, do ducto cístico, ducto colédoco e ductos pancreáticos. Mostramos também exemplos dos principais tipos de dilatação cística congênita das vias biliares. Reconhecer as principais variantes anatômicas e anomalias congênitas das vias biliares e pancreáticas permite otimizar a eficácia diagnóstica da CPRM.

In order to illustrate the role of magnetic resonance cholangiopancreatography (MRCP) in the diagnosis of anatomic variants and congenital abnormalities of the biliary tree, we onducted a retrospective study of MRCP scans performed due to different indications to identify anatomic variants and congenital abnormalities of the pancreas and biliary tree. High field MR machines (1.0 T and 1.5 T) and 3D TSE sequences were used to obtain images in the axial and coronal planes with MIP reconstructions in all studies. MRCP showed low cystic duct insertion, medial cystic duct insertion, and a parallel course of the cystic and hepatic ducts as well as bile duct bifurcation abnormalities and aberrant right and left hapatica duct. We also present examples of the main types of congenital cystic dilatation of the bile ducts. The recognition of the main anatomic variants of the biliary tree and pancreatic ductshelps to optimize the diagnostic accurancy of MR cholangiopancreatography.

Malformación del sistema pancreático-biliar como causa de perforación espontánea de la vía biliar extrahepática / A pancreatobiliary malformation causing spontaneous perforation of the extrahepatic bile duct

La ascitis biliar usualmente resulta de la perforación espontánea o traumática del conducto biliar principal, usualmente en la unión con el conducto cístico. La obstrucción ductal distal, el reflujo hacia la vía biliar común, la debilidad o una muy localizada malformación mural de la pared del conducto común han sido propuestas como causas probables. Presentamos el caso de una niña de 11 meses de edad quien sufrió ascitis biliar como consecuencia de la ruptura del colédoco. La perforación probablemente se debió a una malformación de la unión pancreático-biliar. Se le derivó la bilis hacia el exterior mediante una sonda.

Biliaryascitis usually results from a spontaneous or traumatic perforation of the common bile duct, usually at the junction with the cystic duct. Distal duct obstruction, pancreatic fluid reflux up the common bile duct, congenital weakness or a localized mural malformation of the wall of the common duct have been proposed as possible causes. We present an 11-months-old infant girl who developed a biliary ascitis as a consequence of biliary rupture. Perforation was due to malformation of the pancreatico-biliary junction. An uneventful external derivation by tube was performed.

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.

Biliary atresia with situs inversus: an experience shared

Biliary Atresia [BA] is a well-known entity and can present with multiple congenital anomalies. BA is one of the most common conditions in which pediatric liver transplant is performed. Identification of Biliary atresia with situs inversus [SI] has not been documented in Pakistan. We report two such cases. First was an eighty-day-old baby boy, icteric from day of birth. On further evaluation had dextrocardia, SI, gross hydronephrosis [HN] of left kidney and stasis at pelvi ureteric junction [PUJ]. Liver biopsy showed biliary cirrhosis secondary to extra hepatic biliary atresia [EHBA]. The second baby presented at two months of age. Ultrasound abdomen and hepatobiliary scintigraphy confirmed liver in left hypochondrium [SI] and findings suggestive of BA. Echocardiography confirmed SI with mesocardia. In this paper we have described the association of BA with SI in two patients presenting at the pediatric Gastroenterology, hepatology and nutrition unit

Pancreatico-biliary ductal union.

A long common channel distal to the pancreaticobiliary junction is the commonest anomalous arrangement of the pancreaticobiliary ductal system and is mostly observed in patients with congenital choledochal cysts. APBDU without choledochal cyst is a high-risk condition for the development of gallbladder carcinomas. Prophylactic excision of the extrahepatic biliary system and reconstruction of the biliary tract with hepatico-jejunostomy are recommended. APBDU should always be kept in mind when a patient with a long history of abdominal pain is found to have gall bladder wall thickness even without gallstones on imaging by a CT Scan or Ultra Sound. ERCP should be performed in these patients in order to detect APBDU. This may allow early detection of carcinoma of the biliary tract. Presence of common channel may be associated with a lower incidence of gallstones. However it requires corroboration by other studies. On the other hand carcinoma of the gall bladder appears to have a close association with abnormally long common channel.