Enfermedad de Caroli: a propósito de un caso / Carolis disease: report of one case

Resumen La enfermedad de Caroli es una rara patología caracterizada por la existencia de dilataciones saculares segmentarias del árbol biliar intrahepático, que, por lo general, afectan a todo el parénquima hepático, aunque es poco frecuente el compromiso biliar extrahepático. Presentamos el caso de un varón que ingresó con un cuadro clínico de 45 días de evolución, consistente en ictericia colestásica, baja ponderal y prurito. La colangiorresonancia informó múltiples dilataciones saculares en las vías biliares intrahepáticas.

Abstract Caroli disease is a condition characterized by cystic dilatation of the intrahepatic biliary tree that usually affects the entire liver parenchyma, although extrahepatic biliary involvement is rare. The following is the case of a male patient who was admitted due to cholestatic jaundice, low weight, and pruritus for 45 days. Magnetic resonance cholangiopancreatography reported multiple cystic dilatations in the intrahepatic bile ducts.

Alagille Syndrome: Features and Outcome among Filipino Children

@#We report 13 children fulfilling criteria of Alagille syndrome. All had chronic cholestasis secondary to paucity of intrahepatic bile ducts and triangular facies. Eight children had associated congenital heart disease (six pulmonic stenosis, one each tetralogy of Fallot and patent ductus arteriosus), seven with butterfly vertebrae and one with posterior embryotoxon. Seven of the 13 children are alive and jaundice-free but three with concomitant hypercholesterolemia; the six other children died of liver-related complications.

Comparison of the Clinicopathologic Characteristics of Intraductal Papillary Neoplasm of the Bile Duct according to Morphological and Anatomical Classifications

BACKGROUND: Intraductal papillary neoplasm of the bile duct (IPNB) is a recently defined entity and its clinical characteristics and classifications have yet to be established. We aimed to clarify the clinical features of IPNB and determine the optimal morphological classification criteria. METHODS: From 2003 to 2016, 112 patients with IPNB who underwent surgery were included in the analysis. After pathologic reexamination by a specialized biliary-pancreas pathologist, previously suggested morphological and anatomical classifications were compared using the clinicopathologic characteristics of IPNB. RESULTS: In terms of histologic subtypes, most patients had the intestinal type (n = 53; 48.6%) or pancreatobiliary type (n = 33; 30.3%). The simple “modified anatomical classification” showed that extrahepatic IPNB comprised more of the intestinal type and tended to be removed by bile duct resection or pancreatoduodenectomy. Intrahepatic IPNB had an equally high proportion of intestinal and pancreatobiliary types and tended to be removed by hepatobiliary resection. Morphologic classifications and histologic subtypes had no effect on survival, whereas a positive resection margin (75.9% vs. 25.7%; P = 0.004) and lymph node metastasis (75.3% vs. 30.0%; P = 0.091) were associated with a poor five-year overall survival rate. In the multivariate analysis, a positive resection margin and perineural invasion were important risk factors for survival. CONCLUSION: IPNB showed better long-term outcomes after optimal surgical resection. The “modified anatomical classification” is simple and intuitive and can help to select a treatment strategy and establish the proper scope of the operation.

Spontaneous Rupture of Multiple Intrahepatic Cholangiocarcinoma in a Pregnant Patient

Spontaneous rupture of intrahepatic cholangiocarcinoma is rare due to its abundant fibrous stroma. We report our experience in a case of spontaneous rupture of intrahepatic cholangiocarcinoma in a pregnant patient. In our case, multiple hepatic masses with intratumoral hemorrhage were noted on post-delivery computed tomography and magnetic resonance imaging and they caused hemoperitoneum. A right hemi-hepatectomy was carried out to control the bleeding and the diagnosis of intrahepatic cholangiocarcinoma with metastasis was performed.

As mucinas e o NCAM (CD56) na colangiocarcinogênese intra-hepática / Mucins and NCAM (CD56) in intrahepatic cholangiocarcinogenesis

Introduction: Cholangiocarcinoma is the second most common malignant neoplasm of the hepatobiliary system. During cholangiocarcinogenesis phenotypic changes occur in the ductal epithelium, including the expression of mucins (MUC). However, the evaluating studies of the expression of mucins in the different stages of cholangiocarcinogenesis are scarce. CD56 has also contributed in differentiating benign ductal proliferation and cholangiocarcinoma; however, its expression has not been evaluated in dysplastic epithelium of the bile duct yet. Objective: To assess immunohistochemical profile of (MUC) 1, 2, 5, 6, and CD56 in cholangiocarcinoma, pre-neoplastic and reactive lesions in the epithelium of intrahepatic bile ducts. Material and methods: Immunohistochemical expression of MUC 1, 2, 5, 6, and CD56 were studied for 11 cases of cholangiocarcinoma and 83 intrahepatic bile ducts (67 reactive and 16 dysplastic). Variables were considered significant when p < 0.05. Results: The expression of MUC1 occurred in about 90% of the cholangiocarcinomas, contrasting with the low frequency of positive cases in reactive and dysplastic bile ducts (p < 0.001). However, there was no statistically significant difference in the expression of MUC5, MUC6 and CD56 between the reactive or dysplastic lesions and cholangiocarcinoma. The anti-MUC2 antibody was negative in all cases. Conclusions: MUC1 contributed for the differential diagnosis between cholangiocarcinoma and pre-neoplastic and reactive/regenerative lesions of intrahepatic bile ducts, and it should compose the antibodies panel aiming at improvement of these differential diagnoses. In contrast, MUC2, MUC5, MUC6 and CD56 were not promising in differentiating all the phases of cholangiocarcinogenesis...

Introdução: O colangiocarcinoma é a segunda neoplasia maligna mais comum do sistema hepatobiliar. Durante a colangiocarcinogênese podem ocorrer alterações fenotípicas do epitélio ductal, incluindo a expressão de mucinas. Entretanto, os estudos que avaliam a expressão das mucinas nas diferentes etapas da colangiocarcinogênese são escassos. O CD56, apesar de contribuir na diferenciação entre as proliferações ductais benignas e o colangiocarcinoma, ainda não teve a sua expressão avaliada no epitélio displásico dos ductos biliares. Objetivos: Analisar o perfil das mucinas (MUC) 1, 2, 5, 6 e do CD56 no colangiocarcinoma, nas lesões pré-neoplásicas e reacionais de ductos biliares intra-hepáticos. Material e métodos: A expressão imuno-histoquímica da MUC 1, 2, 5, 6 e do CD56 foram avaliadas em 11 colangiocarcinomas e 83 ductos biliares intra-hepáticos (67 reativos e 16 displásicos). As variáveis foram consideradas como significativas quando p < 0,05. Resultados: A expressão da MUC1 ocorreu em cerca de 90% dos colangiocarcinomas, contrastando com a baixa frequência de casos positivos nos ductos biliares reativos ou displásicos (p < 0,001). Não houve diferença estatisticamente significativa na expressão de MUC5, MUC6 e CD56 entre as lesões reativas, displásicas e o colangiocarcinoma. O anticorpo anti-MUC2 foi negativo em todos os casos. Conclusão: A MUC1 contribuiu no diagnóstico diferencial entre o colangiocarcinoma e as lesões pré-neoplásicas e reacionais/regenerativas dos ductos biliares intra-hepáticos, e deve compor o painel de anticorpos a ser empregado visando o aprimorando destes diagnósticos diferenciais. Contrariamente, a MUC2, MUC5, MUC6 e o CD56 não se mostraram promissoras na diferen...

A Case of Colloid Carcinoma Arising in Association with Intraductal Papillary Neoplasm of the Liver / 대한소화기학회지

Colloid carcinoma of the liver is very rare, and its clinicopathologic features have not been well characterized yet. We describe herein a case of colloid carcinoma of the liver. Imaging revealed a lobulated mass, measuring 12 cm in diameter at the right lobe of the liver with direct invasion of adjacent peripheral intrahepatic bile ducts. Right hemihepatectomy of the liver was performed according to the possibility of the tumor's malignant behavior. Histopathological examination of the specimen revealed large extracelluar stromal mucin pools containing floating cuboidal to columnar neoplastic cells without ovarian-like stroma. This case seemed to be colloid carcinoma arising in association with intraductal papillary neoplasm of the liver.

The Variation of Hepatic Duct Confluence and Asymptomatic Common Bile Duct Stone with Routine Intraoperative Cholangiogram during Laparoscopic Cholecystectomy / 대한소화기학회지

BACKGROUND/AIMS: Intraoperative cholangiogram (IOC) during laparoscopic cholecystectomy (LC) has been used to evaluate bile duct stone. But, the routine use of IOC remains controversial. With routine IOC during LC, we reviewed the variation of hepatic duct confluence and try to suggest the diagnostic criteria of asymptomatic common bile duct (CBD) stone. METHODS: We reviewed the medical record of 970 consecutive patients who underwent LC with IOC from January 1999 to December 2009, retrospectively. RESULTS: Nine hundered seventy patients were enrolled. IOC were successful in 957 (98.7%) and unsuccessful in 13 (1.3%). Eighty two of 957 patients (8.2%) were excluded because of no or poor radiologic image. According to Couinaud's classification, 492 patients (56.2%) had type A hepatic duct confluence, 227 patients (26.1%) type B, 15 patients (17%) type C1, 43 patients (4.9%) type C2, 72 patients (8.2%) type D1, 21 patients (2.4%) type D2, 1 patient (0.1%) type E1, 1 patient (0.1%) type E2, 2 patients (0.2%) type F, and 1 patient (0.1%) no classified type. The CBD stone was found in 116 of 970 (12.2%) patients. In 281 patients, preoperative serologic and radiologic tests did not show abnormality. When preoperative findings were not remarkable, there was no difference of clinical features between patients with or without CBD stones. CONCLUSIONS: Although IOC during LC has some demerits, it is a safe and accurate method for the detection of CBD stone and the anatomic variation of intrahepatic duct.

Complexo de von Meyenburg: relato de caso e revisão da literatura / Von Meyenburg complex: case report and literature review

Os hamartomas biliares, conhecidos como complexo de von Meyenburg, são lesões tipicamente benignas que podem mimetizar metástases aos exames de imagem. Neste estudo é relatado um caso de complexo de von Meyenburg e são discutidos o diagnóstico por imagem, a associação com doença policística do adulto, e o potencial maligno atualmente considerado e frequentemente ignorado deste achado, que torna necessário o acompanhamento.

Biliary hamartomas, also known as von Meyenburg complex, are typically benign lesions that may mimic metastases at imaging studies. The present study reports a case of von Meyenburg complex and discusses the imaging diagnosis, the association with adult polycystic disease and the malignant potential of this finding currently considered and frequently ignored, which raises the necessity of follow-up.

Surgical treatment of caudate hepatolithiasis / 第二军医大学学报

Objective: To summarize our experience on surgical treatment of caudate hepatolithiasis, so as to improve the diagnosis and treatment of caudate hepatolithasis. Methods: The clinical data, mode of operation, post-operation complications, and follow up findings were retrospectively analyzed in 19 patients with caudate hepatolithasis from Jan. 2000 to Dec. 2006. Results: The 19 cases included 8 males and 11 females. Totally they had undergone 30 times of operation, with 12 cases only had once, 4 had twice, 2 had thrice, and 1 had 4 times. All cases received B ultrasound, CT, MRCP or ERCP and were confirmed to have caudate hepatolithiasis. Fourteen cases were complicated with opening stonosis of caudate bile duct. Eleven cases received caudate lobe resection. Eight cases received caudate expansion of the bile duct or plastic repair of duct + caudate duct exploration and calculus extraction, with a success rate of 100% and the patients were stone free after surgery. Eighteen patients (94.7%) were followed up for a mean of (17.6±2.3) months. Two cases had stone recurrence. The excellent and good rate was 88.9% in the present cohort; all those who received resection of the caudate lobe had excellent or good outcomes. Conclusion: It is very complex and difficult to treat caudate bile duct stone; caudate duct exploration and calculus extraction usually have unsatisfactory outcomes. Caudate lobe resection can clear the lesions and has a good long-term effect, which is largely dependent on the rich experience and the skillfulness of the surgeons.

Experience of right hemihepatectomy for treatment of hepatolithiasis in the right or both lobes / 第二军医大学学报

Objective: To summarize our experience on right hemihepatectomy for treatment of hepatolithiasis. Methods: The clinical data of thirty-nine patients suffering from intrahepatic bile duct stones, who had undergone right hemihepatectomy in our hospital from Jan. 2000 to Dec. 2006, were retrospectively analyzed. The outcomes of operation, complication, and the follow-up findings were analyzed; and the clinical experience was summarized. Results: The 39 patients included 8 males and 31 females, with an age range of 23-65 years old and a mean of (44.2 ± 10.5) years old. All patients had various degrees of hepatobiliary stricture. All cases underwent right hemihepatectomy plus eholedoehotomy and T-tube drainage, including 28 also had cholecystectomy, 1 had additional left lateral hepatectomy, 1 had choledoehocystomy plus hepatojejunostomy, and 1 had demolishment of anastomotie stoma of choledochoduodenostomy. The post-operation complications included pleural effusion (17.9%), subphrenic infection (12.8%), liver failure (7.7%), biliary fistula (5.1%), and infection of incisional wound (5.l%). Two patients (5.1%) died of liver failure during the hospital stay. One was indicated of having intrahepatic cholangiocarcinoma and died of recurrence of cholangiocarcinoma 6 months after operation. Thirty-six patients (92.3%) received long-term follow-up (a median of [42.5±2.5] months) and 34 (94.4%) of them were symptom free. Excellent outcomes were achieved in the 31 patients with isolated right intrahepatic bile duct stones. Conclusion: Right hemihepatectomy is effective for right hepatolithiasis, especially for patients combined with right hepatic duct stricture. However, it should be noted that the operation is very difficult and has many complications, so the indications and contraindications of right hemihepatectomy should be strictly selected.

Three-dimensional contrast-enhanced ultrasonic cholangiography in cadaver liver / 中华医学超声杂志（电子版）

Objective To assess the feasibility of three-dimensional contrast-enhanced ultrasonic cholangiography(3D-CEUSC) in cadaver liver.Methods The 3D-CEUSC was performed in 6 cases of cadaver liver.Image quality of 3D-CEUSC was evaluated.The visualization of branching orders,the degree of visibility and coincidence of morphous were compared with those of cholangiagraphy using fluoroscopy.Results The imaging quality of 3D-CEUSC was inferior to that of cholangiography with significant difference.The three-dimensional biliary tree structures were visualized in all 6 3D-CEUSC.The maximum visualization of branching orders in 3D-CEUSC was (3.67±0.52),which was equal to the results(4.00±0.63)by cholangiography (P=0.465).The degrees of visibility of biliary tree were equivalent with those by cholangiography in the first and second order with significant difference.The coincidence of morphous was excellent compared the images of 3D-CEUSC with direct X-ray cholangiography.Conclusion 3D-CEUSC is a new technique as a useful supplement to cholangiography in evaluation of biliary anatomy and variation before graft harvesting in LDLT.

Changes in Sonographic Findings after Treatment of Patients with Clonorchiasis in a Heavy Endemic Area

We measured changes in sonographic findings of patients with clonorchiasis after a treatment in a highly endemic area. A total of 347 residents showed positive stool results for Clonorchis sinensis eggs in a village in northeastern China, and were treated with praziquantel. Of them, 132 patients underwent abdominal sonography both before and 1 year after treatment, and the changes in sonographic findings of 83 cured subjects were compared. Diffuse dilatation of intrahepatic bile ducts (DDIHD) was found in 82 patients (98.2%) before and 80 (96.4%) after treatment, which was improved in 3, aggravated in 1, and unchanged in 79 patients. Increased periductal echogenicity (IPDE) was observed in 42 patients (50.6%) before and 45 (54.2%) after treatment, which was improved in 5, aggravated in 8, and unchanged in 70 patients. Floating echogenic foci in the gallbladder (FEFGB) was detected in 32 patients (38.6%) before and 17 (20.5%) after treatment, which was improved in 20, aggravated in 5, and unchanged in 58 patients. Improvement of FEFGB only was statistically significantly (P = 0.004). The present results confirm that DDIHD and IPDE persist but FEFGB decreases significantly at 1 year after treatment. In a heavy endemic area, the sonographic finding of FEFGB may suggest active clonorchiasis 1 year after treatment.

The Clinicopathological Parameters for Making the Differential Diagnosis of Neonatal Cholestasis

BACKGROUND: The diseases that cause neonatal cholestasis display several overlapping clinical feature. Making the differential diagnosis using liver biopsy specimens from infants with neonatal cholestasis is important for delivering the proper treatment. METHODS: We assessed the clinical manifestations, laboratory data, and histopathologic features of the pretreatment liver biopsy specimens from patients suffering with biliary atresia (n=66), intrahepatic bile duct paucity (n=15), and neonatal hepatitis (n=21). RESULTS: The gender distribution was nearly equal for the patients with biliary atresia and intrahepatic bile duct paucity, whereas males predominated for the cases of neonatal hepatitis. Only the gamma-glutamyl transferase level differed significantly amongst the groups. The diagnostic features for making the differential diagnosis of bile duct lesions included marked bile ductular proliferation, severe fibrosis, and bile duct loss. The difference of the average percentage of portal tracts with bile duct loss was statistically significant between the patients with intrahepatic bile duct paucity (73.9%) and those patients with neonatal hepatitis (39.1%) (p<0.001). CONCLUSIONS: Bile ductular proliferation, bile duct loss, and advanced fibrosis are useful for the differential diagnosis of neonatal cholestasis. Moreover, stricter diagnostic criteria for bile duct loss (more than 2/3 of bile ducts) should be applied for the definitive diagnosis of intrahepatic bile duct paucity, because bile duct loss also frequently occurs in infants suffering with neonatal hepatitis.