Congenital Cystic Malformation of the Bile Ducts: Report of a Case and Review of Related Literature

A 20-year-old woman had a cyst of the proximal part of the common bile duct and a cyst of the left hepatic duct; these lesions were diagnosed preoperatively by intravenous cholangiography and successfully operated upon. At the time of writing, she has been followed up for one year.Congenital defects in the biliary system are rare and, in a review of the literature, only two cases were found similar to this one. It is generally accepted that these lesions are congenital, but the exact pathogenesis is unknown.Alonso-Lej, Rever and Pessagno² reviewed the literature in 1959 and found 403 authentic congenital cysts of the hepatic ducts. The most common congenital defect is a single choledochal cyst of the lower end of the common bile duct. Pain, jaundice and tumour are the main symptoms.Until the advent of intravenous cholangiography, these lesions were seldom recognized preoperatively. Means of operative repair as well as complications and prognosis are reviewed.     

Formalin-induced experimental sclerosing cholangitis in the rat

The few reported cases of sclerosing cholangitis following removal of an echinococcus cyst are thought to be a consequence of the chemical action of formalin used for sterilization of the residual cavity. The aim of this study was to assess this hypothesis. We injected 0.15ml of 2% buffered formalin solution into the central hepatic lobe of five rats, after a midline laparotomy. At 6, 12, 18 and 24 weeks after formalin injection all rats were reoperated upon and a sample of hepatic parenchyma from both the central and the left hepatic lobe was obtained for microscopic evaluation. Our findings, dilatation of portal tracts and bile canaliculi, thickening of the pericanalicular cytoplasm, portal and periportal inflammatory cell infiltration and fibrosis and enlargement of the perisinusoidal space of Disse, suggest that 2% formalin solution leads to the development of essential phenomena of cholestasis and sclerosing cholangitis in the rat, so thus it should be avoided in liver hydatid disease surgery.     

Ultrasonic diagnosis of liver hydatidosis

The paper is concerned with a study of an ultrasound picture of verified hydatidosis in 62 patients. The symptoms of hydatid cysts are the presence of daughter cysts and "hydatid sand", cuticular membrane desquamation, local cyst wall thickening and parietal layers, a necrotic zone in a preparasitic space and cyst calcification. The detection of these symptoms helps to estimate not only the type of a hydatid cyst but also the state and character of parasite activity permitting a choice of therapeutic tactics.     

Intrabiliary rupture of a hydatid liver cyst: a case report

Intrabiliary rupture of a hydatid liver cyst is a rare occurrence which may result in the development of obstructive jaundice and cholangitis. In this report we discuss the diagnostic and therapeutic management of a patient in whom the parasitic nature of cholangitis was underestimated due to the small size and site of the cyst, and to the misleading concomitant presence of cholelithiasis.     

腹腔镜胆囊切除术（LC 术）致胆管损伤的诊治体会

摘要目的：总结腹腔镜胆囊切除术（laparoscopic cholecystectomy,LC 术）中胆管损伤的诊治体会。方法：回顾19 例LC 术致胆管 损伤病例的临床资料,分析其发生的类型及原因,并总结其诊断和治疗要点。结果：19 例患者中,1 例发生在左右肝管汇合以上处 损伤,2 例发生胆总管缺损伤,3 例发生胆总管横断伤,5 例发生胆总管侧面伤,3 例发生胆总管钳夹但未切断,1 例发生右肝管损 伤,4例发生胆囊管残端漏。治疗方法应视胆管损伤类型的不同而不同。采用断端处胆管端端吻合,同时放置T 管引流、单纯胆总 管T 管引流、开腹去除误夹夹子、ERCP检查放置鼻胆管引流及胆肠Rouxeny 吻合术。胆肠Rouxeny吻合术是临床上最常用的修 补胆道损伤的手术方法。随访6 个月～18 年,恢复好,无1 例死亡。结论：胆管损伤是LC 术最常见的并发症之一,规范的操作及 手术适应症的掌握能减少其发生。一旦出现胆管损伤,及时诊断及正确处理能减少其不良后果。     

Structural and functional analyses of liver cysts from the BALB/c-cpk mouse model of polycystic kidney disease

Liver cysts arising from hepatic bile ducts are a common extra-renal pathology associated with both autosomal dominant and recessive polycystic kidney disease in humans. To elucidate the functional and structural changes inherent in cyst formation and growth, hepatic bile duct epithelia were isolated from the BALB/ c-cpk mouse model of polycystic kidney disease. Light and transmission electron microscopy revealed substantial fibrosis in the basal lamina surrounding hepatic bile duct cysts isolated from heterozygous (BALB/c-cpk/+) and homozygous (BALB/c-cpk/cpk) animals. Scanning electron microscopy and length analysis of normal, precystic and cystic bile ducts provided the unique observation that primary cilia in cholangiocytes isolated from bile ducts and cysts of animals expressing the mutated cpk gene had lengths outside the minimal and maximal ranges of those in cells lining bile ducts of wild-type animals. Based on the hypothesis that PKD is one of several diseases characterized as ciliopathies, this abnormal variability in the length of the primary cilia may have functional implications. Electrophysiological analyses of freshly isolated cysts indicate that the amiloride-sensitive epithelial Na(+) channel (ENaC) is inactive/absent and cAMP-mediated anion secretion is the electrogenic transport process contributing to cyst fluid accumulation. Anion secretion can be stimulated by the luminal stimulation of adenylyl cyclase.     

腹腔镜胆囊切除术（LC术）致胆管损伤的诊治体会

目的：总结腹腔镜胆囊切除术（1aparoscopiccholecystectomy,LC术）中胆管损伤的诊治体会。方法：回顾19例LC术致胆管损伤病例的临床资料,分析其发生的类型及原因,并总结其诊断和治疗要点。结果：19例患者中,1例发生在左右肝管汇合以上处损伤,2例发生胆总管缺损伤,3例发生胆总管横断伤,5例发生胆总管侧面伤,3例发生胆总管钳夹但未切断,1例发生右肝管损伤,4例发生胆囊管残端漏。治疗方法应视胆管损伤类型的不同而不同。采用断端处胆管端端吻合,同时放置T管引流、单纯胆总管T管引流、开腹去除误夹夹子、ERCP检查放置鼻胆管引流及胆肠Rouxeny吻合术。胆肠Rouxeny吻合术是临床上最常用的修补胆道损伤的手术方法。随访6个月～18年,恢复好,无1例死亡。结论：胆管损伤是LC术最常见的并发症之一,规范的操作及手术适应症的掌握能减少其发生。一旦出现胆管损伤,及时诊断及正确处理能减少其不良后果。     

Giant hydatid cyst of the liver with a retroperitoneal growth: a case report

ABSTRACT: INTRODUCTION: Hydatid disease is a helminthic anthropozoonosis with worldwide distribution due to the close associations among sheep, dogs, and humans. It can occur almost anywhere in the body with a variety of imaging features, which may change according to the growth stage, associated complications, and affected tissues. A definitive diagnosis requires a combination of imaging, serologic and immunologic studies. Ultrasonography, computed tomography and magnetic resonance imaging are highly accurate in detecting a hepatic hydatid cyst. However, hepatic hydatid cysts in an unusual location and/or of an unusual dimension, with atypical imaging findings, may complicate the differential diagnosis. Surgical treatment remains the best treatment. CASE PRESENTATION: We describe an unusual case of a giant hydatid cyst, with exophytic growth from the right lobe of the liver of a 55-year-old Egyptian man. The cyst was strongly adhered to his ipsilateral kidney, which was displaced in a downwards and anterior direction, close to his abdominal wall, simulating a retroperitoneal origin. This atypical growth raised doubts about the most appropriate surgical approach. Magnetic resonance imaging easily clarified the origin of the cyst as our patient's liver, allowing accurate surgical planning. CONCLUSION: Rarely, hydatid cysts can reach an extremely large size without any additional symptoms. Giant cysts need radical therapy because they might lead to perforation and anaphylaxis in some patients. Magnetic resonance imaging is very useful in the study of hydatid disease because of its capacity to allow a large field of view, multiplanar acquisition, and high contrast resolution. In some unusual hepatic presentations, magnetic resonance imaging can be used to determine the correct anatomical relationships.     

塑料胆道支架引流术治疗复杂性胆总管结石的临床研究

目的:探讨内镜下逆行胰胆管造影术(ERCP)下塑料胆道支架引流术治疗复杂性胆总管结石的临床疗效和安全性。方法:回顾性分析2011年9月至2013年9月在我院经ERCP下胆道支架引流术治疗的32例复杂性胆总管结石患者的临床病例资料。结果:32例患者塑料胆道支架引流术全部成功,平均手术时间15-30分钟。术后,2例发生高淀粉酶血症,经禁食72小时后恢复正常,无穿孔、消化道大出血等ERCP严重并发症发生。术后1周,患者腹痛、发热消失,转氨酶及胆红素水平明显下降,平均住院时间6-15天。3个月复查B超,发现结石缩小19例,结石碎裂1例,支架脱落1例。术后7天、术后3个月的肝功能指标与术前比较均显著改善,差异均有统计学意义(P0.05)。结论:ERCP下塑料胆道支架引流术是一种复杂性胆总管结石安全有效的治疗方法,具有创伤小、风险较低、操作时间短、患者易耐受及手术成功率高等优点。     

Early cancer in congenital choledochal cyst

We report a case of 35-yr-old woman with early cancer in congenital choledochal cyst. She had a five-year history of intermittent right upper abdominal pain and intermittent jaundice. In this period she had a few abdominal ultrasonographies, but the cholelithiasis had not been found. Now, she was admitted to our hospital because she felt right upper abdominal pain with slight jaundice and subfebrile temperature four weeks ago. Abdominal ultrasonography showed enormous dilatation of the common bile duct, which was suspected as choledochal cyst. Computed tomography and endoscopic retrograde cholangiopancreatography revealed cystic dilatation of extrahepatic bile duct. An anomalous pancreaticobiliary junction was not found. The patient with congenital choledochal cyst was operated on. The excision of choledochal cyst was done with hepaticojejunostomy Roux-en-Y. There were no lymph nodes metastases. On the central part of choledochal cysts mucosa, it was shown a white plain area of thickness 0.3 cm and 0.8 cm in diameter. Histologically it was well-differentiated tubular adenocarcinoma, which was limited to the mucosa and which did not penetrate to other parts of the bile duct wall. From our knowledge, only small number cases of early cholangiocarcinoma in choledochal cyst were until now reported. Nearly eight years after the operation the patient feels very well, and has optimal working ability (Karnofsky 100%).     

Focus: Zoonotic Disease: Retrospective Evaluation of Hydatid Cyst Cases During Pregnancy

Aim: Zoonotic parasite infections affect many pregnant people around the world. Hydatid cystic disease is also a zoonotic disease caused by Echinococcus sp. This study aims to present the maternal-fetal results and clinical treatment of pregnant women diagnosed with liver hydatid cyst (CH). This zoonotic disease is discussed again in the light of current literature. Materials and Methods: Pregnant women with hydatid cyst monitored in a tertiary health center between 2018 and 2020 were evaluated. Seven cases were included in this study. We retrospectively collected and analyzed clinical data, which did not interfere with medical treatment. Results: Albendazole was started as medical therapy in six patients, and percutaneous drainage was applied to one patient. Three of our six patients who started medical treatment had to undergo surgery due to maternal complications that developed despite medical treatment. Two of our patients were delivered with a cesarean section due to the obstetric indications. Discussion: Hydatid cysts are most commonly caused by Echinococcus granulosus infection and most common in the liver. The diagnosis of liver hydatid cysts is not difficult, but pregnant women’s treatment methods have some problems. Although both medical and surgical treatments are available, there is no consensus. We would also like to underscore that echinococcal disease of the liver should be kept in mind in the differential diagnosis of abdominal pain, jaundice, and/or fever, especially in endemic regions. We think that when we increase awareness about this disease, we can improve fetal and maternal outcomes by making an early diagnosis and management.     

Surgical alternatives in the treatment of hepatic hydatidosis]

Echinococcosis is an endemic zoonosis in the south of Chile, so we have the opportunity to treat many patients especially the liver location which is the most common situation of this disease. Hepatic hydatid cyst presents its own morbidity and mortality due its complications and associated with surgical procedures. Morbidity has rates between 11 and 86% and is related with previous surgical interventions, cysts complications, the necessity of perform additional procedures during surgery and the treatment of other disease locations. Mortality by otherwise has rates below 5%. But both, morbidity and mortality persist high in spite of technological advances and therapeutical improvement. The surgical treatment of hepatic hydatidosis can be divided in four phases: surgical area isolation, cysts evacuation, treatment of cyst complications (biliary tract rupture, hepatothoracic transit, etc.), and residual cavity treatment. For each one exist different alternatives. On the other hand, hydatid cyst surgery can be classified in conservative and resective procedures. In the first group we consider marsupialization, cystostomy, Knowsly or Posadas operation and cystojejunostomy. In the resective group we include pericystectomy, partial or subtotal cystectomy and the different types of hepatectomies. Finally, some other surgical procedures are under evaluation. These include the laparoscopic approach of liver echinoccosis with few studies in the field, but with hopeful results.     

Pelvic Hydatidosis Mimicking a Malignant Multicystic Ovarian Tumor

Echinococcosis is a multisystem disease and has propensity to involve any organ, an unusual anatomical site, and can mimic any disease process. Primary peritoneal echinococcosis is known to occur secondary to hepatic involvement but occasional cases of primary peritoneal hydatid disease including pelvic involvement have also been reported. We report here 1 such case of primary pelvic hydatidosis mimicking a malignant multicystic ovarian tumor where there was no evidence of involvement of the liver or spleen. Our patient, a 27-year-old female, was detected to have a large right cystic adnexal mass on per vaginal examination which was confirmed by ultrasonography. Her biochemical parameters were normal and CA-125 levels, though mildly raised, were below the cut off point. She underwent surgery and on exploratory laparotomy, another cystic mass was found attached to the mesentery of the small gut. The resected cysts were processed histopathologically. On cut sections both large cysts revealed numerous daughter cysts. Microscopic examination of fluid from the cysts revealed free scolices with hooklets and the cyst wall had a typical laminated membrane with inner germinal layer containing degenerated protoplasmic mass. The diagnosis of pelvic hydatid disease was confirmed and patient was managed accordingly. Hydatid disease must be considered while making the differential diagnosis of pelvic cystic masses, especially in endemic areas.     

Protein composition of liver cyst fluid from the BALB/c‐cpk/+ mouse model of autosomal recessive polycystic kidney disease

Cysts arising from hepatic bile ducts are a common extra‐renal pathology associated with polycystic kidney disease in humans. As an initial step in identifying active components that could contribute to disease progression, we have investigated the protein composition of hepatic cyst fluid in an orthologous animal model of autosomal recessive polycystic kidney disease, heterozygous (BALB/c‐cpk/+) mice. Proteomic analysis of cyst fluid tryptic digests using LC‐MS/MS identified 303 proteins, many of which are consistent with enhanced inflammatory cell processes, cellular proliferation, and basal laminar fibrosis associated with the development of hepatic bile duct cysts. Protein identifications have been submitted to the PRIDE database ( http://www.ebi.ac.uk/pride ), accession number 9227.     

ERCP/EST-LC治疗胆囊结石合并胆总管结石临床疗效及并发症

目的:考察内镜下逆行胰胆管造影术/十二指肠乳头括约肌切开术加腹腔镜胆囊切除术(endoscopic retrograde pancreatic angiography/endoscopic sphincterotomy-1aparoscopic cholecystectomy,ERCP/EST-LC)对胆囊结石合并胆总管结石的临床疗效和安全性。方法:选80例胆囊结石合并胆总管结石患者,随机数字表法分为两组,每组40例,对照组进行LCBDE-LC手术,研究组进行ERCP/EST-LC手术,以手术成功率、围术期相关指标和术后并发症等指标考察对患者的临床疗效。结果:对照组手术成功率为95.0%,研究组患者手术成功率为97.5%,两组无显著差异(P>0.05),研究组患者的手术时间和术中出血量与对照组相比均无显著差异(P>0.05),研究组胃肠功能恢复时间为39.64±5.34 h,显著长于对照组的37.19±3.17 h(P<0.05),研究组住院时间为14.17±2.06 d,显著长于对照组的11.85±2.71 d(P<0.05)。两组患者的胆道感染、急性胰腺炎、肠穿孔、结石残留以及胆管炎的发生率无显著差异(P>0.05),对照组胆漏发生率为7.50%,显著高于研究组的0.00%(P<0.05),而研究组术后出血发生率为10.00%,显著高于对照组的2.50%(P<0.05),对照组并发症总发生率为12.50%,研究组为15.00%,两组比较无显著差异(P>0.05)。对照组术后一年复发率为15.00%,研究组的复发率为17.50%,经统计分析,两组术后复发率无显著差异(P>0.05),其余患者无腹痛、发热、黄疸等情况。结论:ERCP/EST-LC治疗胆囊结石合并胆总管结石临床疗效确切、并发症少,安全性高。     

Infection Status of Hydatid Cysts in Humans and Sheep in Uzbekistan

Uzbekistan is endemic of cystic echinococcosis (CE). In order to estimate endemicity of CE, we collected data from emergency surgery due to CE in 2002-2010 and also investigated the prevalence of hydatid cysts in the liver and lungs of sheep at an abattoir in Uzbekistan from July 2009 to June 2010. In 14 emergency hospitals, 8,014 patients received surgical removal or drainage of CE during 2002-2010, and 2,966 patients were found in 2010. A total of 22,959 sheep were grossly examined of their liver and lungs, and 479 (2.1%) and 340 (1.5%) of them were positive for the cyst in the liver and lungs, respectively. Echinococcus granulosus is actively transmitted both to humans and sheep, and CE is a zoonotic disease of public health priority in Uzbekistan.     

Granular cell tumor of the biliary system: a report of 2 cases with cytologic diagnosis on endoscopic brushing

BACKGROUND: Granular cell tumors (GCTs) of biliary system are rare. GCTs show a striking preponderance for young, black females, who generally present with obstructive jaundice. To our knowledge, these are the first 2 reports of GCT of biliary system identifed on endoscopic brushing cytology. CASES: In case 1, a 24-year-old, black woman presented with a 5-month history of pruritus. Radiographic studies demonstrated a mass in the distal common bile duct. Endoscopic biopsy and bile duct brushing were diagnosed as GCT. A Whipple procedure was confirmatory of GCT. In case 2, a 38-year-old, black female presented with a 7-month history of pruritus and jaundice. Radiographic studies showed a stricture of the common hepatic duct at the hilum. Endoscopic brushing cytology of the stricture yielded only a few sheets of granular cells that were missed on initial screening. Suspicion of cholangiocarcinoma prompted surgery, and final histopathology showed GCT. Both patients were well 1 1/2 and 6 years after presentation. CONCLUSION: GCT of the bile duct can be diagnosed on endoscopic brushing and should be considered in the cytologic differential diagnosis in the appropriate clinical settings.     

Intraabdominal hydatid cyst: a case report

BACKGROUND: Hydatid disease is caused by Echinococcus granulosus, endemic in cattle and sheep-raising regions of the world such as Central Europe, South America, Australia, New Zealand and South Africa. Although hydatid disease is more common in liver and lung, it also affects brain, kidney, spleen and muscle. We present a case of intraabdominal hydatid cyst, diagnosed by fine needle aspiration cytology, producing an indentation of the liver, which is uncommon. CASE: A male patient presented with right side abdominal pain. On ultrasonography an intraabdominal solid mass (right hypochondrial) was revealed, and subsequently FNA was done. Smears were diagnostic of hydatid cyst. CONCLUSION: FNAC is a sensitive and rapid technique in diagnosis of hydatid cysts. The present case is unusual, owing to its presentation as a solid abdominal mass seeding over the liver and mimicking malignancy radiologically.     

Lipids in the laminated layer of liver, lung and daughter hydatid cysts of equine Echinococcus granulosus (Cestoda)

Lipids extracted from the laminated layers of horse liver and lung hydatids, including a daughter liver cyst, were analysed using TLC. No differences in lipid composition was detected in 11 liver cysts, whether from the same or different livers, and di- and triacylglycerols, cholesterol, wax and steryl esters, oleic acid, sphingomyelin, phosphatidyl choline, phosphatidyl inositol and ceramide hexosides were detected. The daughter cyst differed from its "parent" cyst in lacking diacylglycerols and wax and steryl esters. The lung cyst differed from the liver cysts in that cholesterol, wax and steryl esters and diacylglycerols were not detected.     

Bilious pericardial effusion at initial presentation in a patient with lung cancer

Background  

Cardiac tamponade as the initial manifestation of metastatic cancer is a rare clinical entity. Furthermore, a thoraco-biliary fistula is another rare complication of echinococcosis due to rupture of hydatid cysts located at the upper surface of the liver to the pleural or pericardial cavity. We report a case of non-small cell lung cancer with a coexisting hepatic hydatid cyst presenting as a bilious pericardial effusion.