Biliary Irrigation Research Articles

Re-evaluation of Laparoscopic Hepatic Subcapsular Spider-Like Telangiectasis Sign: A Highly Accurate Method to Diagnose Biliary Atresia in Infants.

ObjectiveOperative cholangiography, the gold standard for the diagnosis of biliary atresia (BA), is being challenged due to an increase in the studies of misdiagnosis. A previous study has shown that the laparoscopic hepatic subcapsular spider-like telangiectasis (HSST) sign was accurate for diagnosing BA. This study aims to compare the performance of the HSST sign with cholangiography in the identification of BA.MethodsWe prospectively screened consecutive infants with cholestasis who underwent laparoscopic exploration in this multicenter study. Demographics, intraoperative findings (videos and images), and outcomes were retrospectively analyzed. The data of the HSST sign and cholangiography were compared according to the final diagnosis. Then, the diagnostic accuracy of the BA using the HSST sign and cholangiography was validated in other independent cohorts.ResultsA total of 2,216 patients were enrolled in this study. The sensitivity and negative predictive values were both 100% for diagnosing BA based on the HSST sign and cholangiography. The specificity, negative predictive value, and accuracy of the HSST sign (97.2, 99.2, 99.3%) in discriminating BA were significantly higher than operative cholangiography (81.6, 94.9, 95.8; p < 0.001). Moreover, to realize the early diagnosis of BA, the accuracy of the HSST sign in identifying BA was better than cholangiography in the subgroup of neonates (98.7% vs. 95.0%; p = 0.032). Interestingly, 92 non-BA patients without the HSST sign had positive cholangiography. Among them, 28 infants had negative cholangiography when the common bile duct was compressed and 39 patients displayed visible bile ducts due to repeated postoperative biliary irrigation. The other 25 patients (18 with the Alagille syndrome, 5 with progressive familial intrahepatic cholestasis, and 2 with the neonatal hepatitis syndrome) had consistently positive cholangiography. In the independent validation cohort, the diagnostic accuracy of the HSST sign (99.2%) was higher than cholangiography (95.0%, p = 0.012).ConclusionThe laparoscopic HSST sign is superior to cholangiography in the diagnosis of BA in the infants with cholestasis and has advantages in early diagnosis. This method is expected to become a novel shift for diagnosing BA during ongoing laparoscopy.

Saline Solution Irrigation of the Bile Duct after Stone Removal Reduces the Recurrence of Common Bile Duct Stones.

Common bile duct (CBD) stone is a relatively common but potentially life-threatening disease. Endoscopic sphincterotomy (EST) has been performed as standard therapy for CBD stones, but the rate of recurrence of CBD stones is high. Risk factors have been poorly defined, and no effective means for the prevention of the recurrence of CBD stones have been established so far. We aimed to identify significant risk factors for the recurrence of bile duct stones. This study included 477 patients (231 women; mean age, 80.5 years) who underwent EST and cleared CBD stones on cholangiography. A retrospective analysis was performed for the consecutively collected data. During the follow-up period of 6-75 months, the recurrence of CBD stones was observed in 99 patients (20.8%). The median time to the recurrence was 19.0 months (range 4-72 months). Multivariate analysis identified the need for mechanical lithotripsy, which was used for stone fragmentation, as a risk factor. Mechanical lithotripsy caused cholangiography-negative small residua. Notably, saline solution irrigation of the bile duct reduced the recurrence of CBD stones. These results demonstrate that subsequent biliary irrigation after stone removal may prevent the recurrence of CBD stones by clearing small residual fragments.

A Rare Cause of Obstructive Jaundice: A Case Report

An 81-year-old man with myelodysplastic syndrome and transfusion-dependent anemia was admitted to the hospital for evaluation of jaundice and dark urine of 1-day duration. He denied abdominal pain, nausea, vomiting, melena, rectal bleeding or pale stool. There was no prior history of biliary interventions or trauma. Vitals were within normal limits and he was afebrile. Physical examination was significant for icterus. Laboratory values were- Hb - 5.1 from 8.1 gm/dL 1 week ago, Platelet count -34 K/ul, alanine aminotransferase- 430 IU/L, aspartate aminotransferase- 212 IU/L, alkaline phosphatase-288 IU/L, gamma glutamyl transferase- 918 IU/L, total bilirubin-6.4 mg/dL, direct bilirubin-5.01 mg/dL and INR-1.2. CT abdomen and pelvis revealed intrahepatic biliary dilatation and common bile duct dilatation of 1.1 cm. An endoscopic retrograde cholangiopancreatography [ERCP] was performed and cholangiogram showed multiple elongated deformed filling defects in the main bile duct [figure-1]. Biliary sphincterotomy and balloon sweep was performed that removed numerous large old blood clots from the CBD [figure-2]. Bile duct was adequately irrigated with sterile water and all remaining clots were cleared. Occlusion cholangiogram showed no more filling defects [figure-3]. There were no stones seen or extracted during the entire procedure. Patient required 2 units of blood and 2 units of platelets transfusion prior to the procedure and no post-procedural bleeding was observed. Bilirubin and liver enzymes trended down and hemoglobin remained stable. Patient was discharged home after clinical recovery. Discussion Iatrogenic or accidental trauma from hepatobiliary procedures is the most common etiology for haemobilia. It can also occur as part of certain disorders such as cholangitis, hepatobiliary cyst/cancers, choledocholithiasis and vascular abnormalities in the liver. However, spontaneous haemobilia is rarely reported in literature. Triad of upper abdominal pain, gastrointestinal bleeding, and jaundice are the common clinical presentations. This case is unique in that our patient had obstructive jaundice in the absence of other clinical symptoms such as abdominal pain, hematemesis or melena. Management of haemobilia is aimed at control of bleeding and relieving biliary obstruction. ERCP is helpful in diagnosing the cause of haemobilia and relieving biliary obstruction but trans arterial embolization is the gold standard in management of haemobilia.1244_A.tif Figure 1: Cholangiogram with multiple filling defects1244_B.tif Figure 2: Old blood clots at ampulla1244_C.tif Figure 3: Occlusion cholangiogram after biliary irrigation

Endoscopic Biliary Irrigation with N-Acetylcysteine of Intraductal Papillary Mucinous Neoplasm of Bile Ducts

Endoscopic Biliary Irrigation with N-Acetylcysteine of Intraductal Papillary Mucinous Neoplasm of Bile Ducts

Endoscopic retrograde cholangiopancreatography combined with extracorporeal shock wave lithotripsy in treating intractable choledocholithiasis with huge bile ductal stones in elderly patients

Objective To evaluate the effect of endoscopic retrograde cholangiopancreatography (ERCP) combined with extracorporeal shock wave lithotripsy (ESWL) in treating intractable choledocholi-thiasis with huge bile ductal stones in elderly patients. Methods 46 patients were diagnosed with intractable choledocholithiasis with huge bile ductal stones by ERCP. Integrated nasobiliary and pancreatic stents were placed initially. A second ERCP was performed to remove fragmented stones after ESWL. Results The average number and diameter of bile ductal stones were 1.8 (1~4) and 2.6 cm (2.0~3.2), respectively. All patients successfully underwent ERCP and no ERCP-related complications occurred. The average shock wave energy, frequency and duration of treatment were 24.2 J (20.0~45.0), 4 348 times (3 000~5 000) and 3.2 courses (1~7). All large common bile ductal stones were fragmented into stones with a dia-meter of less than 1cm. There were 2 patients who developed acute cholecystitis, 4 patients acute cholangitis and 2 patients hemobilia after ESWL. One patient was treated with percutaneous transhepatic gallbladder drainage for acute obstructive suppurative cholecystitis and the remaining patients were treated by medical treatment and biliary irrigation through the nasobiliary tube. The fragmented stones in the common bile ducts were removed by a second ERCP. There was no respiratory failure, heart failure, death or any other severe complications. Conclusion ERCP combined with ESWL was a safe and effective treatment for intractable choledocholithiasis with huge bile ductal stones in elderly patients. Key words: Endoscopic retrograde cholangiopancreatography; Extracorporeal shock wave lithotripsy; Choledocholithiasis; Pancreatic stent; Endoscopic nasobiliary drainage; Elderly patients

Salmonellal acute acalcular cholecystitis complicated by biliary candidal obstruction: An unusual presentation

Acute acalcular cholecystitis is a disease that mainly affects the immune compromised and severely ill patients. More often than not, the condition has a subtle presentation, yet it follows an aggressive course. Here, we present a case of acalcular salmonellal cholecystitis, which was further complicated by candidal biliary obstruction. Cholecystostomy and biliary irrigation with amphotericin B solution, in addition to systemic antifungal treatment, helped to resolve the grave situation.

IS P-5 Biliary irrigation resolves intractable cholestasis in a short bowel syndrome infant in whom the bile is not inspissated(International Poster 2,Science and Art for Sick Children)

IS P-5 Biliary irrigation resolves intractable cholestasis in a short bowel syndrome infant in whom the bile is not inspissated(International Poster 2,Science and Art for Sick Children)

Functional liver recovery parallels autologous gut salvage in short bowel syndrome

Background/purpose Parenteral nutrition (PN) is life saving in short bowel syndrome. However, long-term parenteral nutrition is frequently complicated by a syndrome of progressive cholestatic liver disease that is considered to be irreversible beyond the early stages of cholestasis, particularly in the presence of any degree of fibrosis in the liver. The purpose of this study was to examine apparent improvement in PN-associated liver dysfunction in a cohort of children with short bowel syndrome. Methods A retrospective case-record review of all patients managed within a dedicated Intestinal Rehabilitation Program (IRP) identified 13 patients with short bowel who had PN-associated liver dysfunction, defined for this purpose as hyperbilirubinemia or an abnormal liver biopsy. Results At referral, 12 of the 13 patients were exclusively on PN, and one was on 50% PN. At current follow-up, 3 patients have achieved complete enteral autonomy from PN, and 7 patients have had smaller decrements in PN requirements. Specific operative procedures to improve intestinal function were undertaken in 11 patients; 4 patients also underwent cholecystectomies with biliary irrigation at the time of intestinal reconstruction. The median highest bilirubin level in these 13 patients was 10.7 mg% (range, 3.2 to 24.5 mg%). Liver biopsy results indicated that 5 patients were cirrhotic, 3 had bridging fibrosis, and 4 had severe cholestasis or lesser degrees of fibrosis. Of 10 survivors in this series, 9 patients currently have a serum bilirubin less than 1 mg% with a median bilirubin in the group of 0.6 mg% (range, 0.3 to 6.4 mg%). Twelve of the 13 patients in this series were initially referred for liver-small bowel transplantation. Conclusions This preliminary experience suggests that PN-dependent patients with advanced liver dysfunction in the setting of the short bowel syndrome may, in some instances, experience functional and biochemical liver recovery. The latter appears to parallel autologous gut salvage in most cases. As a corollary, the authors believe that even advanced degrees of liver dysfunction should not preclude attempts at autologous gut salvage in very carefully selected patients. Such a policy of “aggressive conservatism” may help avoid the need for liver/intestinal transplantation in some patients who appear to be not responding to PN.

Parenteral nutrition associated cholestasis in the newborn

Parenteral nutrition associated cholestasis in preterm infants and newborn children is a frequent and serious disease with an incidence of 23% depended on duration of parenteral nutrition and birthweight. The incidence of liver cirrhosis is 40% when parenteral nutrition is given 74-242 days. The pathogenesis remains unclear. Several predisposing factors are discussed like immaturity, lack of hormonal stimulation by oral feeding, bacterial infection, liver toxicity of aminoacids and their products of photooxidation, lack of taurine, lack of antioxidation substances, hypermanganesaemia and pollution of infusion solutions. Furthermore sepsis during parenteral nutrition seems to multiply the risk of cholestasis. For prevention controlled studies recommend: 1. Early enteral nutrition. 2. The reduction of parenteral amino acids to less than 3 g/kg/d. 3. Light protection for parenteral solutions. 4. Cyclic infusion of parenteral nutrition. 5. The application of antibiotics (metronidazole, gentamicin) during parenteral nutrition. The most important therapeutic intervention is the beginning of oral feeding. Most of the time this leads to a decrease of icterus within two weeks. An icterus persisting longer than 3 weeks should be treated because of the risk of liver cirrhosis. Further therapeutic interventions are: 1. Cholecystokinin, good results in case studies which still has to be verified by a controlled study. 2. Ursodeoxycholic acid, its choleretic effectiveness is verified in several liver diseases by controlled studies, but it is not proven in parenteral nutrition associated cholestasis. 3. Laparoscopic biliary irrigation, successful in several case studies.

Biliary obstruction in infants with cystic fibrosis requiring Kasai portoenterostomy

Cystic fibrosis is associated with an inspissated bile syndrome producing cholestasis secondary to plugging of macroscopically normal bile ducts. In extreme neonatal forms, with early profound intrahepatic cholestasis, the process can be associated with a marked decrease in ductal diameter, varying from hypoplasia to atresia. From 1990 to 1995 three infants were identified with cystic fibrosis, persistent jaundice, and complete absence of biliary excretion despite expectant and conservative treatment including choleretics and surgical biliary irrigation. Abdominal ultrasounds showed contracted gallbladders and no evidence of dilated ducts. Liver biopsy results in two infants showed portal fibrosis, paucity of bile ducts, and minimal inflammation. The third infant had moderate inflammation, bile duct replication, and plugging. Two infants had undergone intestinal resection followed by hyperalimenation for complications of meconium ileus in the newborn period. Surgical exploration was undertaken at 7 to 12 weeks of age. Gross findings were typical of biliary atresia with microgallbladders and nonpatency of the cystic duct. Cholangiograms failed to document ductal patency in two patients who were then treated with a Kasai portoenterostomy. The third infant had patent hypoplastic ducts and underwent only biliary irrigation. Although bile flow was transiently achieved, jaundice recurred, and at reexploration at 16 weeks of age a Kasai poroenterostomy was performed. Histological review of the biliary specimens showed microscopically patent ducts in two patients and proximal patency and distal atresia in the third. All the ducts had mural fibrosis with cystic changes. Bile drainage was achieved in each instance, although in one patient with hypoplastic ducts scant output of highly concentrated bile proved insufficient to arrest progressive liver failure. The subsequent two patients responded with resolution of hyperbilirubinemia and normalization of liver function. They remain free of biliary complications at 30 and 40 months postoperatively. This manifestation of cystic fibrosis in infants is suggested by prolonged jaundice unresponsive to choleretics, nondilated bile ducts and gallbladder on ultrasound, absent biliary excretion on nuclear scan, and characteristic liver biopsy. Exploration is warranted, and discovery of atrophic bile ducts may be best managed with reconstruction.

Resolution of intractable cholestasis associated with total parenteral nutrition following biliary irrigation

Although the protracted use of total parenteral nutrition (TPN) in infants is known to predispose to cholestasis, which in certain instances may not be reversible, failure to understand the pathogenesis of this condition has hindered the development of an effective medical treatment. That surgical treatment may reverse this process in selected patients is the subject of the present report. Two premature infants and one term infant, with short bowel syndrome acquired in infancy, developed conjugated hyperbilirubinemia following institution of balanced TPN. Infectious and metabolic etiologies for the jaundice were ruled out. No excretion of 99mTc-labeled DISIDA from the biliary tree was demonstrable 24 hours following injection by nuclear scintigraphy, despite the absence of extrahepatic biliary obstruction by abdominal sonography. When all conventional medical methods of managing the problem failed, exploration was performed. Intraoperative cholangiography showed normal intrahepatic and extrahepatic biliary ducts in each instance. Irrigation of the biliary tree with normal saline solution was then performed. In all cases, liver biopsies were obtained that were consistent with TPN-associated cholestasis and in all cases, jaundice resolved promptly following operation. We conclude that intractable cholestasis associated with TPN, regardless of its etiology, is a functional abnormality of biliary excretion that may respond in selected patients to irrigation of the biliary tree with radiographic dye and/or normal saline solution.