Biliary tract abnormalities in patients with arteriohepatic dysplasia (original) (raw)
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Complete biliary obstruction without jaundice due to an anatomic variation
The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology, 2014
An aberrant right posterior hepatic duct is present in 4.8-8.4% of the population. It is one of the causes of bile duct injury during laparascopic cholecystectomy. Herein we present a patient with complete transection of the common hepatic duct during laparascopic cholecystectomy (Stewart-Way class 3). Interestingly, the patient had an intact aberrant right posterior duct draining into the common hepatic duct distal to the obstruction site that prevented early diagnosis of the biliary injury because of drainage of the liver sufficient to prevent the development of jaundice.
Journal of Pediatric and Neonatal Individualized Medicine, 2014
Extrahepatic biliary atresia (EHBA) refers to stenosis or atresia of the extrahepatic biliary tree. It accounts for 25-30% of cases of neonatal cholestasis. If left untreated, EHBA progresses to biliary cirrhosis and is universally fatal within the first 2 years of life. Early diagnosis is crucial since surgical treatment (Kasai procedure) is the only treatment option. Histopathologic examination of liver biopsy specimens is a key element in the diagnostic work-up of infants with suspected EHBA. Pathologic diagnosis aims at excluding non-surgically correctable causes of neonatal cholestasis thereby leading to surgical exploration for confirmation of the diagnosis. All published data indicate that pathologists can diagnose EHBA with high sensitivity, high specificity and reasonable interobserver agreement. The most useful histologic features in the diagnosis of EHBA are portal tract changes including ductular proliferation and bile plugs in ducts and ductules. These lesions are not pathognomonic but can be seen in extrahepatic obstruction of any cause. Total parenteral nutrition (TPN)associated cholestasis and alpha1-antitrypsin (A1AT) deficiency cannot be differentiated from EHBA without access to clinical data and may lead to false-positive diagnosis. False-negative interpretation may be caused by early age at diagnosis or by small/indequate specimens. The pathologist also plays a role in the examination of the resected fibrotic segment and Review
Low convergence of hepatic ducts: A rare extrahepatic biliary tree anatomical variation
International Journal of Hepatobiliary and Pancreatic Diseases, 2015
Introduction: Low convergence of hepatic ducts is extremely rare. Here, the right and the left hepatic ducts course down towards the second part of the duodenum separately and converge just proximal to the ampulla of Vater, forming a short common bile duct. case series: We describe 2 cases of a 43-year-old male and a 53-yearold female with this anatomical anomaly who presented with obstructive jaundice. Endoscopic retrograde cholangiopancreatography (ErcP) demonstrated low convergence of the hepatic ducts. both cases were associated with biliary ectasia and complicated with primary ductal stones. Excision of the anomalous extrahepatic bile ducts with hepaticoenterostomy was performed for one of the cases, however the other was lost to follow-up. conclusion: Definitive operative intervention seems prudent despite
An Unusual Cause of Cholestasis in an Infant: Biliary Atresia Type IIB
Indian Journal of Radiology and Imaging
Biliary atresia (BA) is a progressive destructive cholangiopathy of unknown etiology that presents in early infancy. It has a worldwide frequency of 1:8,000–1:15,000 and is common in Asia than in the west. Based on the level at which the lumen of the extrahepatic duct is obliterated, BA is classified into three types. Type III is the commonest (∼85%) type and has the most proximal level of obstruction in the porta hepatis, while type II in which the atresia is at the level of the common hepatic duct, is the least common (∼2.5%) and has been rarely reported. Here, we report the imaging features of an infant with type IIB biliary atresia.
HPB Surgery, 1991
Biliary tract abnormalities occur in about one of every three people, usually being minor and of no clinical significance.Major abnormalities, however, may present in an unusual manner and provide a major hazard to the unsuspecting surgeon.A patient presenting with cholangitis without jaundice or abnormal liver function tests is reported. Endoscopic retrograde cholangiography failed to demonstrate any bile ducts in the right postero-lateral segments of the liver, the “naked segment sign”. A percutaneous transhepatic cholangiogram demonstrated a stricture obstructing the right posterior segmental hepatic duct with hepatolithiasis above the stricture. At operation an anomalous vessel was found at the site of the stricture.This case highlights the unusual way in which biliary tract anomalies may present and the importance of adequate pre-operative investigation.
Journal of Translational Medicine and Research, 2016
Background: In the current era, the laparoscopic procedure such as laparoscopic cholecystectomy, laparoscopic hepatobiliary surgery and other open procedures such as open cholecystectomy, biliary stricture surgery, are performed regularly throughout the world and extrahepatic biliary tract is one of the most common sites of the surgical procedures. The incidence of biliary tract injury by laparoscopic cholecystectomy has been found to be higher than open cholecystectomy. Apart from various other causes of biliary injuries aberrant anatomical course of extrahepatic biliary system is a well established fact of iatrogenic ductal injury. Thus, an adequate recognition and awareness of anatomical abnormalities of extra hepatic biliary tree with its vessel, can decrease the morbidity and mortality related to the surgery. Methods: Study was done in Department of Surgery, Gandhi Medical College and Hamidia Hospital Bhopal, India on 100 cases, during period of Aug 2014 to Nov 2015, and dissection was carried out in department of Forensic Medicine and Toxicology after taking permission from ethical committee. Results: In 100 cases of study 72 were male and 28 were female in which 16% male and 10.7% female showed variations in their anatomy. The most common variation which we observed in our study was short cystic duct in 8 cases, and second most common variations was cystic artery origin, from left hepatic artery in 3 cases and from proper hepatic artery in 1 case, other variations were floating gall bladder in 1 case, intrahepatic union of left hepatic duct and right hepatic duct in 3 cases, low insertion of cystic duct in 3 cases, high insertion of cystic duct to common hepatic duct in 1 case,and in one case cystic artery passing anterior to common hepatic duct. Conclusion: There was a significant variations seen in extrahepatic biliary apparatus and its related arterial supply in our study, and these variations observed could definintely be useful to hepatobiliary, laparoscopic surgeons and radiologist. And will further contribute to literature available on variations of extrahepatic biliary system.
International journal of contemporary pediatrics, 2024
Infants with BA are typically at-term, normal-weight, and thriving. Early signs include dark urine, acholic stools, jaundice, high serum bile salts, and conjugated bilirubin, possibly indicating cholestasis. 2 Gamma-glutamyl transferase (GGT) levels and matrix metalloproteinase-7 (MMP-7) are crucial in diagnosing BA. 3 BA is divided into perinatal or non-syndromic cases, accounting for over twothirds, and embryonic or syndromic cases, more common with multiple anomalies. 4 Genetically predisposed newborns may develop BA due to in-utero insults, triggering immunity, bile duct injury, bile acid buildup, and epithelial destruction, and a viral trigger is a plausible etiological factor. 5,6 Abdominal ultrasound can detect gallbladder visualization and liver hilum cysts, but magnetic resonance imaging (MRI)-cholangiography is insufficient for infants under 3 months. Endoscopic retrograde cholangiopancreatography (ERCP) and liver biopsy may be helpful. 1 BA is primarily ABSTRACT Biliary atresia is a neonatal onset, obstructive cholangiopathy of the intrahepatic or extrahepatic biliary system, leading to the build-up of bile in the liver. This case discusses a one-month-old infant who was previously diagnosed with jaundice, presenting the symptoms of yellowish eyes and skin, pale stools, and palpable liver. The infant and her mother's liver enzyme levels were found to be higher and her histopathology studies evidenced atretic gall bladder. The liver biopsy revealed mild periportal ductular reaction and diffuse hepatocanalicular cholestasis. She was diagnosed with neonatal cholestasis-extrahepatic biliary atresia (EHBA) and underwent Kasai-portoenterostomy. She was stable and discharged with gallstone dissolution agents, antibiotics, vitamin supplements, and a barbiturates-liver enzyme inducer. After six months, she presented hepatosplenomegaly with ascites and was found to have transaminitis. She was then diagnosed with EHBA-failed Kasai, probable cholangitis, and planned for living donor liver transplantation. Antibiotics, antiviral, antifungal, anticoagulants, and immunosuppressants were prescribed on discharge. The Doppler study of allograft was performed to check the blood flow after transplantation. Acute graft rejection was monitored on day 5 with liver transplant pack reports. One year later, the infant's condition had shown improvement, evidenced by enhanced food intake, absence of symptoms, and the return of liver function tests to normal levels.
2003
Bile duct injuries have been recorded with open cholecystectomy (OC) long before the introduction of laparoscopic cholecystectomy (LC) (Lillemoe et al, 2000). The introduction of OC by Langenbuch of Berlin in 1882 brought about the world wide use of OC for the management of symptomatic gall stones. After that, injuries to the biliary tree became a recognized complication of operation (Roslyn et al, 1993). Over the last decade, the incidence of bile duct injuries after OC has been estimated at 0.1% to 0.3% (Edmond and Marvin, 2001).