Portoenterostomy, Hepatic
Biliary Atresia
Jaundice
Bile Ducts
Cholangitis
Liver Transplantation
Postoperative Complications
Liver
Paediatric liver transplantation: Queen Mary Hospital experience. (1/60)
OBJECTIVE: To assess the results of paediatric liver transplantation in our institution. METHODS: From September 1993 to November 1996, 10 living-related liver transplants (LRLT) and 3 reduced-size liver transplants (RSLT) were performed on 12 children at our hospital. The medical records of the patients were reviewed. All patients suffered from end-stage liver disease resulting from biliary atresia with failed Kasai's operations. Their ages at initial transplantation ranged from 8 months to 11 years. Excluding the 2 older children aged 7.5 and 11 years, the remaining patients were aged 10.5 months on the average and weighed 6 to 9.5 kg (mean: 6.8 kg) at the time of initial transplantation. RESULTS: All living donors were discharged on postoperative day 4 to 8 and resumed their previous normal activities. All recipients were alive with normal liver function and growing after a follow-up period of 3-40 months (mean: 21 months). The patient survival rate was 100%. One patient with RSLT had hepatitis of undetermined aetiology and underwent retransplant with a graft from her mother. The graft survival rate was 92%. Postoperative complications included: postoperative bleeding (n = 3), hepatic vein stenosis (n = I), biliary-enteric anastomotic stenosis (n = 3), intestinal perforation (n = I) and portal vein thrombosis (n = I). They were all treated promptly. In all patients, the hepatic artery (diameter ranged from 1.5 to 2.5 mm) anastomosis was achieved by microvascular technique. There was no hepatic artery thrombosis in our patients. CONCLUSION: With technical refinements, early detection and prompt treatment of complications, and advances in immunotherapy, excellent results can be achieved in paediatric liver transplantation. (+info)Jaundice clearance and cholangitis in the first year following portoenterostomy for biliary atresia. (2/60)
Sixty-one patients with biliary atresia, who underwent portoenterostomy (PE) between 1992 to 1998 in the Institute of Pediatrics, Kuala Lumpur and were followed for a period of one year, were studied to analyze the factors associated with jaundice clearance and cholangitis following PE. Sex distribution was equal. Majority of patients were Malays. Mean age in days at admission to the surgical ward was 66.90 +/- 23.36 and mean age at PE was 75.85 +/- 24.05. At the end of one-year follow-up, six patients (10%) had died, 35 (57%) developed one or more attacks of cholangitis, 35 (57%) had portal hypertension, eight (13%) liver failure and six patients had esophageal variceal bleeding. Thirty-three patients (54%) had jaundice clearance with a mean clearance time of 85 days after PE. The study shows that when the ductules in the porta hepatis were < 150 mu in size, persistence of jaundice after PE and the incidence of cholangitis in the first post-operative year were higher; patients with cholangitis in the first year had lower rate of jaundice clearance. Jaundice clearance was achieved in more patients when their postnatal age at the time of PE was lower but the relationship was not linear. Age at PE also did not have a linear temporal relationship to the incidence of cholangitis and the size of portal ductules. Prospective, multi-center based local studies on a bigger patient population are needed to identify other indicators of successful outcome following PE. This would help to define the indications for primary liver transplantation in the local population. (+info)Early complications after excision with hepaticoenterostomy for infants and children with choledochal cysts. (3/60)
OBJECTIVE: To retrospectively study the early complications of excision with hepaticoenterostomy for infants and children with choledochal cysts. METHODS: We analyzed 16 patients with early postoperative complications out of 173 patients with congenital choledochal cysts aged 27 days to 14 years (mean 2.4 years) who had undergone excisional procedures and biliary tract reconstruction. RESULTS: The early complications included bile leakage (10 patients), abdominal wall dehiscence (3), and hepatic failure, pancreatic juice leakage and postoperative intussusception (each in 1) respectively. Three patients died from bile leakage and 1 from postoperative hepatic failure. No statistical differences were observed between the procedures of biliary tract reconstruction with jejunal segment interposition hepaticoduodenostomy and Roux-en-Y hepaticojejunostomy (P>0.75). The morbidity was significantly higher in infants below 1 year than in children (P<0.005). Prevention and treatment of the complications were discussed. CONCLUSION: Bile leakage and abdominal wall dehiscence are major early postoperative complications. The morbidity of cholechal cysts is higher in infants than in children. Exploratory laparotomy should not be delayed when biliary leakage with diffuse peritonitis appears. The "tension suture in the fascial space of the abdominal wall" is useful to prevent and treat wound dehiscence. (+info)The influence of portoenterostomy on transplantation for biliary atresia. (4/60)
After portoenterostomy (PE) for biliary atresia (BA), many patients suffer progressive deterioration of liver function and ultimately require liver transplantation. We retrospectively reviewed a single center's experience with pediatric liver transplantation for BA from 1988 to 2002. Sixty-six patients underwent 69 liver transplants for BA. Forty-two (63%) patients had previously undergone Kasai PE, 11 (17%) biliary appendicoduodenostomy (BAD), and 13 (20%) had no prior biliary drainage (NBD). The BAD procedure offered only short-term biliary drainage--the mean interval between PE and transplant was more than twice that for Kasai patients than for BAD patients (132 versus 49 weeks). The transplants included 11 cadaveric partial, 27 cadaveric whole, and 31 living related transplants. Three patients required retransplant. Prior PE did not increase the incidence of major perioperative complications or unplanned reexploration. After transplant, the 1-, 5-, and 10-year actuarial graft survival rates were 87%, 86%, and 80%, respectively. The 1-, 5-, and 10-year actuarial patient survival rates were 91%, 89%, and 83%. PE remains an important bridge to transplant. In conclusion, transplantation for BA offers excellent long-term graft and patient survival. (+info)Diagnosis and treatment of biliary atresia: a retrospective study. (5/60)
BACKGROUND: Biliary atresia (BA) is the end results of an inflammatory process, which affect the intrahepatic and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tract with the development of biliary cirrhosis. Early diagnosis of BA is difficult, and there is no specific therapy for BA at present. The purpose of this study was to investigate the diagnosis, treatment and postoperative outcome of BA and to explore new diagnostic and therapeutic strategies. METHODS: Thirty-one children with biliary atresia from our hospital and 201 children with such disease from other hospitals in China were reviewed retrospectively in terms of diagnostic modalities, operative age, operative methods and long-term survival rates after operation. RESULTS: The operative age of the 31 patients varied from 13 to 270 days. It was <60 days in 6 children (19%), 61-90 days in 14 (45%), 91-120 days in 8 (26%), and >120 days in 3 (10%). No children underwent transplantation for BA. Their 1-6 year survival rate on average was 32.3% (10/31). The early operative rate in China was 12.9% (30/232). One child received liver transplantation with a long-term survival rate of 31.9%. CONCLUSIONS: Early recognition of babies with BA is critical for optimal intervention for preventing progressive fibrosis. Clinical presentation, imaging and laparoscopy are helpful in enhancing the early diagnostic rate of BA patients. The long-term survival rates can be obtained after the improvement of operative methods, liver transplantation for children with advanced BA, and development of other medical strategies. (+info)Liver transplantation for arteriohepatic dysplasia (Alagille's syndrome). (6/60)
Thirteen out of 268 children (less than 18 years old) underwent hepatic transplantation (OLT) for end-stage liver disease (ESLD) associated with arteriohepatic dysplasia (AHD). Seven children are alive and well with normal liver function. Six children died, four within 11 days of the operation and the other two at 4 and 10 months after the OLT. Vascular complications with associated septicemia were responsible for the deaths of three children. Two died of heart failure and circulatory collapse, secondary to pulmonary hypertension and congenital heart disease. The remaining patient died of overwhelming sepsis not associated with technical complications. Seven patients had a portoenterostomy or portocholecystostomy early in life; five of these died after the OLT. Severe cardiovascular abnormalities in some of our patients suggest that complete hemodynamic monitoring with invasive studies should be performed in all patients with AHD, especially in cases of documented hypertrophy of the right ventricle. The improved quality of life in our surviving patients confirms the validity of OLT as a treatment of choice in cases of ESLD due to AHD. (+info)Portoenterostomy scissors: a new instrument for surgery in the porta hepatis. (7/60)
A new design of scissors is described which has been found to be extremely useful during dissection of the porta hepatis. In particular its use is recommended for the portoenterostomy procedure of infancy (Kasai's operation). (+info)Extrahepatic biliary atresia: current concepts and future directions. (8/60)
OBJECTIVE: To provide an updated review on extrahepatic biliary atresia, focusing mainly on its etiopathogenesis, diagnosis, treatment and prognosis. SOURCES: MEDLINE and PubMed databases were searched using the following keywords: biliary atresia, etiopathogenesis, diagnosis, treatment, prognosis, children. SUMMARY OF THE FINDINGS: Extrahepatic biliary atresia is the main indication for liver transplantation among pediatric patients. As to its etiology, cytomegalovirus, reovirus and rotavirus have been widely investigated as possible triggers of the immunomediated obstruction of the biliary tree. The immune response, especially the predominant TH1 and interferon-gamma responses, genetic susceptibility and disorders related to the embryonic development of the biliary tree can play a role in the etiopathogenesis of extrahepatic biliary atresia. Yet today, portoenterostomy is the only available treatment, with better results when performed in the first 2 months of life. As to prognosis, all untreated children eventually die due to complications resulting from portal hypertension and liver cirrhosis, and most treated children have to undergo liver transplantation. CONCLUSIONS: Extrahepatic biliary atresia is still the major indication for pediatric liver transplantation, and to change this scenario some more light should be shed upon the etiopathogenesis of biliary atresia in different disease phenotypes. Future research into the role of interferon-gamma and of other cytokines is necessary in order to assess whether these aspects should be potential targets for therapeutic intervention. (+info)A hepatic portoenterostomy, also known as Kasai procedure, is a surgical operation performed on infants with extrahepatic biliary atresia. This condition is characterized by the absence or abnormal formation of the bile ducts that carry bile from the liver to the small intestine, leading to obstruction and damage to the liver.
During a hepatic portoenterostomy, the surgeon creates an anastomosis (connection) between the portal vein, which brings blood to the liver, and a loop of intestine. This connection allows bile to flow directly from the liver into the intestine, bypassing the blocked or absent bile ducts. The goal of the procedure is to restore bile flow and prevent further damage to the liver.
The success of the procedure varies, but it can help improve the child's quality of life and delay or prevent the need for a liver transplant in some cases. However, many children with biliary atresia will eventually require a liver transplant as the disease progresses.
Biliary atresia is a rare, progressive liver disease in infants and children, characterized by the inflammation, fibrosis, and obstruction of the bile ducts. This results in the impaired flow of bile from the liver to the intestine, leading to cholestasis (accumulation of bile in the liver), jaundice (yellowing of the skin and eyes), and eventually liver cirrhosis and failure if left untreated.
The exact cause of biliary atresia is not known, but it is believed to be a combination of genetic and environmental factors. It can occur as an isolated condition or in association with other congenital anomalies. The diagnosis of biliary atresia is typically made through imaging studies, such as ultrasound and cholangiography, and confirmed by liver biopsy.
The standard treatment for biliary atresia is a surgical procedure called the Kasai portoenterostomy, which aims to restore bile flow from the liver to the intestine. In this procedure, the damaged bile ducts are removed and replaced with a loop of intestine that is connected directly to the liver. The success of the Kasai procedure depends on several factors, including the age at diagnosis and surgery, the extent of liver damage, and the skill and experience of the surgeon.
Despite successful Kasai surgery, many children with biliary atresia will eventually develop cirrhosis and require liver transplantation. The prognosis for children with biliary atresia has improved significantly over the past few decades due to earlier diagnosis, advances in surgical techniques, and better postoperative care. However, it remains a challenging condition that requires close monitoring and multidisciplinary management by pediatric hepatologists, surgeons, and other healthcare professionals.
Jaundice is a medical condition characterized by the yellowing of the skin, sclera (whites of the eyes), and mucous membranes due to an excess of bilirubin in the bloodstream. Bilirubin is a yellow-orange pigment produced when hemoglobin from red blood cells is broken down. Normally, bilirubin is processed by the liver and excreted through bile into the digestive system. However, if there's an issue with bilirubin metabolism or elimination, it can accumulate in the body, leading to jaundice.
Jaundice can be a symptom of various underlying conditions, such as liver diseases (hepatitis, cirrhosis), gallbladder issues (gallstones, tumors), or blood disorders (hemolysis). It is essential to consult a healthcare professional if jaundice is observed, as it may indicate a severe health problem requiring prompt medical attention.
Bile ducts are tubular structures that carry bile from the liver to the gallbladder for storage or directly to the small intestine to aid in digestion. There are two types of bile ducts: intrahepatic and extrahepatic. Intrahepatic bile ducts are located within the liver and drain bile from liver cells, while extrahepatic bile ducts are outside the liver and include the common hepatic duct, cystic duct, and common bile duct. These ducts can become obstructed or inflamed, leading to various medical conditions such as cholestasis, cholecystitis, and gallstones.
Cholangitis is a medical condition characterized by inflammation of the bile ducts, which are the tubes that carry bile from the liver to the small intestine. Bile is a digestive juice produced by the liver that helps break down fats in food.
There are two types of cholangitis: acute and chronic. Acute cholangitis is a sudden and severe infection that can cause symptoms such as abdominal pain, fever, jaundice (yellowing of the skin and eyes), and dark urine. It is usually caused by a bacterial infection that enters the bile ducts through a blockage or obstruction.
Chronic cholangitis, on the other hand, is a long-term inflammation of the bile ducts that can lead to scarring and narrowing of the ducts. This can cause symptoms such as abdominal pain, itching, and jaundice. Chronic cholangitis can be caused by various factors, including primary sclerosing cholangitis (an autoimmune disease), bile duct stones, or tumors in the bile ducts.
Treatment for cholangitis depends on the underlying cause of the condition. Antibiotics may be used to treat bacterial infections, and surgery may be necessary to remove blockages or obstructions in the bile ducts. In some cases, medications may be prescribed to manage symptoms and prevent further complications.
The biliary tract is a system of ducts that transport bile from the liver to the gallbladder and then to the small intestine. Bile is a digestive fluid produced by the liver that helps in the breakdown and absorption of fats in the small intestine. The main components of the biliary tract are:
1. Intrahepatic bile ducts: These are the smaller branches of bile ducts located within the liver that collect bile from the liver cells or hepatocytes.
2. Gallbladder: A small pear-shaped organ located beneath the liver, which stores and concentrates bile received from the intrahepatic bile ducts. The gallbladder releases bile into the small intestine when food is ingested, particularly fats, to aid digestion.
3. Common hepatic duct: This is a duct that forms by the union of the right and left hepatic ducts, which carry bile from the right and left lobes of the liver, respectively.
4. Cystic duct: A short duct that connects the gallbladder to the common hepatic duct, forming the beginning of the common bile duct.
5. Common bile duct: This is a larger duct formed by the union of the common hepatic duct and the cystic duct. It carries bile from the liver and gallbladder into the small intestine.
6. Pancreatic duct: A separate duct that originates from the pancreas, a gland located near the liver and stomach. The pancreatic duct joins the common bile duct just before they both enter the duodenum, the first part of the small intestine.
7. Ampulla of Vater: This is the dilated portion where the common bile duct and the pancreatic duct join together and empty their contents into the duodenum through a shared opening called the papilla of Vater.
Disorders related to the biliary tract include gallstones, cholecystitis (inflammation of the gallbladder), bile duct stones, bile duct strictures or obstructions, and primary sclerosing cholangitis, among others.
Liver transplantation is a surgical procedure in which a diseased or failing liver is replaced with a healthy one from a deceased donor or, less commonly, a portion of a liver from a living donor. The goal of the procedure is to restore normal liver function and improve the patient's overall health and quality of life.
Liver transplantation may be recommended for individuals with end-stage liver disease, acute liver failure, certain genetic liver disorders, or liver cancers that cannot be treated effectively with other therapies. The procedure involves complex surgery to remove the diseased liver and implant the new one, followed by a period of recovery and close medical monitoring to ensure proper function and minimize the risk of complications.
The success of liver transplantation has improved significantly in recent years due to advances in surgical techniques, immunosuppressive medications, and post-transplant care. However, it remains a major operation with significant risks and challenges, including the need for lifelong immunosuppression to prevent rejection of the new liver, as well as potential complications such as infection, bleeding, and organ failure.
Postoperative complications refer to any unfavorable condition or event that occurs during the recovery period after a surgical procedure. These complications can vary in severity and may include, but are not limited to:
1. Infection: This can occur at the site of the incision or inside the body, such as pneumonia or urinary tract infection.
2. Bleeding: Excessive bleeding (hemorrhage) can lead to a drop in blood pressure and may require further surgical intervention.
3. Blood clots: These can form in the deep veins of the legs (deep vein thrombosis) and can potentially travel to the lungs (pulmonary embolism).
4. Wound dehiscence: This is when the surgical wound opens up, which can lead to infection and further complications.
5. Pulmonary issues: These include atelectasis (collapsed lung), pneumonia, or respiratory failure.
6. Cardiovascular problems: These include abnormal heart rhythms (arrhythmias), heart attack, or stroke.
7. Renal failure: This can occur due to various reasons such as dehydration, blood loss, or the use of certain medications.
8. Pain management issues: Inadequate pain control can lead to increased stress, anxiety, and decreased mobility.
9. Nausea and vomiting: These can be caused by anesthesia, opioid pain medication, or other factors.
10. Delirium: This is a state of confusion and disorientation that can occur in the elderly or those with certain medical conditions.
Prompt identification and management of these complications are crucial to ensure the best possible outcome for the patient.
The liver is a large, solid organ located in the upper right portion of the abdomen, beneath the diaphragm and above the stomach. It plays a vital role in several bodily functions, including:
1. Metabolism: The liver helps to metabolize carbohydrates, fats, and proteins from the food we eat into energy and nutrients that our bodies can use.
2. Detoxification: The liver detoxifies harmful substances in the body by breaking them down into less toxic forms or excreting them through bile.
3. Synthesis: The liver synthesizes important proteins, such as albumin and clotting factors, that are necessary for proper bodily function.
4. Storage: The liver stores glucose, vitamins, and minerals that can be released when the body needs them.
5. Bile production: The liver produces bile, a digestive juice that helps to break down fats in the small intestine.
6. Immune function: The liver plays a role in the immune system by filtering out bacteria and other harmful substances from the blood.
Overall, the liver is an essential organ that plays a critical role in maintaining overall health and well-being.
A newborn infant is a baby who is within the first 28 days of life. This period is also referred to as the neonatal period. Newborns require specialized care and attention due to their immature bodily systems and increased vulnerability to various health issues. They are closely monitored for signs of well-being, growth, and development during this critical time.
The hepatic artery is a branch of the celiac trunk or abdominal aorta that supplies oxygenated blood to the liver. It typically divides into two main branches, the right and left hepatic arteries, which further divide into smaller vessels to supply different regions of the liver. The hepatic artery also gives off branches to supply other organs such as the gallbladder, pancreas, and duodenum.
It's worth noting that there is significant variability in the anatomy of the hepatic artery, with some individuals having additional branches or variations in the origin of the vessel. This variability can have implications for surgical procedures involving the liver and surrounding organs.
List of MeSH codes (E04)