Stimulation of bile output by gastrointestinal hormones following portoenterostomy for biliary atresia (original) (raw)
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Percutaneus transhepatic cholangiodrainage after hepatic portoenterostomy for biliary atresia
Journal of Pediatric Surgery, 1980
During the past 12 mo, percutaneous transhepatic cholangiodrainage (PTCD) was carried out in 9 infants (12 procedures) who had undergone hepatic portoenterostomy (8) and hepatic portocholecystostomy (1) for biliary atresia. Bile excretion following surgery was observed in all infants. In all patients, cholangiogram at the initiation of PTCD was successful in visualizing the intrahepatic biliary system. PTCD was achieved in 5 infants (9 procedures). In infants with cholangitis, cultures of the intrahepatic bile were positive for enteric flora. Direct administration of antibiotics via the PTCD catheter into the bile ducts was transiently effective in the management of cholangitis. In one patient, it was observed that continuous PTCD over three weeks was effective in reducing serum bilirubin by relief of cholestasis. This technique is useful for: (1) demonstration of the reconstructed biliary system, (2) collection of bile from the intrahepatic biliary system for biochemical and bacteriologic studies, (3} direct administration of antibiotics to the bile ducts for cholangitis and (4) decompression of the intrahepatic system and relief of biliary stasis.
The future role of hepatic portoenterostomy as treatment of biliary atresia
Journal of Pediatric Surgery, 1992
9 According to our recent study, 38 of 93 patients (40.9%) who underwent portoenterostomy at Juntendo University Hospital between 1977 and 1986 survived for more than 5 years. In order to learn the future improvement of the prognosis of biliary atresia patients who undergo portoenterostomy, we investigated the relationship between the prognosis of biliary atresia patients and the age and the histological changes of liver at the time of surgery. Twentyseven of 30 patients (90%) who survived for more than 5 years with no jaundice and no findings of liver cirrhosis had a mild degree of liver fibrosis (FI or F2) and a mild degree of degeneration of intrahepatic bile ducts (B1 or B2) at the time of surgery, in spite of the difference of size of intrahepatic bile ducts at the porta hepatis. On the other hand, all 8 patients who showed severe liver fibrosis (F3) and severe degeneration of intrahepatic bile ducts (B3) did not survive for more than 5 years. Accordingly, we can expect promising prognoses in patients who undergo refined portoenterostomy procedures and receive proper postoperative treatment before they have severe histological changes of liver. The portoenterostomy will still play an important role as treatment of biliary atresia in the future.
[Factors related to the post-portoenterostomy prognosis of biliary atresia]
Jornal de pediatria
This study considered the presence of congenital anomalies, ductal plate malformation, area of fibrosis and, mainly, the patient's age in cases of biliary atresia submitted to surgery. The present study verified the influence of these factors on the follow-up of a biliary atresia sample. A sample of 47 patients with biliary atresia was evaluated in a cross-sectional study. Their histologic specimens were stained for antibody anticytokeratin 19 and CAM 5.2 through immunohistochemistry in order to study biliary structures, and for picrosirius red to evaluate the area of fibrosis. The study of biliary structures was performed by two pathologists and the first author of the study. They were "blind" with regard to the clinical follow-up. The area of fibrosis was quantitatively evaluated. Data on the patients with regard to age, death and occurrence of liver transplantation were searched on the patients records. Age at portoenterostomy varied between 24 and 251 days of life ...
Journal of Pediatric Surgery, 2006
Background/Purpose: Late-onset cholangitis is an uncommon complication after the Kasai operation for biliary atresia. Experience with 3 recent patients illustrates appropriate management. Methods: Retrospective review of patients with late unexplained deterioration in liver function attributed to cholangitis. All underwent quantitative radioisotope hepatobiliary scans to identify the Roux loop obstruction and confirm postoperative resolution. The role of percutaneous transhepatic cholangiography and computed tomography scanning was reviewed. Results: Three patients aged 8, 13, and 17 years were identified. All 3 had had a successful Kasai portoenterostomy for type 3 biliary atresia and presented after several virtually trouble-free years with late-onset cholangitis. The radioisotope scans in all 3 patients showed poor tracer uptake by the liver and pooling of the radioisotope at the porta hepatis. Laparotomy confirmed a proximal Roux loop obstruction at the level of the mesocolic window in all. This required mobilization and adhesiolysis and, in 1, correction of an actual stenosis within the wall of the intestine at this level. All patients had normalization of their liver function postoperatively. All are now well and anicteric at a follow-up of 6 to 12 months. Conclusions: Long-term survivors of the Kasai portoenterostomy for biliary atresia with immediate deterioration in liver function warrant investigation for possible Roux loop obstruction. Resolution of the obstruction allows preservation of their native liver.
Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy
Journal of Pediatric Surgery, 2006
Purpose: This study tests the hypothesis that steroid administration improves the outcome of biliary atresia (BA) by evaluating the efficacy of postoperative steroid use on surgical outcomes in infants with BA. Methods: Steroid use and outcomes in patients with BA were retrospectively analyzed at a tertiary pediatric hospital. Institutional review board approval was obtained. Results: Kasai portoenterostomy (PE) was performed in 43 patients with BA treated from 1992 to 2004 (16 boys and 27 girls). Twenty-one PE patients received steroids and 22 did not. Portoenterostomy was successful in 24 patients (55.8%) with consistent serum bilirubin less than 2 mg/dL. Sixteen (66%) received postoperative steroids. A normal postoperative bilirubin was achieved at 6 months in 16 (76%) of 21 patients with steroids compared with 8 (37%) of 22 in untreated controls (Fisher's Exact test, P = .01). Of the 43 patients, 19 (44%) required liver transplantation, including 7 (37%) of 19 with steroids vs 12 (63%) of 19 without ( P = .2). Twenty-eight infants developed cholangitis (fever with and without changes in hepatic function): 25 after PE and 3 after transplant. Of the 25, 12 (48%) received steroids. Seven died (16%) (range, 7 months to 4 years): 2 while awaiting transplantation (received steroids) and 5 after transplantation (1 received steroids and 4 were untreated). Survival was 86% (18/21) in patients with steroids and 82% (18/22) in those without. Transplant survival (74%) was comparable to previously reported historical controls (82%). Conclusions: The Kasai PE continues to be the procedure of choice in infants with BA younger than 3 months. A significantly improved clearance of postoperative jaundice and lower serum bilirubin levels were observed in patients receiving steroids. However, steroids had no effect on the incidence of cholangitis, need for liver transplantation, and overall survival. A prospective study with standardized dose and length of steroid administration and longer period of follow-up is necessary to more accurately assess the effectiveness of steroids after PE. D 2006 Elsevier Inc. All rights reserved.
Biliary atresia: Outcome and management
The Indian Journal of Pediatrics, 2006
Untreated, biliary atresia remains a fatal condition of the newborn. Most present within four to six weeks of conjugated jaundice and acholic stools, and, although still a challenging diagnosis to make, therein lies the opportunity of changing the course of this otherwise inexorable disease. The aim of surgery is to restore bile flow, alleviate jaundice and abbreviate the cholangiodestructive process within the liver. The Kasai portoenterostomy, introduced almost 50 years ago in Japan, aims to expose microscopic biliary ductules within the fibroinflammatory mass at the porta hepatis, and restore bile drainage into a mobilised Rou• loop. About 50% of infants with BA will be able to clear their jaundice following Kasai alone, given appropriately experienced surgeons and if performed prior to the onset of overt cirrhosis. They have a reasonable expectation of long-term survival to adulthood with a good quality-of-life. The remainder may be candidates for liver transplantation (where available) although donor organ shortage and immunosuppresion-related complications remain significant problems.
Current management of biliary atresia based on 35 years of experience at a single center
Clinics (Sao Paulo, Brazil), 2018
The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this...