目的探讨腹腔镜下行肝门空肠吻合术治疗先天性胆道闭锁的效果。方法2003年8月～2005年9月，对26例胆道闭锁（Ⅲ型）患儿在腹腔镜下行肝门空肠吻合术，充分显露肝门，游离并于肝门纤维块处切除胆囊，提起距Treitz韧带20cm处空肠，经脐将...目的探讨腹腔镜下行肝门空肠吻合术治疗先天性胆道闭锁的效果。方法2003年8月～2005年9月，对26例胆道闭锁（Ⅲ型）患儿在腹腔镜下行肝门空肠吻合术，充分显露肝门，游离并于肝门纤维块处切除胆囊，提起距Treitz韧带20cm处空肠，经脐将空肠提出腹壁外并逐渐拉出远端40cm范围，距Treitz韧带20cm横断空肠并封闭远端，将近端与远侧30～35cm处空肠行端侧吻合，把肠管送回腹腔。将肝支空肠襻经结肠后隧道上提至肝下，肝门空肠端侧吻合。与同期同年龄段34例胆道闭锁（Ⅲ型）行开腹肝门空肠吻合术的患儿进行对照，比较两组手术时间、术中出血量、手术前后肝功能指标、平均住院天数和住院费用及术后转归情况。结果腹腔镜组手术出血量少于开腹组[（15．4±5．0）ml vs（33．8±19．4）ml，t=-4．709，P=0．000]，住院费用高于开腹组[（19153．9±619．5）元 vs （15116．7±898．4）元，t=19．607，P=0．000]；两组手术时间、住院天数无显著差异。术后腹腔镜组血清总胆红素、直接胆红素、ALT和AST比开腹组下降明显。腹腔镜组术后切口疝1例；开腹组急性肝衰竭、伤口裂开各1例，伤口感染2例，两组早期并发症发生率无显著性差异（χ^2=0．395，P=0．530）。术后4个月时随访，腹腔镜组13例（占50％）患儿退黄；开腹组18例（53％）患儿退黄。结论腹腔镜肝门空肠吻合术治疗小儿先天性胆道闭锁微创效果明显，方法安全、有效。展开更多
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 o...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 post operative 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 with in 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 biliaryatresia with immediate deterioration in liver function warrant investigation for possible Roux loop obstruction. Resolution of the obstruction allows preservation of their native liver.展开更多
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. Method...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.展开更多
Background/Purpose: The aim of this paper was to investigate the mechanism of long-term biliary drainage after Kasai portoenterostomy by clinicopathologic study of hepatic morphology in explanted livers.Methods: Expla...Background/Purpose: The aim of this paper was to investigate the mechanism of long-term biliary drainage after Kasai portoenterostomy by clinicopathologic study of hepatic morphology in explanted livers.Methods: Explanted livers from 13 consecutive children undergoing transplantation for biliary atresia were examined in detail using a standardized protocol.Group 1 (n = 6) had no Kasai procedure before transplantation at a median age of 8 m.Group 2 (n = 4) were transplanted at a median age of 10 m after a failed Kasai portoenterostomy.Group 3 (n = 3) had a successful Kasai but required transplantation for complications of chronic liver disease at 12-14 years.Pathology findings were correlated with hepatic morphology determined by pretransplant magnetic resonance imaging.Results: Large perihilar regenerative nodules (8-14 cm diameter) were observed in 2 patients after successful Kasai portoenterostomy, less well-de-fined perihilar nodules in group 2 patients, and no regenerative nodules in group 1.Microscopically, group 1 had diffuse biliary cirrhosis with evidence of progressive ductopenia during infancy.In group 2, perihilar regenerative nodules showed variable portal fibrosis but no cirrhosis and bile ducts were present with 68%-100%of hepatic arteries; in peripheral cirrhotic areas, bile ducts were absent in patients older than 9 m.The perihilar regenerative nodules in group 3 patients had a noncirrhotic architecture with preserved bile ducts, but the peripheral parenchyma was cirrhotic; one patient had diffuse macronodular cirrhosis.These morphologic findings correlated well with magnetic resonance images, highlighting the preservation of relatively normal perihilar liver architecture after successful Kasai portoenterostomy.Conclusions: Unoperated biliary atresia is associated with progressive intrahepatic ductopenia leading to diffuse biliary cirrhosis.Kasai portoenterostomy can result in the growth of large perihilar regenerative nodules, probably as a consequence of surviving intrahepatic ducts in this region.In some patients, long-term success after Kasai portoenterostomy may depend on hyperplasia of the perihilar liver.展开更多
We present a case report of a boy with biliary atresia who,after hepatoportoenterostomy performed on day 21 of life, had immediate resolution of cholestasis and remained anicteric until 3.5 months of age. He then abru...We present a case report of a boy with biliary atresia who,after hepatoportoenterostomy performed on day 21 of life, had immediate resolution of cholestasis and remained anicteric until 3.5 months of age. He then abruptly developed acholic stools.Nuclear medicine imaging study showed no excretion. Broadspectrum antibiotics and corticosteroids were administered but did not lead to clinical improvement; a surgical revision of the original anastomosis was undertaken at 4 months of age. At 14 months of age, the child is anicteric and growing well. In this case, successful revision of hepatoportoenterostomy averted the need for liver transplantation.展开更多
We modified conventional hepatic portoenterostomy by pulling the remnant fibrous mass caudally and placing 5 or 6 numbered and individually clamped absorbable 5- 0 double- needle sutures horizontally in the liver surf...We modified conventional hepatic portoenterostomy by pulling the remnant fibrous mass caudally and placing 5 or 6 numbered and individually clamped absorbable 5- 0 double- needle sutures horizontally in the liver surface of the posterior side of the remnant fibrous mass before excision and apposing the posterior and anterior margins of the hepatic portoenterostomy to resemble a flattened isosceles triangle. Our technique was used to treat 14 cases of biliary atresia. Good postoperative biliary excretion (serum total bilirubin, < 2 mg/dL) was achieved in 13 (92.9% ) of 14 patients.展开更多
Purpose: Prednisolone is used routinely after portoenterostomy (PE) in patients with biliary atresia (BA).The authors reviewed their patients with BA post-PE to assess prednisolone protocols.Methods: Severity of fibro...Purpose: Prednisolone is used routinely after portoenterostomy (PE) in patients with biliary atresia (BA).The authors reviewed their patients with BA post-PE to assess prednisolone protocols.Methods: Severity of fibrosis at PE (moderate or severe), age at PE (30-70 days), size of bile ductules in the fibrotic biliary remnant at the porta hepatis (>100 μm), and type of BA (uncorrectable type) were used as criteria for selecting 63 subjects from our patients with BA post-PE.Subjects were divided into 5 groups according to prednisolone dosage: group 1, no prednisolone; groups 2 to 4, single courses of intravenous prednisolone commencing on day 7 post-PE administered in decreasing dose for 3 days each as follows: group 2, 6, 4, and 2 mg; group 3, 10, 5, and 2.5 mg; group 4, 20, 15, 10, 5, and 2.5 mg; group 5, same as group 4, but stool color was used to monitor bile excretion and a course was restarted from 20 mg whenever stools began to turn pale.If necessary, single courses were repeated until serum total bilirubin was less than 2.0 mg/dL.Protocol efficacy was assessed by comparing the number of patients who became jaundice free, the period taken to become jaundice free, and the incidence of side effects related to prednisolone.Results: The number of patients who became jaundice free in the no prednisolone group (group 1, 7/12 or 58.3%) was not significantly different from the number in the single-course groups (group 2, 8/12 or 66.6%; group 3, 10/13 or 76.9%; and group 4, 11/15 or 73.3%).The number in the stool-monitored group (group 5, 10/11 or 90.9%) was significantly greater (P <.05).The mean period taken to become jaundice free in group 1 (82.6 ±29.1 days) was not significantly different from the single-course groups (group 2, 74.5 ±29.3 days; group 3, 49.6 ±19.8 days; and group 4, 48.3 ±26.0 days).The mean period taken in the stool-monitored group (group 5, 33.3 ±6.4 days) was significantly shorter (P <.05).The number of subjects who developed cholangitis after becoming jaundice free was not significantly different (group 1, 2/7; group 2, 2/8; group 3, 2/10; group 4, 2/11; group 5, 2/10).There were no prednisolone-related complications identified in any subject.Conclusions: These results provide strong evidence that large-dose prednisolone therapy with stool color monitoring of bile flow has a positive impact on the time taken for patients with BA post-PE to become jaundice free and the number of patients who remain jaundice free.展开更多
文摘目的探讨腹腔镜下行肝门空肠吻合术治疗先天性胆道闭锁的效果。方法2003年8月～2005年9月，对26例胆道闭锁（Ⅲ型）患儿在腹腔镜下行肝门空肠吻合术，充分显露肝门，游离并于肝门纤维块处切除胆囊，提起距Treitz韧带20cm处空肠，经脐将空肠提出腹壁外并逐渐拉出远端40cm范围，距Treitz韧带20cm横断空肠并封闭远端，将近端与远侧30～35cm处空肠行端侧吻合，把肠管送回腹腔。将肝支空肠襻经结肠后隧道上提至肝下，肝门空肠端侧吻合。与同期同年龄段34例胆道闭锁（Ⅲ型）行开腹肝门空肠吻合术的患儿进行对照，比较两组手术时间、术中出血量、手术前后肝功能指标、平均住院天数和住院费用及术后转归情况。结果腹腔镜组手术出血量少于开腹组[（15．4±5．0）ml vs（33．8±19．4）ml，t=-4．709，P=0．000]，住院费用高于开腹组[（19153．9±619．5）元 vs （15116．7±898．4）元，t=19．607，P=0．000]；两组手术时间、住院天数无显著差异。术后腹腔镜组血清总胆红素、直接胆红素、ALT和AST比开腹组下降明显。腹腔镜组术后切口疝1例；开腹组急性肝衰竭、伤口裂开各1例，伤口感染2例，两组早期并发症发生率无显著性差异（χ^2=0．395，P=0．530）。术后4个月时随访，腹腔镜组13例（占50％）患儿退黄；开腹组18例（53％）患儿退黄。结论腹腔镜肝门空肠吻合术治疗小儿先天性胆道闭锁微创效果明显，方法安全、有效。
文摘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 post operative 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 with in 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 biliaryatresia with immediate deterioration in liver function warrant investigation for possible Roux loop obstruction. Resolution of the obstruction allows preservation of their native liver.
文摘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.
文摘Background/Purpose: The aim of this paper was to investigate the mechanism of long-term biliary drainage after Kasai portoenterostomy by clinicopathologic study of hepatic morphology in explanted livers.Methods: Explanted livers from 13 consecutive children undergoing transplantation for biliary atresia were examined in detail using a standardized protocol.Group 1 (n = 6) had no Kasai procedure before transplantation at a median age of 8 m.Group 2 (n = 4) were transplanted at a median age of 10 m after a failed Kasai portoenterostomy.Group 3 (n = 3) had a successful Kasai but required transplantation for complications of chronic liver disease at 12-14 years.Pathology findings were correlated with hepatic morphology determined by pretransplant magnetic resonance imaging.Results: Large perihilar regenerative nodules (8-14 cm diameter) were observed in 2 patients after successful Kasai portoenterostomy, less well-de-fined perihilar nodules in group 2 patients, and no regenerative nodules in group 1.Microscopically, group 1 had diffuse biliary cirrhosis with evidence of progressive ductopenia during infancy.In group 2, perihilar regenerative nodules showed variable portal fibrosis but no cirrhosis and bile ducts were present with 68%-100%of hepatic arteries; in peripheral cirrhotic areas, bile ducts were absent in patients older than 9 m.The perihilar regenerative nodules in group 3 patients had a noncirrhotic architecture with preserved bile ducts, but the peripheral parenchyma was cirrhotic; one patient had diffuse macronodular cirrhosis.These morphologic findings correlated well with magnetic resonance images, highlighting the preservation of relatively normal perihilar liver architecture after successful Kasai portoenterostomy.Conclusions: Unoperated biliary atresia is associated with progressive intrahepatic ductopenia leading to diffuse biliary cirrhosis.Kasai portoenterostomy can result in the growth of large perihilar regenerative nodules, probably as a consequence of surviving intrahepatic ducts in this region.In some patients, long-term success after Kasai portoenterostomy may depend on hyperplasia of the perihilar liver.
文摘We present a case report of a boy with biliary atresia who,after hepatoportoenterostomy performed on day 21 of life, had immediate resolution of cholestasis and remained anicteric until 3.5 months of age. He then abruptly developed acholic stools.Nuclear medicine imaging study showed no excretion. Broadspectrum antibiotics and corticosteroids were administered but did not lead to clinical improvement; a surgical revision of the original anastomosis was undertaken at 4 months of age. At 14 months of age, the child is anicteric and growing well. In this case, successful revision of hepatoportoenterostomy averted the need for liver transplantation.
文摘We modified conventional hepatic portoenterostomy by pulling the remnant fibrous mass caudally and placing 5 or 6 numbered and individually clamped absorbable 5- 0 double- needle sutures horizontally in the liver surface of the posterior side of the remnant fibrous mass before excision and apposing the posterior and anterior margins of the hepatic portoenterostomy to resemble a flattened isosceles triangle. Our technique was used to treat 14 cases of biliary atresia. Good postoperative biliary excretion (serum total bilirubin, < 2 mg/dL) was achieved in 13 (92.9% ) of 14 patients.
文摘Purpose: Prednisolone is used routinely after portoenterostomy (PE) in patients with biliary atresia (BA).The authors reviewed their patients with BA post-PE to assess prednisolone protocols.Methods: Severity of fibrosis at PE (moderate or severe), age at PE (30-70 days), size of bile ductules in the fibrotic biliary remnant at the porta hepatis (>100 μm), and type of BA (uncorrectable type) were used as criteria for selecting 63 subjects from our patients with BA post-PE.Subjects were divided into 5 groups according to prednisolone dosage: group 1, no prednisolone; groups 2 to 4, single courses of intravenous prednisolone commencing on day 7 post-PE administered in decreasing dose for 3 days each as follows: group 2, 6, 4, and 2 mg; group 3, 10, 5, and 2.5 mg; group 4, 20, 15, 10, 5, and 2.5 mg; group 5, same as group 4, but stool color was used to monitor bile excretion and a course was restarted from 20 mg whenever stools began to turn pale.If necessary, single courses were repeated until serum total bilirubin was less than 2.0 mg/dL.Protocol efficacy was assessed by comparing the number of patients who became jaundice free, the period taken to become jaundice free, and the incidence of side effects related to prednisolone.Results: The number of patients who became jaundice free in the no prednisolone group (group 1, 7/12 or 58.3%) was not significantly different from the number in the single-course groups (group 2, 8/12 or 66.6%; group 3, 10/13 or 76.9%; and group 4, 11/15 or 73.3%).The number in the stool-monitored group (group 5, 10/11 or 90.9%) was significantly greater (P <.05).The mean period taken to become jaundice free in group 1 (82.6 ±29.1 days) was not significantly different from the single-course groups (group 2, 74.5 ±29.3 days; group 3, 49.6 ±19.8 days; and group 4, 48.3 ±26.0 days).The mean period taken in the stool-monitored group (group 5, 33.3 ±6.4 days) was significantly shorter (P <.05).The number of subjects who developed cholangitis after becoming jaundice free was not significantly different (group 1, 2/7; group 2, 2/8; group 3, 2/10; group 4, 2/11; group 5, 2/10).There were no prednisolone-related complications identified in any subject.Conclusions: These results provide strong evidence that large-dose prednisolone therapy with stool color monitoring of bile flow has a positive impact on the time taken for patients with BA post-PE to become jaundice free and the number of patients who remain jaundice free.
