In vitro fertilization: an unexpected finding in a cohort of patients with biliary atresia

In biliary atresia (BA), efforts to prevent premature liver transplantation (LT) are aimed at early diagnosis, timing of Kasai-portoenterostomy (KPE), and centralization of care. This report presents the clinical picture, treatment strategies, and outcomes of BA patients with no previous treatment. A retrospective cohort study (Jan/2001 to Jan/2021) was conducted to evaluate the outcome of patients with BA referred to a single team. Study groups were: 1) Kasai-only group (K-only) n=9), 2) LT-only group (n=7), and 3) Kasai+LT group (K+LT) (n=23). Survival with native liver and overall survival were 22.9 and 94.8%, respectively, at 120 months of follow-up. There was no difference in age at KPE in the K-only group (46.8±21.8 days) vs K+LT (52.1±22 days), P=0.4. Ten (25.6%) patients were babies conceived through in vitro fertilization (IVF). Four IVF patients (40%) presented associated congenital heart disease vs 5 patients (17%) in the remaining group (P=0.14). Two of the IVF patients were premature (<37 weeks). Median maternal age at birth was 35 years (33 to 41 years). Excellent patient survival is expected for patients with BA with the available treatment strategies. IVF+BA was an unexpected prevalent association in this cohort, and further studies are required to better understand these findings.

To compare the different hepatic portal exposure techniques in Kasai surgery during their different perioperative periods / 中华肝胆外科杂志

Objective:To compare the impact of different portal exposure techniques in the Kasai surgery on children with type Ⅲ. biliary atresia during their different perioperative periods.Methods:A retrospective study was performed on the data of children with type Ⅲ. biliary atresia who underwent Kasai surgery at Fujian Children's Hospital from January 2017 to October 2020. Of 45 children enrolled in this study, there were 24 males and 21 females, aged (71.3±21.0) days. Patients who had left and right branches of the portal vein and the left and right hepatic arteries in the portal area being completely freed and elastically stretched during the Kasai operation were included into the free group ( n=22) and the remaining patients were included in the control group ( n=23). Postoperative hospital stay, postoperative direct bilirubin levels, postoperative complications and transplant-free survival after the Kasai operation were compared between the 2 groups. Results:Postoperative hospital stay of (17.1±4.4) d in the free group was significantly lower than that in the control group (20.1±5.4) d, ( t=2.07, P=0.044). The direct bilirubin level at 3 months after surgery for the control group was 30.0 (109, 108.0)μmol/L, which was significantly higher than that of 14.5 (4.0, 37.5) μmol/L in the free group ( Z=-2.16, P=0.031). Twenty-one patients (91.3%) in the control group had frequent attacks of postoperative cholangitis, compared with 13 patients (59.1%) in the free group. The difference was statistically significant (χ 2=4.69, P=0.030). Eleven surviving patients (47.8%) in the control group did not undergo liver transplantation at one year after surgery, compared with 15 patients (68.2%) in the free group. At two years after surgery, 7 surviving patients (30.4%) in the control group did not undergo liver transplantation compared with 10 patients (45.5%) in the free group. Conclusion:For children with type Ⅲ. biliary atresia, completely freeing the left and right branches of portal vein, and left and right hepatic arteries in the liver portal area, and elastically stretching these vessels to expose the portal area of the liver during Kasai surgery increased surgical safety and reduced hospital stay.

Pediatric liver transplantation for Alagille syndrome: a single-center experience / 器官移植

Objective To evaluate the clinical efficacy of liver transplantation in children with Alagille syndrome (ALGS). Methods Clinical data of 12 children with ALGS were collected and retrospectively analyzed. Clinical characteristics of children with ALGS, pathological characteristics of liver tissues, characteristics of liver transplantation, postoperative complications and follow-up of children with ALGS were analyzed. Results JAG1 gene mutation and typical facial features was present in all 12 children. Jaundice was the most common initial symptom, which occurred at 7 (3, 40) d after birth. Upon liver transplantation, the Z scores of height and body weight were calculated as -2.14 (-3.11, -1.83) and -2.32 (-3.12, -1.12). Five children developed severe growth retardation and 4 children with severe malnutrition. Eight of 12 children were diagnosed with cardiovascular abnormalities. Pathological examination showed that the lobular structure of the diseased livers of 4 children was basically maintained, and 8 cases of nodular liver cirrhosis in different sizes including 1 case of single early moderately-differentiated hepatocellular carcinoma. Three children were misdiagnosed with biliary atresia and underwent Kasai portoenterostomy. Eight children underwent living donor liver transplantation, three children underwent cadaveric donor liver transplantation (two cases of split liver transplantation and one case of cadaveric total liver transplantation), and one child underwent domino liver transplantation (donor liver was derived from a patient with maple syrup urine disease). during the follow-up of 30.0(24.5, 41.7) months, the survival rates of the children and liver grafts were both 100%. During postoperative follow-up, the Z scores of height and body weight were calculated as -1.24 (-2.11, 0.60) and -0.83 (-1.65, -0.43), indicating that the growth and development of the children were significantly improved after operation. Conclusions Liver transplantation is an efficacious treatment for children with ALGS complicated with decompensated cirrhosis, severe itching and poor quality of life. For children with ALGS complicated with cardiovascular abnormalities, explicit preoperative evaluation should be delivered, and consultation with pediatric cardiologists should be performed if necessary.

Analysis of risk factors of postoperative biliary leakage in patients with perihilar cholangiocarcinoma / 中华肝胆外科杂志

Objective:To analyse the risk factors of biliary leakage after surgical resection in patients with perihilar cholangiocarcinoma (PHCC).Methods:The medical data on 179 patients who underwent surgical resection for PHCC at the Department of Hepatopancreatobiliary Surgery, Ningbo Medical Centre Lihuili Hospital, Ningbo University from April 2000 to April 2020 were collected, and 160 patients were finally enrolled into this study. There were 86 males and 74 females, aged (63.4±10.8) years. The 44 patients with class B biliary leakage and the 5 patients with class C biliary leakage were classified into the biliary leakage group, while the remaining 111 patients were classified into the control group. Risk factors of biliary leakage were analysed by univariate and multivariate logistic regression analyses.Results:Operation time ≥360 min, resection and reconstruction of hepatic hilar vessels on the preserved side of liver and number of bile duct openings of >3 in remnant liver were significantly higher in the biliary leakage than the control group (all P<0.05). Multivariate analysis showed that resection and reconstruction of hepatic hilar vessels on the preserved side ( OR=2.322, 95% CI: 1.078-5.002, P=0.028) and 3 or more bile duct openings in the remnant liver ( OR=2.656, 95% CI: 1.198-5.892, P=0.016) were significantly associated with biliary leakage. Conclusion:Resection and reconstruction of hepatic hilar vessels on the preserved side of liver and 3 or more bile duct openings in remnant liver were independent risk factors for biliary leakage after PHCC resection.

Efficacy of percutaneous jejunal drainage in treatment of hepatolithiasis after choledochojejunostomy / 中华肝胆外科杂志

Objective:To study the feasibility and safety of percutaneous jejunal drainage in treatment of hepatolithiasis after choledochojejunostomy.Methods:The clinical data of 24 patients with hepatolithiasis after choledochojejunostomy treated by percutaneous jejunal drainage at the Cholelithiasis Center, Shanghai East Hospital Affiliated to Tongji University from May 2021 to May 2022 were retrospectively analyzed. There were 16 males and 8 females, aged (50.46±10.89) years old. Ultrasound and X ray guided percutaneous jejunography was performed under local anesthesia for patients with hepatolithiasis after choledochojejunostomy. Then the fistula was directly dilated to 16.0Fr, and percutaneous jejunal choledochoscopy was performed 3 days later. The success rate, complication rate and stone removal rate were analysed.Results:Twenty-four patients were treated with percutaneous jejunography, with a success rate of 79.2%(19/24), including 19 patients after anterior colonic cholangiojejunostomy with a success rate of 94.7%(18/19), and 5 patients after retrocolonic cholangiojejunostomy with a success rate of 20.0%(1/5). There was no complication including bleeding, intestinal leakage and bile leakage. In 19 patients with successful percutaneous jejunography, the success rate of fistula dilation was 100%(19/19), and there was no complication. Five patients with failed percutaneous jejunography underwent open choledocholithotomy through the jejunal output-loop, and bile leakage occurred in one patient. Thirteen patients with anastomotic stenosis, 5 with intrahepatic biliary strictures, and 6 with anastomotic and intrahepatic biliary strictures were diagnosed by choledochoscopy and selective cholangiography in these 24 patients. After choledochoscopic electrotomy, cylindrical balloon dilation, stone removal, stenting and other treatments, the stenosis relief rate was 100%(24/24), and the clearance rate of intrahepatic bile duct stones was 91.7%(22/24).Conclusion:Percutaneous jejunal drainage was a feasible, safe and minimally invasive method for treatment of hepatolithiasis after choledochojejunostomy. The procedure was especially suitable for patients with anterior colonic cholangiojejunostomy.

Comparative study of two biliary drainage strategies in perioperative period of cholangiojejunostomy / 国际外科学杂志

Objective:To evaluate the clinical efficacy of PTCD and ERCP combined with T tube in the perioperative period of biliary anastomosis in patients with moderate to severe obstructive jaundice.Methods:The data of 93 patients with moderate obstructive jaundice who met the inclusion criteria and planned to undergo cholangiojejunostomy (including reconstruction after pancreaticoduodenectomy) in Shuguang Hospital Affiliated to Shanghai University of traditional Chinese medicine from March 2015 to December 2021 were collected. According to the perioperative drainage strategy, 54 cases were divided into PTCD group and 39 cases were divided into ERCP combined with intraoperative T-tube drainage group. Retrospective cohort method was used to analyze the bile duct diameter, postoperative hospital stay, operation cost, time of jaundice reduction, degree of jaundice reduction, average postoperative bile loss, postoperative bile leakage, abdominal infection of the two groups. The measurement data were expressed as mean ± standard deviation( ± s), and t test was used for comparison between groups. The data of skewed distribution were represented by M( Q1, Q3), and nonparametric rank sum test was used. Chi-square test was used for counting data. Results:The preoperative yellowing reduction time, operation cost, mean bile loss 7 days after operation, time to remove biliary drainage tube, and postoperative hospital stay in PTCD group were (5.9±3.1) d, (59 846.3±9 129.7) yuan, 210.0(170.0, 325.0) mL/d, (18.4±5.6) d, and(10.7±4.4) d, respectively, while those in ERCP group were (9.5±4.4) d, (77 833.1±12 164.5) yuan, 285.0(225.0, 370.0) mL/d, (41.4±17.2) d and(15.3±5.6) d, respectively. The difference between the two groups was statistically significant ( P<0.05). There was no significant difference between the two groups in general condition before drainage, cholangitis and hemorrhage after drainage, degree of jaundice reduction, bile leakage after surgery, abdominal infection, etc. Conclusions:When performing biliary enterostomy in patients with moderate or above obstructive jaundice, PTCD and ERCP combined with intraoperative T-tube drainage are effective means to improve the safety of perioperative period. PTCD has some advantages in reducing jaundice efficiency, operation cost and rapid postoperative rehabilitation.

Guía de práctica clínica en atresia de las vías biliares / Clinical practice guidelines in biliary atresia

Basada en la mejor evidencia científica disponible, se presenta la guía de práctica clínica en atresia de vías biliares, la cual se define como una obstrucción progresiva de las vías biliares intra- o extrahepáticas en recién nacidos y lactantes pequeños y causa ictericia colestásica grave y cirrosis hepática. Es una enfermedad poco frecuente, de etiología desconocida, con mayor incidencia en países asiáticos. Clínicamente se expresa por ictericia obstructiva, acolia, coluria y hepatoesplenomegalia. Los complementarios expresan una hiperbilirrubinemia directa con aumento de las enzimas hepáticas, y el diagnóstico se confirma en nuestro hospital con la colangiografía, generalmente en el curso de una laparoscopía. El tratamiento es quirúrgico y consiste en la portoenterostomía de Kasai, con mejores resultados en cuanto al drenaje biliar si se realiza antes de los 60 días de vida, así como el trasplante hepático. La enfermedad tiene un curso progresivo hacia la cirrosis hepática en etapas tempranas de la vida, sobre todo si no se realiza el diagnóstico y tratamiento quirúrgico precozmente, con implicaciones en la supervivencia y calidad de vida de estos pacientes. Por tanto, referir precozmente al paciente con sospecha de atresia de vías biliares a un centro especializado es la piedra angular de la actitud médica. La presente guía de práctica clínica pretende ofrecer las herramientas técnicas estandarizadas para mejorar los resultados a los pacientes con esta enfermedad, así como contribuir con la docencia y las investigaciones(AU)

Based on the best scientific evidence available, it is presented the clinical practice guidelines on biliary atresia. This disease is defined as a progressive obstruction of the intra and/or extrahepatic bile ducts in newborns and young infants, causing severe cholestatic jaundice and cirrhosis of the liver. It is a rare disease of unknown etiology, with a higher incidence in Asian countries. It is clinically expressed by obstructive jaundice, acholia, choluria and hepatosplenomegaly. Laboratory tests show a direct hyperbilirubin and elevated liver enzymes, and in our hospital, the diagnosis is confirmed by a cholangiography, usually during a laparoscopy procedure. It has surgical treatment and it involves a Kasai portoenterostomy, with better results regarding biliary drainage if it is performed before 60 days of life, as well as liver transplant. This condition has a progressive course towards liver cirrhosis in early stages of life, mainly if the diagnosis and surgical treatment are not made timely, with implications for the survival and quality of life of these patients. Therefore, early referral of the patient with suspected biliary atresia to a specialized center is the cornerstone of the medical attitude. This clinical practice guidelines aims to offer standardized technical tools to improve the outcome for patients with this disease, as well as to contribute to teaching and research(AU)

Atresia de vías biliares extrahepaticas en pediatria y citomegalovirus como causante / Extrahepatic biliary atresia in pediatrics and cytomegalovirus as a cause

La atresia de vías biliares es una alteración con gran importancia clínica, más frecuente en la primera infancia, se caracteriza por la obliteración proliferativa de los conductos biliares que progresa a fibrosis hepática, cirrosis e insuficiencia hepática en etapa terminal. El objetivo es presentar un caso clínico inusual y conocer la forma de actuar ante un caso similar. Se trata de paciente femenino de 4 meses de edad, con piel y mucosas ictéricas - verdosas, con hígado palpable debajo del reborde costal derecho, bazo palpable debajo del reborde costal izquierdo. Serología para Citomegalovirus positivo. La ecografía abdominal reporto: Atresia de vías biliares. Se indicó una portoenterostomia de Kasai, sin embargo por la edad de la paciente, el procedimiento quirúrgico no se realizó. La atresia de vías biliares es una patología poco frecuente, su etiología es desconocida, pero es importante saber reconocerla durante los primeros 2 meses de vida, para evitar complicaciones.

Biliary Atresia is a disorder with great clinical importance more frequent in early childhood, characterized by proliferative obliteration of the bile ducts that progresses to liver fibrosis, cirrhosis and end stage liver failure. The objective is to present an unusual clinical case and to know how to act in such a case. It is a female patient of 4 months of age, with icteric skin and mucosa - greenish, with palpable liver below the right costal ridge, palpable spleen below the left costal ridge. Serology for Cytomegalovirus positive.Abdominal ultrasound reported: Biliary Atresia A portoenterostomy of Kasai was indicated, however due to the age of the patient, the surgical procedure was not performed. Biliary Atresia is a rare disease, its etiology is unknown, but it is important to recognize it during the first 2 months of life, to avoid complications.

Análise multivariada de variáveis relacionadas ao fluxo biliar e sobrevida pós-Kasai em pacientes com atresia biliar / Multivariate analysis of biliary flow-related factors and post-kasai survival in biliary atresia patients

ABSTRACT BACKGROUND: Biliary atresia represents the most common surgically treatable cause of cholestasis in newborns. If not corrected, secondary biliary cirrhosis invariably results. OBJECTIVE: To evaluate, through multivariate analysis, the prognostic factors associated with the presence of biliary flow and survival with the native liver following Kasai portoenterostomy. METHODS: The study analyzed data from 117 biliary atresia patients who underwent portoenterostomy and had suitable histological material for evaluation. A logistic regression model was used to assess the presence of biliary flow. Survival was investigated through Kaplan-Meier curves and Cox-adjusted models. RESULTS: One third of patients achieved biliary flow and the median age at surgery was 81 days. Age at surgery, albumin, postoperative complications, biliary atresia structural malformation (BASM), liver architecture, larger duct diameter at porta hepatis, and cirrhosis (Ishak score) were the initial variables for the multivariate analysis. Age at surgery >90 days was the only variable associated with the absence of biliary drainage. Survival analysis revealed that the absence of biliary flow (P<0.0001), age at surgery >90 days (P=0.035), and the presence of BASM (P<0.0001), alone, could predict death or need for liver transplantation. Multivariate analysis demonstrated that the absence of biliary flow (P<0.0001 hazard ratio [HR] 6.25, 95% confidence interval [CI] 3.19-12.22) and the presence of BASM (P=0.014 HR 2.16, 95% CI 1.17-3.99) were associated with lowest survival with the native liver. CONCLUSION: Age at surgery >90 days was associated with absence of biliary flow. The presence of biliary drainage and the absence of structural malformations are cornerstone features for higher survival rates with the native liver.

RESUMO CONTEXTO: A atresia biliar representa a principal causa de colestase tratada cirurgicamente durante o período neonatal. Se a criança não for operada, ela evolui invariavelmente para cirrose biliar secundária. OBJETIVO: Avaliar, através de análise multivariada, os fatores prognósticos associados à presença de fluxo biliar e à sobrevida com fígado nativo após a realização da portoenterostomia de Kasai. MÉTODOS: O estudo analisou 117 pacientes com atresia biliar submetidos à portoenterostomia e com material histológico adequado para avaliação. O modelo de regressão logística foi utilizado para avaliar a presença de fluxo biliar. Sobrevida foi estudada através das curvas Kaplan-Meier e ajuste do modelo de Cox. RESULTADOS: Um terço dos pacientes obteve fluxo biliar e a mediana de idade à cirurgia foi de 81 dias. Idade à cirurgia, albumina, complicação pós-operatória, BASM (do inglês, biliary atresia structural malformation), arquitetura hepática, diâmetro do maior canalículo no porta hepatis e cirrose, segundo o escore de Ishak, foram as variáveis iniciais da análise multivariada. Idade à cirurgia maior que 90 dias de vida foi a única variável associada à ausência de drenagem biliar. A análise de sobrevida mostrou que as variáveis: ausência de fluxo biliar (P<0,0001), idade à cirurgia maior que 90 dias (P=0,035) e presença de BASM (P<0,0001), isoladamente, predizem morte ou necessidade de transplante hepático. Na análise multivariada, ausência de fluxo biliar (P<0,0001 HR:6,25 [IC95% 3,19; 12,22]) e presença de BASM (P=0,014 HR:2,16 [IC95% 1,17; 3,99]) mostraram-se associadas, com significância estatística, a menor sobrevida com fígado nativo. CONCLUSÃO: Idade à cirurgia maior que 90 dias foi identificada como fator de risco independente para ausência de fluxo biliar. Além disso, a presença de drenagem biliar e a ausência de malformações estruturais da atresia biliar são variáveis fundamentais para a maior sobrevida com fígado nativo.

The comparison of desflurane and sevoflurane on postoperative hepatic function of infant with biliary atresia undergoing Kasai operation

BACKGROUND: Biliary atresia is an extrahepatic progressive obliterate cholangiopathy that occurs in infants. Kasai procedure, a surgical method that can help re-establish bile flow from the liver into the intestine, is its first line treatment. Since infants with biliary atresia already have advanced hepatic dysfunction, all kinds of schemes should be considered to minimize further liver damage during surgery. The objective of this study was to compare the postoperative hepatic functions between the two commonly used inhalational anesthetics in infants undergoing the Kasai procedure (i.e., desflurane and sevoflurane). METHODS: This prospective, randomized, double-blind, single-center, and parallel group study included 40 children undergoing Kasai procedure. They were randomly allocated to Group S (sevoflurane) or Group D (desflurane). All the patients were anesthetized with designated anesthetic agent with the end-tidal concentration of about 0.8–1 minimum alveolar concentration. Postoperative hepatic functions were assessed by aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, prothrombin time, and total bilirubin. RESULTS: A total of 38 patients were selected for the study. In both groups, AST, ALT were increased in magnitude to the peak on postoperative day 0 and decreased to preoperative value at postoperative day 3. There were no significant differences between the groups in any laboratory results related to liver function. CONCLUSIONS: Sevoflurane and desflurane, inhalation anesthetics for maintaining anesthesia used in infants undergoing the Kasai procedure, did not show any difference in preserving postoperative hepatic function.

The effect of modified basin-forming biliary-intestinal anastomosis on recurrent stones after choledochojejunostomy / 中华普通外科杂志

Objective@#To evaluate the effect of modified basin-forming anastomosis for recurrent stones after choledochojejunostomy.@*Methods@#A total of 83 patients suffering from recurrent choledocholithiasis undergoing re-operation at our department from Jan 2013 to Dec 2017 were divided into two groups. 46 patients treated by routine choledochojejunostomy(controls), and 37 patients by modified basin-forming biliary-intestinal anastomosis(study group).@*Results@#Control vs study group: intraoperative blood loss were(262±86)ml vs(121±77)ml, blood transfusion: (139±256)ml vs(22±92)ml , and operative time: (316±75)min vs(245±73)min , the number of patients with liver resection were 8 vs 3(all P<0.05). The number of patients with fever, biliary tract infection, abdominal infection, anemia, and postoperative hospital stay were 14 vs 4, 12 vs 3, 7 vs 0, 11 vs 1, (11.5±1.8) d vs (8.5±1.9) d (all P<0.05).@*Conclusions@#The modified basin-forming biliary-intestinal anastomosis is a simple, safe and effective surgical method for recurrent choledocholithiasis after choledochojejunostomy.

The effect of modified basin-forming biliary-intestinal anastomosis on recurrent stones after choledochojejunostomy / 中华普通外科杂志

Objective To evaluate the effect of modified basin-forming anastomosis for recurrent stones after choledochojejunostomy.Methods A total of 83 patients suffering from recurrent choledocholithiasis undergoing re-operation at our department from Jan 2013 to Dec 2017 were divided into two groups.46 patients treated by routine choledochojejunostomy (controls),and 37 patients by modified basin-forming biliary-intestinal anastomosis(study group).Results Control vs study group:intraoperative blood loss were(262 ±86)ml vs(121 ±77) ml,blood transfusion:(139 ±256)ml vs(22 ± 92) ml,and operative time:(316 ± 75) min vs (245 ± 73) min,the number of patients with liver resection were 8 vs 3 (all P＜0.05).The number of patients with fever,biliary tract infection,abdominal infection,anemia,and postoperative hospital stay were 14 vs 4,12 vs 3,7 vs 0,11 vs 1,(11.5 ± 1.8) d vs (8.5 ± 1.9) d (all P ＜ 0.05).Conclusions The modified basin-forming biliary-intestinal anastomosis is a simple,safe and effective surgical method for recurrent choledocholithiasis after choledochojejunostomy.

Current management of biliary atresia based on 35 years of experience at a single center

OBJECTIVE: 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. METHODS: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. RESULTS: 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 period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%. CONCLUSION: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.

The Prognostic Value of CD8⁺ and CD45RO⁺ T Cells Infiltration and Beclin1 Expression Levels for Early Postoperative Cholangitis of Biliary Atresia Patients after Kasai Operation

BACKGROUND: Postoperative cholangitis is a common but severe complication after Kasai portoenterostomy for biliary atresia (BA). This study aimed to identify its prognostic factors. METHODS: Two sets of liver paraffin-embedded tissue samples were collected from BA patients who received Kasai portoenterostomy (n = 25 and n = 31, respectively). Patients were divided into non-cholangitis and cholangitis groups. The infiltration of CD4+, CD8+, CD45RO+, CD68+ cells and expression of Beclin1 were quantitatively evaluated in immunohistochemical analysis. RESULTS: Cholangitis group had a significantly lower CD8+ T cell infiltration but a higher CD45RO+ cell infiltration, and a lower Beclin1 level than non-cholangitis group (all P < 0.01). Multivariate logistic regression analysis indicated that infiltration of CD8+ cells (odds ratio [OR], 0.112; 95% confidence interval [CI], 0.022–0.577) and CD45RO+ cells (OR, 3.88; 95% CI, 1.37–11.03), and Beclin1 level (OR, 0.088; 95% CI, 0.018–0.452) were independent influence factors for early postoperative cholangitis. Receiver operating characteristic (ROC) analysis showed that area under ROC curve (AUROC) values for CD8+ cells, CD45RO+ cells and Beclin1 were 0.857, 0.738 and 0.900, respectively. CONCLUSION: Our findings demonstrated the CD8+ cells, CD45RO+ cells and Beclin1 level possessed the prognostic value for early postoperative cholangitis following Kasai operation, which may be helpful to develop new prevention and treatment strategies for postoperative cholangitis.

Hepato-intestinal anastomosis in the treatment of complex stricture of high bile duct / 中华普通外科杂志

Objective To evaluate hepato-intestinal anastomosis in the treatment of complex stricture of high bile duct.Methods From Jan 2010 to Dec 2016,43 patients undergoing traditional biliary-intestinal anastomosis were grouped into control,45 patients undergoing hepato-intestinal anastomosis were allocated into study group.Results (1) Control vs study grounp,the operative time was (24 ±3)min vs.(15 ± 3)min,intraoperative blood loss and blood transfusion were (384 ± 51)ml vs.(280 ± 41) ml,(649 ± 3) ml vs.(454 ± 8) ml,number of patients with intraoperative blood transfusion,and liver resection were 10 vs.3,and 8 vs.3 respectively,with statistically differences (t =12.48,10.46,144.65,x2 =43.68,49.50,all P ＜ 0.05).(2) Postoperatively efficacy:fever was in 7 vs.3 cases,incision infection in 5 vs.3 cases,abdominal infection was in 5 vs.2 cases,biliary fistula was in 7 vs.3 cases,number of un-planed readmission was in 7 vs.3 cases,and postoperative hospital stay were (14.3 ± 1.5) d vs.(10.7 ± 0.7) d,respectively,between the control group and the study group,with statistically differences (x2 =52.55,58.91,62.23,52.55,52.55,t =16.28,all P ＜0.05).(3) Follow-up situation:all the 88 patients were followed-up for 6 to 96 months (median time,30 months).Biliary restenosis occurred in 5 vs.1 patients between the control group and the study group,respectively,with statistically differences (x2 =65.64,P ＜ 0.05).Conclusions Hepato-intestinal anastomosis is effective in the treatment of complex stricture of high bile duct.

Seguimiento de pacientes con atresia biliar: cirugía tardía y desarrollo de lagunas biliares / Follow up for a cohort of patients with biliary atresia: late surgery and development of biliary cysts

Las dilataciones quísticas de las vías biliares, conocidas como lagunas biliares, se han descrito en el seguimiento de los pacientes sometidos a portoenterostomía o cirugía de Kasai por atresia de las vías biliares (AVB). Su aparición se ha asociado con el desarrollo de colangitis y consecuentemente a un peor pronóstico. OBJETIVO: Describir las características clínicas y pronóstico de pacientes con AVB, sometidos a cirugía de Kasai, con énfasis en aquellos que desarrollaron lagunas biliares. PACIENTES Y MÉTODO: Estudio retrospectivo de pacientes sometidos a portoenterostomía de Kasai por AVB durante los años 2008 a 2016. Se tabuló la información demográfica y variables asociadas a la portoenterostomía de Kasai, trasplante hepático, lagunas o quistes biliares y episodios de colangitis. Se crearon curvas de Kaplan Meier y comparación mediante el test de Log Rank para evaluar sobrevida global, libre de colangitis y con hígado nativo, considerando un valor p como significativo. RESULTADOS: Se analizó el seguimiento de 13 pacientes. El tiempo promedio de realización del Kasai fue a los 85 meses (rango 42-193, DS 40,3), seis pacientes (46%) fueron sometidos a cirugía de Kasai después de los 90 días de vida por derivación tardía. Cuatro (31%) desarrollaron lagunas biliares múltiples, todos presentaron episodios de colangitis. La sobrevida libre de colangitis fue significativamente menor para los portadores de lagunas biliares. Nueve pacientes (69%) recibieron un trasplante hepático, en promedio a los 16 meses de edad (rango 6-40, DS 12,1), en 3 de ellos la causa fue colangitis recurrente. No se encontraron diferencias significativas en la sobrevida con hígado nativo ni en sobrevida global entre portadores y no portadores de lagunas biliares. CONCLUSIONES: En esta cohorte, la incidencia de lagunas biliares luego de la portoenterostomía es similar a la descrita en la literatura. Los resultados concuerdan con la relación propuesta entre ellas y el desarrollo de colangitis. En los pacientes de nuestra serie, el diagnóstico y derivación de AVB fue realizado tardíamente, determinando un peor pronóstico.

Since the introduction of Kasai’s hepatic portoenterostomy, the prognosis of patients with biliary atresia has improved. The presence of intrahepatic biliary cysts or bile lakes has been reported in some patients after the intervention. Bile lakes have been related to cholangitis and a poor outcome. OBJECTIVE: To describe the clinical features and prognosis of patients with biliary atresia after Kasai portoenterostomy, with special emphasis in those who developed biliary cysts. PATIENTS AND METHOD: Data from a retrospective cohort of 13 patients with biliary atresia with a Kasai portoenterostomy from 2008 to 2016 was analyzed. Demographic variables associated to Kasai portoenterostomy, hepatic transplant, biliary cysts and colangitis episodes were tabulated. Kaplan Meir and Log Rank test were used to evaluate colangitis-free and native liver survival. RESULTS: The mean age at Kasai was 85 months (SD 40.3, 42-193 months), six patients (46%) had a Kasai operation after 90 days of life. Four patients (31%) developed multiple biliary cysts; all of them had at least one episode of cholangitis. Cholangitis-free survival was significantly lower for those who developed bile lakes. Nine patients (69%) underwent liver transplant, 3 of them because of recurrent cholangitis. There were no differences in global survival or native liver survival between patients with or without biliary cysts. CONCLUSIONS: The incidence of biliary cysts after Kasai portoenterostomy in this series is similar to the reported. The results are consistent with the relationship proposed between the development of biliary cysts and cholangitis. Our patients, some already derived for evaluation and liver transplantation, underwent Kasai operation at an advanced age, which determines a poor prognosis.

Recent Trends in the Diagnosis and Management of Biliary Atresia in Developing Countries.

Need and purpose of review: Biliary atresia is a progressive obstructive cholangiopathy and is fatal if left untreated within 2 years of life. Delay in referral is because of difficulties in differentiating it from physiologic jaundice and identifying an abnormal stool color. This paper presents an overview on the diagnosis and discusses the current strategies in the management of this disease in developing countries. Methods: Articles were retrieved from the PubMed database using the terms ‘biliary atresia’, ‘Kasai portoenterostomy’ and ‘pediatric liver transplantation’. Contents of the article are also based on personal experience of the authors. Conclusion: A national screening program using stool color cards as part of standard care in the neonatal period will greatly improve early detection of biliary atresia. Outcomes will improve if it is diagnosed at the earliest after birth, the child is referred to an experienced pediatric hepatobiliary unit for evaluation, and undergoes an early Kasai procedure. If an early Kasai portoenterostomy is performed, nearly half of all children survive into adolescence, and about one-third are likely to have a long-term, symptom-free life with normal liver biochemistry. Sequential treatment combining Kasai as first line and liver transplantation as second line results in 90% survival for children with biliary atresia.

Effects of three suture techniques on complications after choledochojejunostomy / 临床肝胆病杂志

ObjectiveTo observe the effects of different suture techniques on complications after choledochojejunostomy. MethodsA retrospective analysis was performed on the clinical data of 162 patients who underwent choledochojejunostomy from January 2005 to December 2014 in our hospital. The patients were divided into group A undergoing simple interrupted suture (n=68), group B undergoing simple continuous suture (n=45), and group C undergoing parachute type continuous suture (n=49). The intraoperative conditions and postoperative complications were compared between the three groups. Comparison of continuous data between the three groups was made by ANOVA and pairwise comparison between any two groups was made by LSD t-tests; comparison of categorical data between the groups was made by chi-square test. ResultsThe anastomosis time and the rate of T-tube insertion showed significant differences between the three groups (P＜0.05). Groups B and C had significantly shorter anastomosis time than group A(P＜0.001), but there was no significant difference in the anastomosis time between groups B and C (P＞0.05). Among the three groups, group A had the highest rate of T-tube insertion; group B had the second highest rate; group C had the lowest rate. Significant differences were found between any two groups in the rate of T-tube insertion by pairwise comparison (P＜0.05). However, there were no significant differences between the three groups in the incidence rates of postoperative bile leakage and choleperitonitis (P＞0.05). There was significant difference in biliary stricture between the three groups in the follow-up examination 6 months after surgery (P＜0.001) and the differences analyzed by pairwise comparison were also significant (P＜0.05). Among the three groups, the degree of biliary stricture in the group A was the highest and that in the group C was the lowest. ConclusionParachute-type continuous suture is a fine suture technique for choledochojejunostomy, which can reduce the anastomosis time, the rate of T-tube insertion, and the degree of biliary stricture.

Effects of three suture techniques on complications after choledochojejunostomy / 临床肝胆病杂志

ObjectiveTo observe the effects of different suture techniques on complications after choledochojejunostomy. MethodsA retrospective analysis was performed on the clinical data of 162 patients who underwent choledochojejunostomy from January 2005 to December 2014 in our hospital. The patients were divided into group A undergoing simple interrupted suture (n=68), group B undergoing simple continuous suture (n=45), and group C undergoing parachute type continuous suture (n=49). The intraoperative conditions and postoperative complications were compared between the three groups. Comparison of continuous data between the three groups was made by ANOVA and pairwise comparison between any two groups was made by LSD t-tests; comparison of categorical data between the groups was made by chi-square test. ResultsThe anastomosis time and the rate of T-tube insertion showed significant differences between the three groups (P＜0.05). Groups B and C had significantly shorter anastomosis time than group A(P＜0.001), but there was no significant difference in the anastomosis time between groups B and C (P＞0.05). Among the three groups, group A had the highest rate of T-tube insertion; group B had the second highest rate; group C had the lowest rate. Significant differences were found between any two groups in the rate of T-tube insertion by pairwise comparison (P＜0.05). However, there were no significant differences between the three groups in the incidence rates of postoperative bile leakage and choleperitonitis (P＞0.05). There was significant difference in biliary stricture between the three groups in the follow-up examination 6 months after surgery (P＜0.001) and the differences analyzed by pairwise comparison were also significant (P＜0.05). Among the three groups, the degree of biliary stricture in the group A was the highest and that in the group C was the lowest. ConclusionParachute-type continuous suture is a fine suture technique for choledochojejunostomy, which can reduce the anastomosis time, the rate of T-tube insertion, and the degree of biliary stricture.

BILIARY ATRESIA: evaluation on two distinct periods at a reference pediatric service / Atresia biliar: avaliação em dois períodos distintos em um serviço de referência

Context Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that pre­sents with biliary obstruction exclusively in the neonatal period. Objectives To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods. Methods Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011. Results Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001). On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15), but difference was found when comparing the age at surgery (P = 0.002). Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up. Conclusions Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors. .

Contexto A atresia biliar é uma doença progressiva, idiopática, fibro obliterativa, da vias bilares extra hepáticas que se apresenta com obstrução biliar exclusivamente no periodo neonatal. Objetivos Caracterizar duas épocas distintas visando avaliar se ocorreram alterações quanto a idade de encaminhamento das crianças, idade cirúrgica, duração do tempo entre internação e realização da cirurgia de Kasai, resultados cirúrgicos quanto a presença de fluxo biliar e sobrevida entre dois períodos. Métodos Estudo retrospectivo de crianças com diagnóstico de atresia biliar em dois períodos: 1983-1993 e 1998-2011. Resultados Foram admitidas 129 crianças com diagnóstico de AB, 48 (casuística I) e 81 (casuística II), com idade de admissão mediana de 94 e 60 dias, nas casuísticas I e II, respectivamente (P = 0,0001). Na avaliação dos pacientes submetidos à portoenterostomia com idade até 120 dias de vida, não houve diferença em relação ao tempo entre internação e realização da cirurgia de Kasai (P = 0,15), mas ocorreu ao comparar a idade à cirurgia entre os dois períodos (P = 0,002). A probabilidade estimada de sobrevida dos pacientes sem fluxo biliar livre de transplante ou óbito em 18 meses foi de 44,6%, 38,7% nas casuísticas I e II, respectivamente. Com fluxo biliar, todos estavam vivos ao longo do seguimento da casuística I e 80,3% em sete anos na casuística II. Conclusões Apesar dos pacientes chegarem mais cedo e serem submetidos à portoenterostomia, ainda se opera tarde. Encaminhamento tardio e a propedêutica prolongada são os principais contribuintes. Quanto maior a idade cirúrgica, menor a probabilidade de se obter fluxo biliar e consequentemente menor sobrevida. .