hepatic portoenterostomy


Summary: Operation for BILIARY ATRESIA by anastomosis of the BILE DUCTS into the JEJUNUM or DUODENUM.

Top Publications

  1. Visser B, Suh I, Hirose S, Rosenthal P, Lee H, Roberts J, et al. The influence of portoenterostomy on transplantation for biliary atresia. Liver Transpl. 2004;10:1279-86 pubmed
    ..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. ..
  2. Ernest van Heurn L, Saing H, Tam P. Cholangitis after hepatic portoenterostomy for biliary atresia: a multivariate analysis of risk factors. J Pediatr. 2003;142:566-71 pubmed
    ..72; 95% CI, 2.89-11.3) or poor (odds ratio, 3.29; 95% CI, 1.89-5.7) bile drainage. Cholangitis was not an independent risk factor for death or liver transplantation. ..
  3. Kobayashi H, Yamataka A, Urao M, Okazaki T, Yanai T, Koga H, et al. Innovative modification of the hepatic portoenterostomy. Our experience of treating biliary atresia. J Pediatr Surg. 2006;41:e19-22 pubmed
    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 ..
  4. Chittmittrapap S, Chandrakamol B, Poovorawan Y, Suwangool P. Factors influencing outcome after hepatic portoenterostomy for biliary atresia: a logistic regression analysis. J Med Assoc Thai. 2005;88:1077-82 pubmed
    The association of many factors with the outcome in Biliary atresia (BA) after hepatic portoenterostomy has drawn the attention of many pediatric hepatologists and hepatobiliary surgeons...
  5. Suzuki T, Hashimoto T, Kondo S, Sato Y, Hussein M. Evaluating patients' outcome post-Kasai operation: a 19-year experience with modification of the hepatic portoenterostomy and applying a novel steroid therapy regimen. Pediatr Surg Int. 2010;26:825-30 pubmed publisher
    We reported a modification of the hepatic portoenterostomy (HPE) for biliary atresia with favorable results. HPE is associated with a risk of hepatic impairment, so we adopted a novel steroid therapy regimen well suited to our procedure...
  6. Tanano H, Hasegawa T, Kimura T, Sasaki T, Kawahara H, Kubota A, et al. Proposal of fibrosis index using image analyzer as a quantitative histological evaluation of liver fibrosis in biliary atresia. Pediatr Surg Int. 2003;19:52-6 pubmed
    ..Liver biopsies were performed in 46 BA patients at hepatic portoenterostomy (HPE) and stoma closure...
  7. Sumida W, Kaneko K, Ono Y, Tainaka T, Ando H. Different polyunsaturated fatty acid profiles in patients with biliary atresia after successful Kasai operation and liver transplantation. Pediatr Surg Int. 2009;25:255-9 pubmed publisher
    ..83). The n-3/n-6 ratio was abnormal after Kasai operation despite normal liver function. This seems to be one factor of persisting inflammation in Glisson's area. ..
  8. Hsieh C, Huang L, Lee S, Huang C, Lee C, Chuang J, et al. Evaluation of hepatocyte growth factor in patients with biliary atresia. J Pediatr Surg. 2008;43:1333-7 pubmed publisher
    ..Hepatocyte growth factor might have a protective role, probably through the induction of hepatocyte proliferation, during the development of BA-associated liver cirrhosis. ..
  9. Ohi R. Surgery for biliary atresia. Liver. 2001;21:175-82 pubmed
    ..of biliary atresia has been improved in recent years, particularly in the era of liver transplantation, hepatic portoenterostomy, e.g., the Kasai operation, is still the first line of surgical treatment...

More Information


  1. Shteyer E, Ramm G, Xu C, White F, Shepherd R. Outcome after portoenterostomy in biliary atresia: pivotal role of degree of liver fibrosis and intensity of stellate cell activation. J Pediatr Gastroenterol Nutr. 2006;42:93-9 pubmed
    ..Fibrosis scores and intensity of alpha-SMA expression may be predictors of outcome after KP and may indicate those patients who might benefit from trials of potential antifibrotic agents early in the course of BA. ..
  2. Hasegawa T, Sasaki T, Kimura T, Sawai T, Nose K, Kamata S, et al. Prenatal ultrasonographic appearance of type IIId (uncorrectable type with cystic dilatation) biliary atresia. Pediatr Surg Int. 2002;18:425-8 pubmed
    ..and underwent excision of the cystic lesions and dissection of the portal bile-duct remnants, followed by hepatic portoenterostomy. Case 1 showed persistent jaundice and finally underwent liver transplantation (LTx), case 2 became ..
  3. Davenport M. Biliary atresia: outcome and management. Indian J Pediatr. 2006;73:825-8 pubmed
    ..The remainder may be candidates for liver transplantation (where available) although donor organ shortage and immunosuppresion-related complications remain significant problems. ..
  4. Escobar M, Jay C, Brooks R, West K, Rescorla F, Molleston J, et al. Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy. J Pediatr Surg. 2006;41:99-103; discussion 99-103 pubmed
    ..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. ..
  5. Pacheco M, Campbell K, Bove K. Ductal plate malformation-like arrays in early explants after a Kasai procedure are independent of splenic malformation complex (heterotaxy). Pediatr Dev Pathol. 2009;12:355-60 pubmed publisher
    ..Ductal plate malformation-like arrays do not distinguish perinatal BA from BA associated with heterotaxy in liver explants after a failed Kasai procedure. ..
  6. Tiao M, Tsai S, Kuo H, Chen C, Yang C. Epidemiological features of biliary atresia in Taiwan, a national study 1996-2003. J Gastroenterol Hepatol. 2008;23:62-6 pubmed
    ..Taiwan has the second-highest incidence of BA reported in world literature. Viral infection outbreaks remain a potential candidate as a cause of BA. The management of BA has been improving, with a better 5-year overall survival rate. ..
  7. Kieling C, Santos J, Vieira S, Ferreira C, Linhares A, Lorentz A, et al. Biliary atresia: we still operate too late. J Pediatr (Rio J). 2008;84:436-41 pubmed publisher
    ..0001). Late performance of portoenterostomy was a constant in the past 25 years, and this delay led to shorter survival periods with native livers for biliary atresia patients. ..
  8. McKiernan P, Baker A, Lloyd C, Mieli Vergani G, Kelly D. British paediatric surveillance unit study of biliary atresia: outcome at 13 years. J Pediatr Gastroenterol Nutr. 2009;48:78-81 pubmed publisher
    ..Children with biliary atresia should be treated in experienced centres to maximize the chance of successful surgery. ..
  9. Shimadera S, Iwai N, Deguchi E, Kimura O, Ono S, Furukawa T, et al. Predicting factors on the occurrence of cystic dilatation of intrahepatic biliary system in biliary atresia. Pediatr Surg Int. 2010;26:611-4 pubmed publisher
    ..In this study, we investigated the predicting factors of CDIB development after jaundice resolved following hepatoportoenterostomy (HPE)...
  10. Bu L, Chen H, Chang C, Ni Y, Hsu H, Lai H, et al. Prophylactic oral antibiotics in prevention of recurrent cholangitis after the Kasai portoenterostomy. J Pediatr Surg. 2003;38:590-3 pubmed
    ..09 and.018). Use of TMP/SMZ or neomycin is effective as a prophylactic agent against the recurrence of cholangitis after the Kasai portoenterostomy, but there is no difference in efficacy between these 2 regimens. ..
  11. Davenport M, Gonde C, Redkar R, Koukoulis G, Tredger M, Mieli Vergani G, et al. Immunohistochemistry of the liver and biliary tree in extrahepatic biliary atresia. J Pediatr Surg. 2001;36:1017-25 pubmed
  12. Sugawara Y, Makuuchi M, Kaneko J, Ohkubo T, Mizuta K, Kawarasaki H. Impact of previous multiple portoenterostomies on living donor liver transplantation for biliary atresia. Hepatogastroenterology. 2004;51:192-4 pubmed
    ..Our surgical results suggest that multiple previous portoenterostomies might have negative short-term effects in patients who undergo living donor liver transplantation for biliary atresia. ..
  13. Lao O, Larison C, Garrison M, Healey P, Goldin A. Steroid use after the Kasai procedure for biliary atresia. Am J Surg. 2010;199:680-4 pubmed publisher
    ..03-6.97). Perioperative steroids after the Kasai procedure are associated with shorter postoperative length of stay. Work is needed to ascertain whether this relationship is causal. ..
  14. Kobayashi H, Yamataka A, Koga H, Okazaki T, Tamura T, Urao M, et al. Optimum prednisolone usage in patients with biliary atresia postportoenterostomy. J Pediatr Surg. 2005;40:327-30 pubmed
  15. Kotb M, Sheba M, El Koofy N, Mansour S, El Karaksy H, Dessouki N, et al. Post-portoenterostomy triangular cord sign prognostic value in biliary atresia: a prospective study. Br J Radiol. 2005;78:884-7 pubmed
    ..026). Post-portoenterostomy TC sign is associated with more morbidity and mortality; and reflects inadequate surgical technique. ..
  16. Matthews R, Russo P, Berry G, Piccoli D, Rand E. Biliary atresia associated with a fatty acid oxidation defect. J Pediatr Gastroenterol Nutr. 2002;35:624-8 pubmed
  17. Okada A, Hasegawa T, Oguchi Y, Nakamura T. Recent advances in pathophysiology and surgical treatment of congenital dilatation of the bile duct. J Hepatobiliary Pancreat Surg. 2002;9:342-51 pubmed
  18. Dhawan A, Trivedi P, Cheeseman P, Baker A, Howard E, Mieli Vergani G. Serum hyaluronic acid as an early prognostic marker in biliary atresia. J Pediatr Surg. 2001;36:443-6 pubmed
    ..High serum concentrations of HA at diagnosis may help to identify at an early stage those patients with BA who have a poor prognosis and will require liver transplant by 5 years of age. ..
  19. Palanivelu C, Rangarajan M, Parthasarathi R, Amar V, Senthilnathan P. Laparoscopic management of choledochal cysts: technique and outcomes--a retrospective study of 35 patients from a tertiary center. J Am Coll Surg. 2008;207:839-46 pubmed publisher
    ..The accepted mode of treatment is total excision with hepaticojejunostomy. In this retrospective study, we present our technique and results of laparoscopic choledochal cyst excision...
  20. Ayuso L, Vila Carbo J, Lluna J, Hernandez E, Marco A. [Laparoscopic Kasai portoenterostom: present and future of biliary atresia treatment]. Cir Pediatr. 2008;21:23-6 pubmed
    ..The advantages of laparoscopic portoenterostomy are yet to be proved whenever liver transplantation is indicated. ..
  21. Agarwal G, Sharma R, Bhatnagar V. Assessment of latent manganese toxicity as a prognostic factor following surgery for biliary atresia. Eur J Pediatr Surg. 2008;18:22-5 pubmed publisher
    ..The aim of this study was to investigate latent manganese toxicity in patients with biliary atresia and to use this tool to predict the prognosis of biliary atresia patients after Kasai's portoenterostomy...
  22. Mi Y, Li R, Xu K, Li S, Wang P, Wu L, et al. Modified method of hepatic portal choledochoplasty to treat benign strictures of hilar biliary ducts. J Gastroenterol Hepatol. 2008;23:e395-8 pubmed publisher
    ..05). Compared to RYHJ, HPC is a safer and more efficient method to treat benign strictures of hilar biliary ducts. It preserves the sphincter of Oddi and normal biliary duct pressure, thus avoiding bile reflux into the bile duct. ..
  23. Okada T, Sasaki F, Honda S, Naito S, Todo S. Microbial flora alterations in jejunum and colon after chemical bowel preparation before Kasai hepatoportojejunostomy. Eur J Pediatr Surg. 2007;17:304-7 pubmed
    ..The authors propose that colonic-type flora are generally found in the feces before and after the administration of oral antibiotics, and no bacteria are detected in the bilioenteric conduits. ..
  24. Shimadera S, Iwai N, Deguchi E, Kimura O, Fumino S, Ono S. The significance of steroid therapy after hepatoportoenterostomy in infants with biliary atresia. Eur J Pediatr Surg. 2007;17:100-3 pubmed
    ..However, there was a significant correlation between the mean steroid dose and the period of jaundice (p = 0.021). A high mean dose of steroids could shorten the jaundice period after HPE in the uncorrectable type of BA. ..
  25. Aspelund G, Ling S, Ng V, Kim P. A role for laparoscopic approach in the treatment of biliary atresia and choledochal cysts. J Pediatr Surg. 2007;42:869-72 pubmed
    ..Longer follow-up of a larger patient cohort is needed. ..
  26. Dutta S, Woo R, Albanese C. Minimal access portoenterostomy: advantages and disadvantages of standard laparoscopic and robotic techniques. J Laparoendosc Adv Surg Tech A. 2007;17:258-64 pubmed
    ..We believe both surgical modalities are feasible from a technical point of view. However, the optical and dexterity advantages of the robotic system were offset by the large instrument size and lack of force feedback. ..
  27. Pulitano C, Kobayashi A, Aldrighetti L. Effects of adjuvant therapy on the outcomes of surgical management of extrahepatic biliary atresia. Hepatology. 2008;48:342-3 pubmed publisher
  28. Petersen C. Biliary atresia: interdisciplinary initiatives focus on a rare disease. Pediatr Surg Int. 2007;23:521-7 pubmed
  29. Cleghorn G. Biliary atresia and its micromanagement: does it really matter?. J Pediatr. 2005;147:142-3 pubmed
  30. Houben C, Phelan S, Davenport M. Late-presenting cholangitis and Roux loop obstruction after Kasai portoenterostomy for biliary atresia. J Pediatr Surg. 2006;41:1159-64 pubmed
    ..Resolution of the obstruction allows preservation of their native liver. ..
  31. Lee H, Lewis J, Schoen B, Brand T, Ricketts R. Kasai portoenterostomy: differences related to race. J Pediatr Surg. 2001;36:1196-8 pubmed
    ..Increased effort at identifying biliary atresia in AA children may lead to earlier diagnosis and treatment and improved outcomes. ..
  32. Sreevastava D, Setlur R, Sharma V, Padmini H, Puri B, Bhargava A. Anesthetic management of an infant with lupus and congenital complete heart block. Paediatr Anaesth. 2006;16:216-7 pubmed
  33. Yamataka A, Yanai T, Hosoda Y, Segawa O, Kobayashi H, Miyano T. A case of biliary atresia with duplication of the common bile duct. J Pediatr Surg. 2001;36:506-7 pubmed
    ..A diagnosis of BA with duplicated common bile duct was made. Postoperative course was uneventful, and at a follow-up assessment at 7 months, she was jaundice free, and her progress has been unremarkable...
  34. Lobe T, Oldham K, Richardson C. Successful separation of a conjoined biliary tract in a set of omphalopagus twins. J Pediatr Surg. 1989;24:930-2 pubmed
    ..The intestines were divided to give each infant an equal share, and an hepaticoenterostomy was constructed in each of the twins...
  35. Wong K, Chung P, Chan K, Fan S, Tam P. Should open Kasai portoenterostomy be performed for biliary atresia in the era of laparoscopy?. Pediatr Surg Int. 2008;24:931-3 pubmed publisher
    ..Laparoscopic Kasai portoenterostomy seems to be associated with more post-operative complications and worse early clinical outcome. As a result, we remain guarded about the present-day technique of laparoscopy for biliary atresia. ..
  36. Suchy F. Neonatal cholestasis. Pediatr Rev. 2004;25:388-96 pubmed
  37. Sasson S, Yerushalmi B, Mordechay Y, Cohen Z. [Long term results of Kasai portoenterostomy for the management of biliary atresia]. Harefuah. 2009;148:161-4, 211 pubmed
    ..41). The long term results of our center meet the reported results from other parts of the world, with no significant difference between the two populations. ..
  38. Chang H, Park Y, Koh H, Kim S, Chung K, Oh J, et al. Hepatic fibrosis scan for liver stiffness score measurement: a useful preendoscopic screening test for the detection of varices in postoperative patients with biliary atresia. J Pediatr Gastroenterol Nutr. 2009;49:323-8 pubmed publisher
    ..FibroScan is a novel, noninvasive, and useful screening method for the preendoscopic detection of varices in postoperative patients with BA. ..
  39. Matsuo S, Suita S, Kubota M, Shono K, Kamimura T, Kinugasa Y. Hazards of hepatic portocholecystostomy in biliary atresia. Eur J Pediatr Surg. 2001;11:19-23 pubmed
    ..1.90 +/- 0.39 mm (n = 11) in NH, p<0.01). HPC was thus found to be an excellent operative method for preventing postoperative cholangitis in BA, however, many clinical problems still need to be overcome for such a narrow distal duct. ..
  40. Sangkhathat S, Patrapinyokul S, Tadtayathikom K, Osatakul S. Peri-operative factors predicting the outcome of hepatic porto-enterostomy in infants with biliary atresia. J Med Assoc Thai. 2003;86:224-31 pubmed
    ..Early cholangitis was an accelerator of progressive cirrhosis. Stool color and bilirubin level at one month after surgery can be used as predictors of jaundice clearance. ..
  41. Sokol R. Corticosteroid treatment in biliary atresia: Tonic or toast?. Hepatology. 2007;46:1675-8 pubmed
  42. Stringer M, Davison S, Rajwal S, McClean P. Kasai portoenterostomy: 12-year experience with a novel adjuvant therapy regimen. J Pediatr Surg. 2007;42:1324-8 pubmed
    ..In this series, adjuvant postoperative treatment with a short course of oral dexamethasone and longer-term ursodeoxycholic acid significantly improved the outcome after Kasai portoenterostomy. ..
  43. Lee S, DuBois J. Hepatic inflammatory pseudotumor: case report, review of the literature, and a proposal for morphologic classification. Pediatr Surg Int. 2001;17:555-9 pubmed
    ..Type 1 lesions are large, solitary lesions, often with central necrosis, giving a characteristic radiographic appearance. Type 2 lesions are multiple smaller, solid nodules indistinguishable from metastatic malignancy. ..
  44. Takahashi A, Masuda N, Suzuki M, Shimura T, Nomoto K, Suzuki N, et al. Evidence for segmental bile drainage by hepatic portoenterostomy for biliary atresia: Cholangiographic, hepatic venographic, and histologic evaluation of the liver taken at liver transplantation. J Pediatr Surg. 2004;39:1-5 pubmed
    The result of hepatic portoenterostomy for biliary atresia (BA) has improved, but there are some patients who experience worsened liver function in the long term after one decrease in jaundice owing to portoenterostomy...
  45. Kuroiwa M, Suzuki N, Hatakeyama S, Takahashi A, Ikeda H, Sakai M, et al. Magnetic resonance angiography of portal collateral pathways after hepatic portoenterostomy in biliary atresia: comparisons with endoscopic findings. J Pediatr Surg. 2001;36:1012-6 pubmed
    ..MRA was highly sensitive and specific in detecting esophagogastric collateral vessels of the portal venous system. An endoscopic examination is unnecessary until collateral vessels are seen on MRA after surgery for BA. ..
  46. Volpert D, White F, Finegold M, Molleston J, DeBaun M, Perlmutter D. Outcome of early hepatic portoenterostomy for biliary atresia. J Pediatr Gastroenterol Nutr. 2001;32:265-9 pubmed
    The outcome of the hepatic portoenterostomy (Kasai) procedure for biliary atresia is improved when it is performed before 90 days of age...
  47. Mshelbwala P, Sabiu L, Lukong C, Ameh E. Management of biliary atresia in Nigeria: the ongoing challenge. Ann Trop Paediatr. 2007;27:69-73 pubmed
    ..A concerted effort to encourage primary practitioners to detect and refer these infants at an earlier age is critical to outcome. ..
  48. Dolgin S. Answered and unanswered controversies in the surgical management of extra hepatic biliary atresia. Pediatr Transplant. 2004;8:628-31 pubmed
    ..The Biliary Atresia Research Consortium, a multi-institutional National Institutes of Health (NIH)-supported project, will address many of the unanswered issues. ..
  49. Meyers R, Book L, O Gorman M, Jackson W, Black R, Johnson D, et al. High-dose steroids, ursodeoxycholic acid, and chronic intravenous antibiotics improve bile flow after Kasai procedure in infants with biliary atresia. J Pediatr Surg. 2003;38:406-11 pubmed
    ..Adjuvant therapy using high-dose steroids, ursodeoxycholic acid, and intravenous antibiotics may accelerate the clearance of jaundice and decrease the need for early liver transplantation after Kasai portoenterostomy. ..
  50. Raval M, Dzakovic A, Bentrem D, Reynolds M, Superina R. Trends in age for hepatoportoenterostomy in the United States. Surgery. 2010;148:785-91; discussion 791-2 pubmed publisher
    ..Both hospital and socioeconomic factors play a role in the early treatment of biliary atresia. ..
  51. Leonhardt J, Kuebler J, Turowski C, von Wasielewski R, Pfister E, Becker T, et al. [Predictive parameters in children with biliary atresia]. Chirurg. 2009;80:628-33 pubmed publisher
    ..In conclusion our data demonstrate that a lack of predictive factors must prevent primary liver transplantation in BA patients. ..
  52. Tsao K, Rosenthal P, Dhawan K, Danzer E, Sydorak R, Hirose S, et al. Comparison of drainage techniques for biliary atresia. J Pediatr Surg. 2003;38:1005-7 pubmed
    ..Although the appendix may serve as an alternative biliary conduit, traditional Kasai portoenterostomy appears to achieve better biliary drainage. ..
  53. Chardot C, Carton M, Spire Bendelac N, Le Pommelet C, Golmard J, Reding R, et al. Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia?. J Pediatr. 2001;138:224-8 pubmed
    ..Preoperative evaluation should exclude patients with advanced liver disease for whom liver transplantation should not be delayed. ..