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Internal surgical drainage for bile lakes following hepatoportoenterostomy for biliary atresia
Journal of Pediatric Surgery Case Reports 43 (2019) 71–73
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Internal surgical drainage for bile lakes following hepatoportoenterostomy for biliary atresia
T
Edward C. Deea, Benjamin Zendejasb, Rima Fawazc, Heung Bae Kimb,∗ a
Harvard Medical School, Boston, MA, USA Department of Pediatric Surgery, Boston Children's Hospital, Boston, MA, USA c Division of Gastroenterology and Nutrition, Boston Children's Hospital, Boston, MA, USA b
A R T I C LE I N FO
A B S T R A C T
Keywords: Bile lake Biliary atresia Cystjejunostomy
Bile lakes may be present at time of diagnosis or develop later following hepatoportoenterostomy (HPE) in patients with biliary atresia (BA). We report a patient with recurrent cholangitis following HPE who developed multiple large biliary cystic lesions. Percutaneous drainage resulted in resolution of jaundice and cholangitis but capping of drains resulted in recurrence of symptoms. Therefore, internal drainage of the bile lakes was accomplished using cystjejunostomy to the roux limb of the previous portoenterostomy. At 18 months of follow-up, the patient has remained jaundice free with only one recurrent cholangitis episode which occurred after stopping her antibiotic prophylaxis. Although this patient will likely require liver transplantation in the next few years, this case suggests that cystjejunostomy may be a viable surgical option to improve bile flow and prevent cholangitis in patients who develop bile lakes following HPE.
1. Introduction About 25% of patients with biliary atresia (BA) will develop at least one intrahepatic biliary cyst, or “bile lake” [1–4]. Bile lakes are thought to result from bile stasis and the formation of intrabiliary calculi, leading to repeated local inflammation, with subsequent damage to and fusion of bile ducts [5,6]. The presence of bile lakes is associated with a poor prognosis in patients with BA [1,6], possibly related to an increased risk of recurrent cholangitis [7] and progression to cirrhosis [6]. The Kasai hepatoportoenterostomy (HPE) was developed to allow for effective biliary drainage but outcomes depend on both adequate clearance of jaundice as well as avoidance of complications such as cholangitis [8]. Therefore, addressing bile lakes effectively may be an important adjunct to improve outcomes [9]. Percutaneous transhepatic drainage of bile lakes is an option for reducing rates of cholangitis in some patients [10], but safe and prolonged percutaneous drainage may be difficult in some cases [1,4]. In this case we report the use of cystjejunostomy as a viable long term surgical option to provide internal biliary drainage of bile lakes in a patient with BA following HPE. 2. Case report A 52-day female underwent HPE for BA without any other
malformations after she presented with cholestatic jaundice, acholic stools, and a non-excreting hepatobiliary iminodiacetic acid (HIDA) scan. Her abdominal ultrasound revealed a hypoplastic gallbladder without any evidence of intrahepatic abnormalities. At the time of her laparotomy done two days after presentation to our hospital, no identifiable gallbladder lumen was found so an intraoperative cholangiogram could not be performed. A Kasai HPE was completed, with bile drainage noted intraoperatively. Pathology revealed a fibrotic bile duct with patchy inflammatory infiltrate. The patient did well and was discharged home on the fifth postoperative day on fat soluble vitamins, ursodiol, and trimethoprim/sulfamethoxazole for cholangitis prophylaxis as per our practice. By two months postoperatively, her total and direct bilirubin were 0.8 and 0.4 mg/dl respectively. She developed cholangitis three months after her surgery. Her abdominal ultrasound revealed diffusely prominent periportal echoes in the liver thought to represent periportal edema or inflammation as well as ascites. She was treated with a prolonged course of antibiotics. Her total and direct bilirubin levels peaked at 14.8 and 11.8 mg/dl respectively. Follow up imaging done three months after her initial cholangitis revealed possible cystic lesions at the porta hepatis. She was referred for liver transplant evaluation; however, listing was deferred due to her clinical improvement, resolution of her ascites, and improving total and direct bilirubin at that
∗
Corresponding author. 300 Longwood Avenue, Fegan, 3rd Floor, Boston, MA 02115, USA. E-mail addresses: [email protected] (E.C. Dee), [email protected] (B. Zendejas), [email protected] (R. Fawaz), [email protected] (H.B. Kim). https://doi.org/10.1016/j.epsc.2019.02.015 Received 12 February 2019; Accepted 20 February 2019 Available online 25 February 2019 2213-5766/ © 2019 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Journal of Pediatric Surgery Case Reports 43 (2019) 71–73
E.C. Dee, et al.
Fig. 1. Magnetic resonance imaging demonstrating multiple intrahepatic fluid collections. These collections include branching fluid-filled structures consistent with dilated bile ducts (A) and multiple rounded fluid-filled structures originating distal to the dilated structures suggestive of bile lakes (B). Fig. 2. Intraoperative photograph of cystic bile lake (“bl”) and jejunum (“j”) before (A) and after (B) construction of cystjejunostomy (“*”).
time to 5.4 and 3.7 mg/dl respectively. She was followed by her local provider for persistent cholestasis and poor weight gain in spite of nasogastric feeds and formula fortification. Her stools remained pigmented. She presented to our institution six months after last seen with fever, cachexia, and a hard, large liver with a protuberant mass. She was admitted urgently and treated with intravenous broad spectrum antibiotics. Ultrasound suggested the presence of large intrahepatic cysts and magnetic resonance imaging (MRI) of her abdomen confirmed the presence of multiple branching fluid-filled structures throughout the liver consistent with dilated bile ducts and bile lakes (Fig. 1). Blood cultures grew Citrobacter Freundii complex 12 h later. Percutaneous placement of two separate drains into these bile lakes resulted in resolution of her infection and marked improvement in jaundice. Biliary cultures grew the same organism. Over the next several weeks, drain output stabilized with about 200 mL of bile drainage from each side per day. She received nutritional rehabilitation and was listed for liver
transplantation. After two months of antibiotics and multiple percutaneous drain replacements, repeat abdominal imaging demonstrated improvement in her biliary dilation; however, bile lakes were persistent despite adequate drainage (total bilirubin decreased to 1.1 mg/dl) and resolution of jaundice. Given the clinical and laboratory improvement with adequate biliary drainage, we felt that internal surgical drainage would benefit our patient, so she was inactivated on the liver transplant list. Upon re-exploration of her abdomen, moderate ascites and some features of portal hypertension were noted; the liver was firm with a micronodular appearance, which on biopsy demonstrated evidence of cholestasis and biliary-type cirrhosis. One of the bile lakes was noted on the surface of segments IVb/III where the anterior abdominal percutaneous drain was entering. The drain was removed and the cyst wall 72
Journal of Pediatric Surgery Case Reports 43 (2019) 71–73
E.C. Dee, et al.
cystjejunostomy to the prior portoenterostomy jejunal roux limb as a viable means to treat bile lakes following HPE in patients with biliary atresia.
was excised. The adjacent jejunal roux limb from the previous HPE was anastomosed to the cyst wall in side-to-side configuration to create a cystjejunostomy (Fig. 2). Of note, bile was noted both within the roux limb coming from the portoenterostomy as well as within the cyst. The other large posteriorly located bile lake that was being drained by the right flank percutaneous drain was not amenable to operative exposure without adding substantial morbidity, and was not pursued. She recovered well from this operation without apparent complications. The excised biliary cyst wall demonstrated benign fibrous tissue with no recognizable epithelial lining on pathology, consistent with a bile lake [6]. Subsequently, her right flank percutaneous drain fell out resulting in a biliary fistula that was managed with drainage into a stoma bag until it spontaneously closed several months later. After one cholangitis-free year, prophylactic antibiotic treatment (levofloxacin) was stopped. She developed cholangitis two months later and was retreated with antibiotics. At last follow-up, 18 months after surgery, she remains jaundice free (total and direct bilirubin pf 0.7 and 0.4 mg/dl, respectively) with adequate liver function and good growth and development.
Informed consent Consent to publish the case report was not obtained. This report does not contain any personal information that could lead to the identification of the patient. Informed consent was waived by our IRB due to the fact that the data collected for this study was for a case report and was de-identified. Authorship All authors attest that they meet the current ICMJE criteria for authorship. Edward C. Dee: Data collection, drafting article, critical revision of article. Benjamin Zendejas: Concept and design, data collection, drafting article, critical revision of article, approval of article. Rima Fawaz: Concept and design, data collection, drafting article, critical revision of article, approval of article. Heung Bae Kim: Concept and design, data collection, drafting article, critical revision of article, approval of article.
3. Discussion The etiology of bile lake formation in patients with biliary atresia is unclear. Tainaka et al. proposed the following mechanism for bile lake formation: bile stasis occurs in intrahepatic bile ducts leading to calculi formation within bile ducts; calculi result in recurrent cholangitis, which damages the bile ducts, which then fuse together and form bile lakes [5]. Bile lakes are defined as solitary cystic lesions with fibrotic walls lacking biliary epithelia, and were described as retention pseudocysts secondary to obstruction and damage of bile ducts [6]. Bile lakes are associated with recurrent cholangitis and progressive liver disease [6,7]. These are in contrast to biliary duct dilation secondary to HPE stricture, which is associated with a favorable prognosis given the possibility of interventions to treat the stricture [6]. Our patient had “benign fibrous tissue with no recognizable epithelial lining,” most consistent with Tainaka's histologic description of a true bile lake, for which adequate drainage may decrease the risk of progressive liver disease [6]. When cysts are single or communicating, percutaneous drainage may be effective at resolving cholangitis and jaundice [10,11]. Werlin et al. recommend percutaneous drainage as initial management with possible progression to surgical drainage if cholangitis does not remit [11]. They also recommend weighing the potential benefit of surgical drainage against the added complexity of liver transplantation that may result from additional operations [11]. Saito et al. reported a successful case of surgical drainage of bile lakes using Penrose drains to the abdominal wall with no recurrent episodes of fever or jaundice at 32 months' follow-up [9]. This patient was reported to be doing well 10 years postoperatively [12]. Tsuchida et al. report a successful similarly treated patient, and further report a literature review of eight cases of bile lakes treated with laparotomy either with simple drainage or internal intestinal drainage, though the method of internal intestinal drainage was not specified [12]. Nakama et al. report a patient in whom laparotomy and cyst drainage was performed as percutaneous drainage was deemed too dangerous but long-term follow-up was not reported [4].
Conflict of interest The following authors have no financial disclosures: ECD, BZ, RF, HBK. Funding No funding or grant support was received to conduct this study. References [1] Bu L-N, Chen H-L, Ni Y-H, Peng S, Jeng Y-M, Lai H-S. Multiple intrahepatic biliary cysts in children with biliary atresia. J Pediatr Surg 2002;37:1183–7. https://doi. org/10.1053/jpsu.2002.34468. [2] Takahashi A, Tsuchida Y, Suzuki N, Kuroiwa M, Ikeda H, Hirato J, et al. Incidence of intrahepatic biliary cysts in biliary atresia after hepatic portoenterostomy and associated histopathologic findings in the liver and porta hepatis at diagnosis. J Pediatr Surg 1999;34:1364–8. [3] Betz BW, Bisset GS, Johnson ND, Daugherty CC, Balistreri WF. MR imaging of biliary cysts children with biliary atresia: clinical associations correlation and pathologic correlation. AJR 1994;162:167–71. [4] Nakama T, Kitamura T, Matsui A, Makino S, Faruk O. Ultrasonographic findings and management of intrahepatic tract abnormalities after portoenterostomy. J Pediatr Surg 1991;1:32–6. [5] Tainaka T, Kaneko K, Nakamura S, Ono Y, Sumida W, Ando H. Histological assessment of bile lake formation after hepatic portoenterostomy for biliary atresia. Pediatr Surg Int 2008;24:265–9. https://doi.org/10.1007/s00383-007-2099-z. [6] Tainaka T, Kaneko K, Seo T, Ono Y, Sumida W, Ando H. Intrahepatic cystic lesions after hepatic portoenterostomy for biliary atresia with bile lake and dilated bile ducts. J Pediatr Gastroenterol Nutr 2007;104. 7. [7] Inoue Y, Kato Y, Tamura T, Kobayashi H, Ichikawa S, Lane GJ, et al. Prognostic implications of bile lakes after surgery for biliary atresia. J Pediatr Surg 2008;43:2165–8. https://doi.org/10.1016/j.jpedsurg.2008.08.039. [8] Lally KP, Kanegaye J, Matsumura M, Rosenthal P, Sinatra F, Atkinson JB. Perioperative factors affecting the outcome following repair of biliary atresia. 1989;83. [9] Saito S, Nishina T, Tsuchida Y. Intrahepatic cysts in biliary atresia after successful hepatoportoenterostomy. Arch Dis Child 1983;59:274–5. [10] Gleghorn EE, Rosenthal P, Vachon L, Diament M. Long-term external catheter biliary drainage for recurrent cholangitis after hepatoportoenterostomy. J Pediatr Gastroenterol Nutr 1986;5:485–8. [11] Werlin SL, Sty JR, Starshak RJ, Glicklich M, Nathan R. Intrahepatic biliary tract abnormalities in children with corrected extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr 1985;4:537–41. [12] Tsuchida Y, Honna T, Kawarasaki H. Cystic dilatation of the intrahepatic biliary system in biliary atresia after hepatic portoenterostomy. J Pediatr Surg 1994;29:630–4.
4. Conclusion Here we report the successful use of internal surgical drainage of a bile lake following HPE via cystjejunostomy to the prior Roux limb. Short term follow-up demonstrates adequate resolution of jaundice and avoidance of cholangitis with ongoing antibiotic prophylaxis. To the best of our knowledge, this is the first report to describe
73
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Internal surgical drainage for bile lakes following hepatoportoenterostomy for biliary atresia
T
Edward C. Deea, Benjamin Zendejasb, Rima Fawazc, Heung Bae Kimb,∗ a
Harvard Medical School, Boston, MA, USA Department of Pediatric Surgery, Boston Children's Hospital, Boston, MA, USA c Division of Gastroenterology and Nutrition, Boston Children's Hospital, Boston, MA, USA b
A R T I C LE I N FO
A B S T R A C T
Keywords: Bile lake Biliary atresia Cystjejunostomy
Bile lakes may be present at time of diagnosis or develop later following hepatoportoenterostomy (HPE) in patients with biliary atresia (BA). We report a patient with recurrent cholangitis following HPE who developed multiple large biliary cystic lesions. Percutaneous drainage resulted in resolution of jaundice and cholangitis but capping of drains resulted in recurrence of symptoms. Therefore, internal drainage of the bile lakes was accomplished using cystjejunostomy to the roux limb of the previous portoenterostomy. At 18 months of follow-up, the patient has remained jaundice free with only one recurrent cholangitis episode which occurred after stopping her antibiotic prophylaxis. Although this patient will likely require liver transplantation in the next few years, this case suggests that cystjejunostomy may be a viable surgical option to improve bile flow and prevent cholangitis in patients who develop bile lakes following HPE.
1. Introduction About 25% of patients with biliary atresia (BA) will develop at least one intrahepatic biliary cyst, or “bile lake” [1–4]. Bile lakes are thought to result from bile stasis and the formation of intrabiliary calculi, leading to repeated local inflammation, with subsequent damage to and fusion of bile ducts [5,6]. The presence of bile lakes is associated with a poor prognosis in patients with BA [1,6], possibly related to an increased risk of recurrent cholangitis [7] and progression to cirrhosis [6]. The Kasai hepatoportoenterostomy (HPE) was developed to allow for effective biliary drainage but outcomes depend on both adequate clearance of jaundice as well as avoidance of complications such as cholangitis [8]. Therefore, addressing bile lakes effectively may be an important adjunct to improve outcomes [9]. Percutaneous transhepatic drainage of bile lakes is an option for reducing rates of cholangitis in some patients [10], but safe and prolonged percutaneous drainage may be difficult in some cases [1,4]. In this case we report the use of cystjejunostomy as a viable long term surgical option to provide internal biliary drainage of bile lakes in a patient with BA following HPE. 2. Case report A 52-day female underwent HPE for BA without any other
malformations after she presented with cholestatic jaundice, acholic stools, and a non-excreting hepatobiliary iminodiacetic acid (HIDA) scan. Her abdominal ultrasound revealed a hypoplastic gallbladder without any evidence of intrahepatic abnormalities. At the time of her laparotomy done two days after presentation to our hospital, no identifiable gallbladder lumen was found so an intraoperative cholangiogram could not be performed. A Kasai HPE was completed, with bile drainage noted intraoperatively. Pathology revealed a fibrotic bile duct with patchy inflammatory infiltrate. The patient did well and was discharged home on the fifth postoperative day on fat soluble vitamins, ursodiol, and trimethoprim/sulfamethoxazole for cholangitis prophylaxis as per our practice. By two months postoperatively, her total and direct bilirubin were 0.8 and 0.4 mg/dl respectively. She developed cholangitis three months after her surgery. Her abdominal ultrasound revealed diffusely prominent periportal echoes in the liver thought to represent periportal edema or inflammation as well as ascites. She was treated with a prolonged course of antibiotics. Her total and direct bilirubin levels peaked at 14.8 and 11.8 mg/dl respectively. Follow up imaging done three months after her initial cholangitis revealed possible cystic lesions at the porta hepatis. She was referred for liver transplant evaluation; however, listing was deferred due to her clinical improvement, resolution of her ascites, and improving total and direct bilirubin at that
∗
Corresponding author. 300 Longwood Avenue, Fegan, 3rd Floor, Boston, MA 02115, USA. E-mail addresses: [email protected] (E.C. Dee), [email protected] (B. Zendejas), [email protected] (R. Fawaz), [email protected] (H.B. Kim). https://doi.org/10.1016/j.epsc.2019.02.015 Received 12 February 2019; Accepted 20 February 2019 Available online 25 February 2019 2213-5766/ © 2019 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Journal of Pediatric Surgery Case Reports 43 (2019) 71–73
E.C. Dee, et al.
Fig. 1. Magnetic resonance imaging demonstrating multiple intrahepatic fluid collections. These collections include branching fluid-filled structures consistent with dilated bile ducts (A) and multiple rounded fluid-filled structures originating distal to the dilated structures suggestive of bile lakes (B). Fig. 2. Intraoperative photograph of cystic bile lake (“bl”) and jejunum (“j”) before (A) and after (B) construction of cystjejunostomy (“*”).
time to 5.4 and 3.7 mg/dl respectively. She was followed by her local provider for persistent cholestasis and poor weight gain in spite of nasogastric feeds and formula fortification. Her stools remained pigmented. She presented to our institution six months after last seen with fever, cachexia, and a hard, large liver with a protuberant mass. She was admitted urgently and treated with intravenous broad spectrum antibiotics. Ultrasound suggested the presence of large intrahepatic cysts and magnetic resonance imaging (MRI) of her abdomen confirmed the presence of multiple branching fluid-filled structures throughout the liver consistent with dilated bile ducts and bile lakes (Fig. 1). Blood cultures grew Citrobacter Freundii complex 12 h later. Percutaneous placement of two separate drains into these bile lakes resulted in resolution of her infection and marked improvement in jaundice. Biliary cultures grew the same organism. Over the next several weeks, drain output stabilized with about 200 mL of bile drainage from each side per day. She received nutritional rehabilitation and was listed for liver
transplantation. After two months of antibiotics and multiple percutaneous drain replacements, repeat abdominal imaging demonstrated improvement in her biliary dilation; however, bile lakes were persistent despite adequate drainage (total bilirubin decreased to 1.1 mg/dl) and resolution of jaundice. Given the clinical and laboratory improvement with adequate biliary drainage, we felt that internal surgical drainage would benefit our patient, so she was inactivated on the liver transplant list. Upon re-exploration of her abdomen, moderate ascites and some features of portal hypertension were noted; the liver was firm with a micronodular appearance, which on biopsy demonstrated evidence of cholestasis and biliary-type cirrhosis. One of the bile lakes was noted on the surface of segments IVb/III where the anterior abdominal percutaneous drain was entering. The drain was removed and the cyst wall 72
Journal of Pediatric Surgery Case Reports 43 (2019) 71–73
E.C. Dee, et al.
cystjejunostomy to the prior portoenterostomy jejunal roux limb as a viable means to treat bile lakes following HPE in patients with biliary atresia.
was excised. The adjacent jejunal roux limb from the previous HPE was anastomosed to the cyst wall in side-to-side configuration to create a cystjejunostomy (Fig. 2). Of note, bile was noted both within the roux limb coming from the portoenterostomy as well as within the cyst. The other large posteriorly located bile lake that was being drained by the right flank percutaneous drain was not amenable to operative exposure without adding substantial morbidity, and was not pursued. She recovered well from this operation without apparent complications. The excised biliary cyst wall demonstrated benign fibrous tissue with no recognizable epithelial lining on pathology, consistent with a bile lake [6]. Subsequently, her right flank percutaneous drain fell out resulting in a biliary fistula that was managed with drainage into a stoma bag until it spontaneously closed several months later. After one cholangitis-free year, prophylactic antibiotic treatment (levofloxacin) was stopped. She developed cholangitis two months later and was retreated with antibiotics. At last follow-up, 18 months after surgery, she remains jaundice free (total and direct bilirubin pf 0.7 and 0.4 mg/dl, respectively) with adequate liver function and good growth and development.
Informed consent Consent to publish the case report was not obtained. This report does not contain any personal information that could lead to the identification of the patient. Informed consent was waived by our IRB due to the fact that the data collected for this study was for a case report and was de-identified. Authorship All authors attest that they meet the current ICMJE criteria for authorship. Edward C. Dee: Data collection, drafting article, critical revision of article. Benjamin Zendejas: Concept and design, data collection, drafting article, critical revision of article, approval of article. Rima Fawaz: Concept and design, data collection, drafting article, critical revision of article, approval of article. Heung Bae Kim: Concept and design, data collection, drafting article, critical revision of article, approval of article.
3. Discussion The etiology of bile lake formation in patients with biliary atresia is unclear. Tainaka et al. proposed the following mechanism for bile lake formation: bile stasis occurs in intrahepatic bile ducts leading to calculi formation within bile ducts; calculi result in recurrent cholangitis, which damages the bile ducts, which then fuse together and form bile lakes [5]. Bile lakes are defined as solitary cystic lesions with fibrotic walls lacking biliary epithelia, and were described as retention pseudocysts secondary to obstruction and damage of bile ducts [6]. Bile lakes are associated with recurrent cholangitis and progressive liver disease [6,7]. These are in contrast to biliary duct dilation secondary to HPE stricture, which is associated with a favorable prognosis given the possibility of interventions to treat the stricture [6]. Our patient had “benign fibrous tissue with no recognizable epithelial lining,” most consistent with Tainaka's histologic description of a true bile lake, for which adequate drainage may decrease the risk of progressive liver disease [6]. When cysts are single or communicating, percutaneous drainage may be effective at resolving cholangitis and jaundice [10,11]. Werlin et al. recommend percutaneous drainage as initial management with possible progression to surgical drainage if cholangitis does not remit [11]. They also recommend weighing the potential benefit of surgical drainage against the added complexity of liver transplantation that may result from additional operations [11]. Saito et al. reported a successful case of surgical drainage of bile lakes using Penrose drains to the abdominal wall with no recurrent episodes of fever or jaundice at 32 months' follow-up [9]. This patient was reported to be doing well 10 years postoperatively [12]. Tsuchida et al. report a successful similarly treated patient, and further report a literature review of eight cases of bile lakes treated with laparotomy either with simple drainage or internal intestinal drainage, though the method of internal intestinal drainage was not specified [12]. Nakama et al. report a patient in whom laparotomy and cyst drainage was performed as percutaneous drainage was deemed too dangerous but long-term follow-up was not reported [4].
Conflict of interest The following authors have no financial disclosures: ECD, BZ, RF, HBK. Funding No funding or grant support was received to conduct this study. References [1] Bu L-N, Chen H-L, Ni Y-H, Peng S, Jeng Y-M, Lai H-S. Multiple intrahepatic biliary cysts in children with biliary atresia. J Pediatr Surg 2002;37:1183–7. https://doi. org/10.1053/jpsu.2002.34468. [2] Takahashi A, Tsuchida Y, Suzuki N, Kuroiwa M, Ikeda H, Hirato J, et al. Incidence of intrahepatic biliary cysts in biliary atresia after hepatic portoenterostomy and associated histopathologic findings in the liver and porta hepatis at diagnosis. J Pediatr Surg 1999;34:1364–8. [3] Betz BW, Bisset GS, Johnson ND, Daugherty CC, Balistreri WF. MR imaging of biliary cysts children with biliary atresia: clinical associations correlation and pathologic correlation. AJR 1994;162:167–71. [4] Nakama T, Kitamura T, Matsui A, Makino S, Faruk O. Ultrasonographic findings and management of intrahepatic tract abnormalities after portoenterostomy. J Pediatr Surg 1991;1:32–6. [5] Tainaka T, Kaneko K, Nakamura S, Ono Y, Sumida W, Ando H. Histological assessment of bile lake formation after hepatic portoenterostomy for biliary atresia. Pediatr Surg Int 2008;24:265–9. https://doi.org/10.1007/s00383-007-2099-z. [6] Tainaka T, Kaneko K, Seo T, Ono Y, Sumida W, Ando H. Intrahepatic cystic lesions after hepatic portoenterostomy for biliary atresia with bile lake and dilated bile ducts. J Pediatr Gastroenterol Nutr 2007;104. 7. [7] Inoue Y, Kato Y, Tamura T, Kobayashi H, Ichikawa S, Lane GJ, et al. Prognostic implications of bile lakes after surgery for biliary atresia. J Pediatr Surg 2008;43:2165–8. https://doi.org/10.1016/j.jpedsurg.2008.08.039. [8] Lally KP, Kanegaye J, Matsumura M, Rosenthal P, Sinatra F, Atkinson JB. Perioperative factors affecting the outcome following repair of biliary atresia. 1989;83. [9] Saito S, Nishina T, Tsuchida Y. Intrahepatic cysts in biliary atresia after successful hepatoportoenterostomy. Arch Dis Child 1983;59:274–5. [10] Gleghorn EE, Rosenthal P, Vachon L, Diament M. Long-term external catheter biliary drainage for recurrent cholangitis after hepatoportoenterostomy. J Pediatr Gastroenterol Nutr 1986;5:485–8. [11] Werlin SL, Sty JR, Starshak RJ, Glicklich M, Nathan R. Intrahepatic biliary tract abnormalities in children with corrected extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr 1985;4:537–41. [12] Tsuchida Y, Honna T, Kawarasaki H. Cystic dilatation of the intrahepatic biliary system in biliary atresia after hepatic portoenterostomy. J Pediatr Surg 1994;29:630–4.
4. Conclusion Here we report the successful use of internal surgical drainage of a bile lake following HPE via cystjejunostomy to the prior Roux limb. Short term follow-up demonstrates adequate resolution of jaundice and avoidance of cholangitis with ongoing antibiotic prophylaxis. To the best of our knowledge, this is the first report to describe
73