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Intrahepatic biliary cyst after a Kasai procedure
Journal of Pediatric Surgery Case Reports 31 (2018) 32–35
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Intrahepatic biliary cyst after a Kasai procedure Naoya Sakamoto
a,b,∗
c
b
T d
, Toshihiro Muraji , Kouji Masumoto , Toshihiro Yanai , Kentaro Ono
b
a
Department of Pediatric Surgery, JA Toride Medical Center, 2-1-1, Hongoh, Toride, 302-0022, Ibaraki, Japan Department of Pediatric Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennoudai, Tsukuba, 305-8575, Ibaraki, Japan Department of Pediatric Surgery, Kirishima Medical Center, 3320, Hayato-cho Matsunaga, Kirishima, 899-5112, Kagoshima, Japan d Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito, 311-4145, Ibaraki, Japan b c
A B S T R A C T A 30-day-old female infant presented with a cystic lesion at the porta hepatis. The cyst was first detected on a prenatal ultrasound at 16 weeks of gestation and diagnosed as congenital biliary dilatation. The size of the cyst did not change over the course of gestation and the infant's liver function was stable after birth, although she had a persisting yellowish stool. Surgical cholangiography was performed at 30 days, and a complete obstruction of the hepatic hilum was identified as a type IIId cyst. However, cholangitis recurred at 10 months post-surgery, with marked dilation of the right and left hepatic ducts. Right cyst-intestinal anastomosis and left cyst-gastric anastomosis were performed. Cyst recurrence is sometimes a complication in patients with type III biliary atresia who undergo the Kasai procedure. The mechanisms of cyst formation are still unknown, and there is no consensus regarding the treatment. Our case suggests that cysto-enterstomy is a feasible treatment for recurrent cysts.
1. Introduction Since the first report regarding the use of portoenterostomy by Morio Kasai in 1959, various complications of the Kasai procedure have been reported. Intrahepatic biliary cysts (IBCs) are one of these complications, but there is no consensus regarding their treatment. IBC sometimes leads to severe cholangitis and subsequent liver transplantation. Although percutaneous transhepatic cholangio-drainage (PCTD) has been performed to drain bile, liver transplantation is sometimes required. A new treatment of recurrent cysts is essential for a good clinical course after a Kasai procedure in patients with biliary atresia (BA). We reported on a patient with an IBC that resulted in a good clinical course. Cyst-enterostomy may remedy the recurrence of cysts. 2. Case report The prenatal ultrasound of a female fetus at 16 weeks of gestation revealed a 31 × 23-mm cystic lesion at the hepatic hilum. The size of the cyst appeared to remain stable over the rest of gestation. The baby was born at full term via vaginal delivery and without complications. She was referred by the Department of Obstetrics of our institution to our medical unit, two days after birth. On routine examination, there was no evidence of heart murmur, hemi-vertebra, facial anomalies, or decrease in renal function, which excluded Alagille syndrome as a
∗
diagnosis. On ultrasound examination, a 26 × 25 mm cyst was observed at the hepatic hilum that was connected to the intrahepatic duct, which was not dilated. The gallbladder was identified, and its size was within the normal range. A blood analysis revealed jaundice (total bilirubin: 8.9 mg/dL, direct bilirubin: 5.2 mg/dL) and slight hepatic dysfunction (aspartate aminotransferase: 80 IU/L, alanine aminotransferase: 54 IU/L). The coagulation system was normal. The fetus's stool was yellowish, and the color did not change until immediately before surgery. Because we could not differentiate the lesion as congenital biliary dilatation or type I cystic BA, surgical exploration was planned to confirm the diagnosis and for treatment, as appropriate. Intraoperative cholangiography was performed on postnatal day 30 (Fig. 1), revealing atrophy of the gallbladder, with no patency of either the intrahepatic duct (Fig. 2a) or common bile duct (Fig. 2b). The operation was converted to portoenterostomy (Kasai procedure) for treatment. Following the Kasai procedure, we identified a smooth white structure resembling an eggshell membrane in the lumen of the cyst (Fig. 1b–c). This membrane was removed and intraoperative cholangiography was repeated, during which the intrahepatic bile duct could not be identified. A microscopic examination of the liver revealed the findings of low-grade hepatitis (giant cell transformation with ballooning and apoptosis of the hepatocytes) with mild fibrosis, showing that regeneration of the bile ductule and mild bile stasis were present. These findings were consistent with the diagnosis of BA.
Corresponding author. Department of Pediatric Surgery, JA Toride Medical Center, 2-1-1, Hongoh, Toride, 302-0022, Ibaraki, Japan. Tel.: +81 297 74 5551. E-mail address: [email protected] (N. Sakamoto).
https://doi.org/10.1016/j.epsc.2017.12.006 Received 30 September 2017; Received in revised form 2 December 2017; Accepted 9 December 2017 Available online 12 December 2017 2213-5766/ © 2017 The Authors. 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 31 (2018) 32–35
N. Sakamoto et al.
Fig. 1. The upper left panel (a) shows the cyst at the hepatic hilum, and the lumen of the cyst is shown in the upper right panel (b). A smooth, white structure, which was comparable to the membrane of an eggshell, is observable. The cavity within the white eggshell-like membrane is shown in the lower left panel (c). After resection of the membrane, which is shown in the lower right panel (d), complete obstruction of the common bile duct was confirmed.
Fig. 2. Intraoperative cholangiography shows complete obstruction of both the right and left hepatic ducts (a) and the common hepatic duct (b). Even after resection of the white membrane, the right and left hepatic ducts were completely obstructed.
3. Discussion
Postoperatively, the patient recovered well, including resolution of the jaundice after a short course of corticosteroid administration. However, cholangitis reoccurred 10 months after surgery. Considering the marked dilation of the right and left hepatic ducts (Fig. 3), we performed right cyst-intestinal anastomosis and left cyst-gastric anastomosis (Fig. 4). The cyst contained serous fluid that appeared to be lymphatic in nature, with no evidence of bilirubin (we could not collect a fluid sample; therefore, we could not determine that bile was present). The surgical procedure successfully corrected bile drainage. An immunoreactive D2-40 assessment of a tissue specimen from the cyst revealed the presence and proliferation of lymph vessels (Fig. 5). After surgery, profuse ascites persisted for two weeks, although a drainage tube was in place. One year after cystic-gastric and cystic-jejunum drainage were performed, the patient underwent a detailed computed tomography examination and upper gastrointestinal endoscopy, during which varicies, scarring of the stomach, and recurrent cyst formation were not identified. The patient did well clinically at 3 years post-surgery, with normal liver function.
Since Kasai reported the first use of portoenterostomy in 1959 [1], the procedure's profits and complications have been revealed [2]. Although the Kasai procedure gives patients a better clinical course, an IBC may appear after a Kasai operation in about 20% of patients with type III BA, and 50% of patients with an IBC required liver transplantation [3]. Although PTCD is used for drainage, it does not alleviate the IBC. Liver transplantation is the final solution. Ling-Nan et al. [4] cited the following as factors: (1) bile leakage during the intrahepatic fibroobliterative process; (2) an ongoing inflammatory process resulting in intrahepatic biliary obstruction, cholangitis, and bile cysts; (3) an exaggerated, irregular configuration of the intrahepatic bile ducts during cirrhotic changes of the liver; and (4) ductal plate malformation. In our case, several issues may have contributed to cyst recurrence after the initial Kasai procedure. First, resection of the bile duct in the initial procedure may have been insufficient, considering that the bile ducts were patent into the deep bifurcations (Fig. 1d). The entire 33
Journal of Pediatric Surgery Case Reports 31 (2018) 32–35
N. Sakamoto et al.
internal pressure of lymphoid fluid could cause cyst recurrence. We were unable to confirm the type of fluid within the cyst in our patient due to technical difficulties. Even if the main component is lymphoid fluid instead of bilirubin, lymphoid fluid can lead the reduction of jaundice [5]. Our cyst-enterostomy procedure has advantages and disadvantages. Its disadvantages include operative stress. A Roux-en-Y bypass that was performed during porto-enterostomy caused the lack of enough intestine to perform a new rout. We managed to treat the patient using cystgastric anastomosis instead of cyst-intestinal anastomosis. Another concern is ulcer or cancer. However, the advantage of the procedure is that a great amount of permanent drainage can be achieved. Furthermore, cyst-enterostomy leads to more efficient lymphoid flow than bile flow. This case also offers valuable insight regarding the origin of cystic BA. First, type I cysts are commonly identified as a natural component of correctable BA [6]. However, cysts can metamorphose during gestation and, as reported by Masumoto et al. [7], can transform from a correctable to a non-correctable type. Similarly, Fujishiro et al. [8] reported a case in which a cyst of the hepatic hilum was diagnosed antenatally, but disappeared over the subsequent period of prenatal observation, resulting in an uncorrectable type IIId BA. As Komuro et al. [9] reported, the absence of bile flow may lead to type I BA without cyst formation, and the severity of inflammation could cause clinical variation in the presentation of the disease. In these cases, the blockage of bile flow is considered to result from high intrahepatic pressure.
Fig. 3. Recurring cysts in both the right and left hepatic hilar region.
closure point of the bile duct should be resected to eliminate the risk of cyst recurrence. Second, the intrahepatic pressure may have been sufficiently high to enlarge the cystic remnant. The pathology of the liver and cyst showed proliferation of lymphoid ducts. This indicates that the
Fig. 4. A schematic illustration of the cystic-jejunum and cystic-gastric anastomosis procedures that were performed.
Fig. 5. The left panel (a) shows the cyst tissue that was stained with D2-40, which was a marker of the endothelium on the lymphoid duct. The right panel (b) shows the specimen of a liver biopsy that was obtained during surgery, showing evidence of lymph duct proliferation.
34
Journal of Pediatric Surgery Case Reports 31 (2018) 32–35
N. Sakamoto et al.
4. Conclusion
References
We reported the case of a patient with BA with recurrence of the cyst at the hepatic hilum. We demonstrated that cyst-enterostomy could be performed instead of PTCD. A large and permanent drainage route is essential for not only bile flow but also lymphoid flow.
[1] Kasai M. A new operation for “non-correctable” biliary atresia: Hepatic portoenterostomy. Shujutsu 1959;13:733–9. [2] Wildhaber BE. Biliary atresia: 50 years after the first Kasai. ISRN Surg 2012;2012:132089. [3] Watanabe M, et al. Intrahepatic biliary cysts in children with biliary atresia who have had a Kasai operation. J Pediatr Surg 2007;42(7):1185–9. [4] Bu LN, et al. Multiple intrahepatic biliary cysts in children with biliary atresia. J Pediatr Surg 2002;37(8):1183–7. [5] Fonkalsrud EW, Kitagawa S, Longmire WP. Hepatic lymphatic drainage to the jejunum for congenital biliary atresia. Am J Surg 1966;112(2):188–94. [6] Nio M, et al. Long-term outcome in type I biliary atresia. J Pediatr Surg 2006;41(12):1973–5. [7] Masumoto K, et al. A case of cystic biliary atresia with an antenatally detected cyst: the possibility of changing from a correctable type with a cystic lesion (I cyst) to an uncorrectable one (IIId). Pediatr Surg Int 2011;27(1):99–102. [8] Fujishiro J, et al. Disappearing cyst of the hepatic hilum in uncorrectable biliary atresia. Pediatr Surg Int 2005;21(2):116–8. [9] Komuro H, et al. Type I biliary atresia without extrahepatic biliary cyst. Pediatr Radiol 2008;38(8):907–8.
Consent for publication The parents of the patient provided consent to publish this article.
Competing interests All authors do not have any financial competing interests related to the publication of this case to report.
35
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Intrahepatic biliary cyst after a Kasai procedure Naoya Sakamoto
a,b,∗
c
b
T d
, Toshihiro Muraji , Kouji Masumoto , Toshihiro Yanai , Kentaro Ono
b
a
Department of Pediatric Surgery, JA Toride Medical Center, 2-1-1, Hongoh, Toride, 302-0022, Ibaraki, Japan Department of Pediatric Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennoudai, Tsukuba, 305-8575, Ibaraki, Japan Department of Pediatric Surgery, Kirishima Medical Center, 3320, Hayato-cho Matsunaga, Kirishima, 899-5112, Kagoshima, Japan d Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito, 311-4145, Ibaraki, Japan b c
A B S T R A C T A 30-day-old female infant presented with a cystic lesion at the porta hepatis. The cyst was first detected on a prenatal ultrasound at 16 weeks of gestation and diagnosed as congenital biliary dilatation. The size of the cyst did not change over the course of gestation and the infant's liver function was stable after birth, although she had a persisting yellowish stool. Surgical cholangiography was performed at 30 days, and a complete obstruction of the hepatic hilum was identified as a type IIId cyst. However, cholangitis recurred at 10 months post-surgery, with marked dilation of the right and left hepatic ducts. Right cyst-intestinal anastomosis and left cyst-gastric anastomosis were performed. Cyst recurrence is sometimes a complication in patients with type III biliary atresia who undergo the Kasai procedure. The mechanisms of cyst formation are still unknown, and there is no consensus regarding the treatment. Our case suggests that cysto-enterstomy is a feasible treatment for recurrent cysts.
1. Introduction Since the first report regarding the use of portoenterostomy by Morio Kasai in 1959, various complications of the Kasai procedure have been reported. Intrahepatic biliary cysts (IBCs) are one of these complications, but there is no consensus regarding their treatment. IBC sometimes leads to severe cholangitis and subsequent liver transplantation. Although percutaneous transhepatic cholangio-drainage (PCTD) has been performed to drain bile, liver transplantation is sometimes required. A new treatment of recurrent cysts is essential for a good clinical course after a Kasai procedure in patients with biliary atresia (BA). We reported on a patient with an IBC that resulted in a good clinical course. Cyst-enterostomy may remedy the recurrence of cysts. 2. Case report The prenatal ultrasound of a female fetus at 16 weeks of gestation revealed a 31 × 23-mm cystic lesion at the hepatic hilum. The size of the cyst appeared to remain stable over the rest of gestation. The baby was born at full term via vaginal delivery and without complications. She was referred by the Department of Obstetrics of our institution to our medical unit, two days after birth. On routine examination, there was no evidence of heart murmur, hemi-vertebra, facial anomalies, or decrease in renal function, which excluded Alagille syndrome as a
∗
diagnosis. On ultrasound examination, a 26 × 25 mm cyst was observed at the hepatic hilum that was connected to the intrahepatic duct, which was not dilated. The gallbladder was identified, and its size was within the normal range. A blood analysis revealed jaundice (total bilirubin: 8.9 mg/dL, direct bilirubin: 5.2 mg/dL) and slight hepatic dysfunction (aspartate aminotransferase: 80 IU/L, alanine aminotransferase: 54 IU/L). The coagulation system was normal. The fetus's stool was yellowish, and the color did not change until immediately before surgery. Because we could not differentiate the lesion as congenital biliary dilatation or type I cystic BA, surgical exploration was planned to confirm the diagnosis and for treatment, as appropriate. Intraoperative cholangiography was performed on postnatal day 30 (Fig. 1), revealing atrophy of the gallbladder, with no patency of either the intrahepatic duct (Fig. 2a) or common bile duct (Fig. 2b). The operation was converted to portoenterostomy (Kasai procedure) for treatment. Following the Kasai procedure, we identified a smooth white structure resembling an eggshell membrane in the lumen of the cyst (Fig. 1b–c). This membrane was removed and intraoperative cholangiography was repeated, during which the intrahepatic bile duct could not be identified. A microscopic examination of the liver revealed the findings of low-grade hepatitis (giant cell transformation with ballooning and apoptosis of the hepatocytes) with mild fibrosis, showing that regeneration of the bile ductule and mild bile stasis were present. These findings were consistent with the diagnosis of BA.
Corresponding author. Department of Pediatric Surgery, JA Toride Medical Center, 2-1-1, Hongoh, Toride, 302-0022, Ibaraki, Japan. Tel.: +81 297 74 5551. E-mail address: [email protected] (N. Sakamoto).
https://doi.org/10.1016/j.epsc.2017.12.006 Received 30 September 2017; Received in revised form 2 December 2017; Accepted 9 December 2017 Available online 12 December 2017 2213-5766/ © 2017 The Authors. 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 31 (2018) 32–35
N. Sakamoto et al.
Fig. 1. The upper left panel (a) shows the cyst at the hepatic hilum, and the lumen of the cyst is shown in the upper right panel (b). A smooth, white structure, which was comparable to the membrane of an eggshell, is observable. The cavity within the white eggshell-like membrane is shown in the lower left panel (c). After resection of the membrane, which is shown in the lower right panel (d), complete obstruction of the common bile duct was confirmed.
Fig. 2. Intraoperative cholangiography shows complete obstruction of both the right and left hepatic ducts (a) and the common hepatic duct (b). Even after resection of the white membrane, the right and left hepatic ducts were completely obstructed.
3. Discussion
Postoperatively, the patient recovered well, including resolution of the jaundice after a short course of corticosteroid administration. However, cholangitis reoccurred 10 months after surgery. Considering the marked dilation of the right and left hepatic ducts (Fig. 3), we performed right cyst-intestinal anastomosis and left cyst-gastric anastomosis (Fig. 4). The cyst contained serous fluid that appeared to be lymphatic in nature, with no evidence of bilirubin (we could not collect a fluid sample; therefore, we could not determine that bile was present). The surgical procedure successfully corrected bile drainage. An immunoreactive D2-40 assessment of a tissue specimen from the cyst revealed the presence and proliferation of lymph vessels (Fig. 5). After surgery, profuse ascites persisted for two weeks, although a drainage tube was in place. One year after cystic-gastric and cystic-jejunum drainage were performed, the patient underwent a detailed computed tomography examination and upper gastrointestinal endoscopy, during which varicies, scarring of the stomach, and recurrent cyst formation were not identified. The patient did well clinically at 3 years post-surgery, with normal liver function.
Since Kasai reported the first use of portoenterostomy in 1959 [1], the procedure's profits and complications have been revealed [2]. Although the Kasai procedure gives patients a better clinical course, an IBC may appear after a Kasai operation in about 20% of patients with type III BA, and 50% of patients with an IBC required liver transplantation [3]. Although PTCD is used for drainage, it does not alleviate the IBC. Liver transplantation is the final solution. Ling-Nan et al. [4] cited the following as factors: (1) bile leakage during the intrahepatic fibroobliterative process; (2) an ongoing inflammatory process resulting in intrahepatic biliary obstruction, cholangitis, and bile cysts; (3) an exaggerated, irregular configuration of the intrahepatic bile ducts during cirrhotic changes of the liver; and (4) ductal plate malformation. In our case, several issues may have contributed to cyst recurrence after the initial Kasai procedure. First, resection of the bile duct in the initial procedure may have been insufficient, considering that the bile ducts were patent into the deep bifurcations (Fig. 1d). The entire 33
Journal of Pediatric Surgery Case Reports 31 (2018) 32–35
N. Sakamoto et al.
internal pressure of lymphoid fluid could cause cyst recurrence. We were unable to confirm the type of fluid within the cyst in our patient due to technical difficulties. Even if the main component is lymphoid fluid instead of bilirubin, lymphoid fluid can lead the reduction of jaundice [5]. Our cyst-enterostomy procedure has advantages and disadvantages. Its disadvantages include operative stress. A Roux-en-Y bypass that was performed during porto-enterostomy caused the lack of enough intestine to perform a new rout. We managed to treat the patient using cystgastric anastomosis instead of cyst-intestinal anastomosis. Another concern is ulcer or cancer. However, the advantage of the procedure is that a great amount of permanent drainage can be achieved. Furthermore, cyst-enterostomy leads to more efficient lymphoid flow than bile flow. This case also offers valuable insight regarding the origin of cystic BA. First, type I cysts are commonly identified as a natural component of correctable BA [6]. However, cysts can metamorphose during gestation and, as reported by Masumoto et al. [7], can transform from a correctable to a non-correctable type. Similarly, Fujishiro et al. [8] reported a case in which a cyst of the hepatic hilum was diagnosed antenatally, but disappeared over the subsequent period of prenatal observation, resulting in an uncorrectable type IIId BA. As Komuro et al. [9] reported, the absence of bile flow may lead to type I BA without cyst formation, and the severity of inflammation could cause clinical variation in the presentation of the disease. In these cases, the blockage of bile flow is considered to result from high intrahepatic pressure.
Fig. 3. Recurring cysts in both the right and left hepatic hilar region.
closure point of the bile duct should be resected to eliminate the risk of cyst recurrence. Second, the intrahepatic pressure may have been sufficiently high to enlarge the cystic remnant. The pathology of the liver and cyst showed proliferation of lymphoid ducts. This indicates that the
Fig. 4. A schematic illustration of the cystic-jejunum and cystic-gastric anastomosis procedures that were performed.
Fig. 5. The left panel (a) shows the cyst tissue that was stained with D2-40, which was a marker of the endothelium on the lymphoid duct. The right panel (b) shows the specimen of a liver biopsy that was obtained during surgery, showing evidence of lymph duct proliferation.
34
Journal of Pediatric Surgery Case Reports 31 (2018) 32–35
N. Sakamoto et al.
4. Conclusion
References
We reported the case of a patient with BA with recurrence of the cyst at the hepatic hilum. We demonstrated that cyst-enterostomy could be performed instead of PTCD. A large and permanent drainage route is essential for not only bile flow but also lymphoid flow.
[1] Kasai M. A new operation for “non-correctable” biliary atresia: Hepatic portoenterostomy. Shujutsu 1959;13:733–9. [2] Wildhaber BE. Biliary atresia: 50 years after the first Kasai. ISRN Surg 2012;2012:132089. [3] Watanabe M, et al. Intrahepatic biliary cysts in children with biliary atresia who have had a Kasai operation. J Pediatr Surg 2007;42(7):1185–9. [4] Bu LN, et al. Multiple intrahepatic biliary cysts in children with biliary atresia. J Pediatr Surg 2002;37(8):1183–7. [5] Fonkalsrud EW, Kitagawa S, Longmire WP. Hepatic lymphatic drainage to the jejunum for congenital biliary atresia. Am J Surg 1966;112(2):188–94. [6] Nio M, et al. Long-term outcome in type I biliary atresia. J Pediatr Surg 2006;41(12):1973–5. [7] Masumoto K, et al. A case of cystic biliary atresia with an antenatally detected cyst: the possibility of changing from a correctable type with a cystic lesion (I cyst) to an uncorrectable one (IIId). Pediatr Surg Int 2011;27(1):99–102. [8] Fujishiro J, et al. Disappearing cyst of the hepatic hilum in uncorrectable biliary atresia. Pediatr Surg Int 2005;21(2):116–8. [9] Komuro H, et al. Type I biliary atresia without extrahepatic biliary cyst. Pediatr Radiol 2008;38(8):907–8.
Consent for publication The parents of the patient provided consent to publish this article.
Competing interests All authors do not have any financial competing interests related to the publication of this case to report.
35