Biliary cysts in children - Long term follow-up in Taiwan

Hung Chang Lee, Chun Yan Yeung, Shiuh Bin Fang, Chuen Bin Jiang, Jin Cherng Sheu, Nien Lu Wang

研究成果: 雜誌貢獻文章

9 引文 (Scopus)

摘要

Background: This study analyzed the clinical presentation, postoperative morbidity and mortality and incidence of associated extrahepatic biliary atresia in children with biliary cysts in Taiwan. Methods: We retrospectively reviewed the records of 158 pediatric patients with biliary cysts seen between June 1981 and July 2004, with follow-up ranging from 12 months to 22 years (mean, 11.2 ± 6.1 years). Patients were divided into three groups: biliary atresia-associated biliary cyst (BABC, 21 patients), non-biliary atresia-associated choledochal cyst (NBACC) in infancy (37 patients), and late NBACC (> 1 year of age, 100 patients). Results: BABC accounted for 36.2% of the infantile biliary cysts in this study. Extrahepatic cysts in late NBACC had a greater mean diameter than those in infantile NBACC and BABC (21.5 mm vs. 16.0 mm vs. 7.9 mm, p <0.001). Cholangitis was the most serious complication within 3 months postoperatively in all three groups, resulting in four deaths (two in the infantile NBACC group and one each in the other two groups). Liver cirrhosis developed during long-term follow-up in nine of the 21 patients with BABC, four of whom died. Three of these nine patients underwent liver transplantation and remained well during follow-up. Chronic complications in NBACC occurred mainly in late IVa cases, with persistent intrahepatic dilatation developing in 12 of 35 patients and intrahepatic stones in five. Elevation of serum alanine aminotransferase (ALT) was found preoperatively in 85% of late NBACC and 35% of infantile NBACC cases. Postoperative normalization of ALT occurred after a mean of 152 ± 23 days and 158 ± 67 days in late NBACC and infantile NBACC, respectively. Higher ALT levels before operation were associated with a longer period until normalization. Conclusion: The possibility of BABC must be included in the differential diagnosis when a small extrahepatic cyst (<8 mm in diameter) with prolonged jaundice is found in infancy. Postoperative follow-up is essential for patients with NBACC due to their frequently prolonged elevation of serum ALT and possibility of residual intrahepatic dilatation. Cholangitis was the major cause of death within 3 months postoperatively in this study.
原文英語
頁(從 - 到)118-124
頁數7
期刊Journal of the Formosan Medical Association = Taiwan yi zhi
105
發行號2
出版狀態已發佈 - 二月 2006
對外發佈Yes

指紋

Choledochal Cyst
Taiwan
Cysts
Alanine Transaminase
Biliary Atresia
Cholangitis
Dilatation
Jaundice
Serum
Liver Cirrhosis
Liver Transplantation
Cause of Death
Differential Diagnosis

ASJC Scopus subject areas

  • Medicine(all)

引用此文

Lee, H. C., Yeung, C. Y., Fang, S. B., Jiang, C. B., Sheu, J. C., & Wang, N. L. (2006). Biliary cysts in children - Long term follow-up in Taiwan. Journal of the Formosan Medical Association = Taiwan yi zhi, 105(2), 118-124.

Biliary cysts in children - Long term follow-up in Taiwan. / Lee, Hung Chang; Yeung, Chun Yan; Fang, Shiuh Bin; Jiang, Chuen Bin; Sheu, Jin Cherng; Wang, Nien Lu.

於: Journal of the Formosan Medical Association = Taiwan yi zhi, 卷 105, 編號 2, 02.2006, p. 118-124.

研究成果: 雜誌貢獻文章

Lee, HC, Yeung, CY, Fang, SB, Jiang, CB, Sheu, JC & Wang, NL 2006, 'Biliary cysts in children - Long term follow-up in Taiwan', Journal of the Formosan Medical Association = Taiwan yi zhi, 卷 105, 編號 2, 頁 118-124.
Lee, Hung Chang ; Yeung, Chun Yan ; Fang, Shiuh Bin ; Jiang, Chuen Bin ; Sheu, Jin Cherng ; Wang, Nien Lu. / Biliary cysts in children - Long term follow-up in Taiwan. 於: Journal of the Formosan Medical Association = Taiwan yi zhi. 2006 ; 卷 105, 編號 2. 頁 118-124.
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title = "Biliary cysts in children - Long term follow-up in Taiwan",
abstract = "Background: This study analyzed the clinical presentation, postoperative morbidity and mortality and incidence of associated extrahepatic biliary atresia in children with biliary cysts in Taiwan. Methods: We retrospectively reviewed the records of 158 pediatric patients with biliary cysts seen between June 1981 and July 2004, with follow-up ranging from 12 months to 22 years (mean, 11.2 ± 6.1 years). Patients were divided into three groups: biliary atresia-associated biliary cyst (BABC, 21 patients), non-biliary atresia-associated choledochal cyst (NBACC) in infancy (37 patients), and late NBACC (> 1 year of age, 100 patients). Results: BABC accounted for 36.2{\%} of the infantile biliary cysts in this study. Extrahepatic cysts in late NBACC had a greater mean diameter than those in infantile NBACC and BABC (21.5 mm vs. 16.0 mm vs. 7.9 mm, p <0.001). Cholangitis was the most serious complication within 3 months postoperatively in all three groups, resulting in four deaths (two in the infantile NBACC group and one each in the other two groups). Liver cirrhosis developed during long-term follow-up in nine of the 21 patients with BABC, four of whom died. Three of these nine patients underwent liver transplantation and remained well during follow-up. Chronic complications in NBACC occurred mainly in late IVa cases, with persistent intrahepatic dilatation developing in 12 of 35 patients and intrahepatic stones in five. Elevation of serum alanine aminotransferase (ALT) was found preoperatively in 85{\%} of late NBACC and 35{\%} of infantile NBACC cases. Postoperative normalization of ALT occurred after a mean of 152 ± 23 days and 158 ± 67 days in late NBACC and infantile NBACC, respectively. Higher ALT levels before operation were associated with a longer period until normalization. Conclusion: The possibility of BABC must be included in the differential diagnosis when a small extrahepatic cyst (<8 mm in diameter) with prolonged jaundice is found in infancy. Postoperative follow-up is essential for patients with NBACC due to their frequently prolonged elevation of serum ALT and possibility of residual intrahepatic dilatation. Cholangitis was the major cause of death within 3 months postoperatively in this study.",
keywords = "Biliary atresia, Biliary atresia-associated biliary cyst, Biliary cyst, Non-biliary atresia-associated choledochal cyst",
author = "Lee, {Hung Chang} and Yeung, {Chun Yan} and Fang, {Shiuh Bin} and Jiang, {Chuen Bin} and Sheu, {Jin Cherng} and Wang, {Nien Lu}",
year = "2006",
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pages = "118--124",
journal = "Journal of the Formosan Medical Association",
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T1 - Biliary cysts in children - Long term follow-up in Taiwan

AU - Lee, Hung Chang

AU - Yeung, Chun Yan

AU - Fang, Shiuh Bin

AU - Jiang, Chuen Bin

AU - Sheu, Jin Cherng

AU - Wang, Nien Lu

PY - 2006/2

Y1 - 2006/2

N2 - Background: This study analyzed the clinical presentation, postoperative morbidity and mortality and incidence of associated extrahepatic biliary atresia in children with biliary cysts in Taiwan. Methods: We retrospectively reviewed the records of 158 pediatric patients with biliary cysts seen between June 1981 and July 2004, with follow-up ranging from 12 months to 22 years (mean, 11.2 ± 6.1 years). Patients were divided into three groups: biliary atresia-associated biliary cyst (BABC, 21 patients), non-biliary atresia-associated choledochal cyst (NBACC) in infancy (37 patients), and late NBACC (> 1 year of age, 100 patients). Results: BABC accounted for 36.2% of the infantile biliary cysts in this study. Extrahepatic cysts in late NBACC had a greater mean diameter than those in infantile NBACC and BABC (21.5 mm vs. 16.0 mm vs. 7.9 mm, p <0.001). Cholangitis was the most serious complication within 3 months postoperatively in all three groups, resulting in four deaths (two in the infantile NBACC group and one each in the other two groups). Liver cirrhosis developed during long-term follow-up in nine of the 21 patients with BABC, four of whom died. Three of these nine patients underwent liver transplantation and remained well during follow-up. Chronic complications in NBACC occurred mainly in late IVa cases, with persistent intrahepatic dilatation developing in 12 of 35 patients and intrahepatic stones in five. Elevation of serum alanine aminotransferase (ALT) was found preoperatively in 85% of late NBACC and 35% of infantile NBACC cases. Postoperative normalization of ALT occurred after a mean of 152 ± 23 days and 158 ± 67 days in late NBACC and infantile NBACC, respectively. Higher ALT levels before operation were associated with a longer period until normalization. Conclusion: The possibility of BABC must be included in the differential diagnosis when a small extrahepatic cyst (<8 mm in diameter) with prolonged jaundice is found in infancy. Postoperative follow-up is essential for patients with NBACC due to their frequently prolonged elevation of serum ALT and possibility of residual intrahepatic dilatation. Cholangitis was the major cause of death within 3 months postoperatively in this study.

AB - Background: This study analyzed the clinical presentation, postoperative morbidity and mortality and incidence of associated extrahepatic biliary atresia in children with biliary cysts in Taiwan. Methods: We retrospectively reviewed the records of 158 pediatric patients with biliary cysts seen between June 1981 and July 2004, with follow-up ranging from 12 months to 22 years (mean, 11.2 ± 6.1 years). Patients were divided into three groups: biliary atresia-associated biliary cyst (BABC, 21 patients), non-biliary atresia-associated choledochal cyst (NBACC) in infancy (37 patients), and late NBACC (> 1 year of age, 100 patients). Results: BABC accounted for 36.2% of the infantile biliary cysts in this study. Extrahepatic cysts in late NBACC had a greater mean diameter than those in infantile NBACC and BABC (21.5 mm vs. 16.0 mm vs. 7.9 mm, p <0.001). Cholangitis was the most serious complication within 3 months postoperatively in all three groups, resulting in four deaths (two in the infantile NBACC group and one each in the other two groups). Liver cirrhosis developed during long-term follow-up in nine of the 21 patients with BABC, four of whom died. Three of these nine patients underwent liver transplantation and remained well during follow-up. Chronic complications in NBACC occurred mainly in late IVa cases, with persistent intrahepatic dilatation developing in 12 of 35 patients and intrahepatic stones in five. Elevation of serum alanine aminotransferase (ALT) was found preoperatively in 85% of late NBACC and 35% of infantile NBACC cases. Postoperative normalization of ALT occurred after a mean of 152 ± 23 days and 158 ± 67 days in late NBACC and infantile NBACC, respectively. Higher ALT levels before operation were associated with a longer period until normalization. Conclusion: The possibility of BABC must be included in the differential diagnosis when a small extrahepatic cyst (<8 mm in diameter) with prolonged jaundice is found in infancy. Postoperative follow-up is essential for patients with NBACC due to their frequently prolonged elevation of serum ALT and possibility of residual intrahepatic dilatation. Cholangitis was the major cause of death within 3 months postoperatively in this study.

KW - Biliary atresia

KW - Biliary atresia-associated biliary cyst

KW - Biliary cyst

KW - Non-biliary atresia-associated choledochal cyst

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