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Biliary cysts in children--long-term follow-up in Taiwan
Hung-Chang Lee1, Chun-Yan Yeung, Shiuh-Bin Fang
1Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan, ROC. ped2435@ms2.mmh.org.tw
Insights
Biliary cysts in children require careful diagnosis, especially small ones with jaundice. Postoperative monitoring is crucial for non-biliary atresia-associated choledochal cysts due to potential complications like cholangitis and elevated ALT levels.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Biliary cysts are congenital anomalies requiring analysis of clinical presentation.
- Associated extrahepatic biliary atresia (BABC) is a critical consideration in pediatric biliary cysts.
- Taiwanese children with biliary cysts present unique clinical and epidemiological data.
Purpose of the Study:
- To analyze clinical presentation, postoperative outcomes, and associated biliary atresia in pediatric biliary cysts.
- To differentiate outcomes between biliary atresia-associated biliary cyst (BABC) and non-biliary atresia-associated choledochal cyst (NBACC) in infants and older children.
- To identify risk factors and complications associated with different types of biliary cysts.
Main Methods:
- Retrospective review of 158 pediatric patients with biliary cysts (1981-2004).
- Patients categorized into BABC, infantile NBACC, and late NBACC groups.
- Analysis of clinical data, surgical outcomes, morbidity, mortality, and long-term follow-up.
Main Results:
- BABC constituted 36.2% of infantile biliary cysts.
- Late NBACC cysts were significantly larger than infantile NBACC and BABC cysts.
- Cholangitis was the leading cause of early postoperative death; liver cirrhosis and intrahepatic dilatation were significant long-term complications in BABC and NBACC, respectively.
Conclusions:
- Small (<8 mm) extrahepatic cysts with prolonged jaundice in infants warrant consideration for BABC.
- Long-term follow-up for NBACC is vital due to potential for persistent high ALT levels and intrahepatic dilatation.
- Cholangitis remains a major cause of mortality within three months post-surgery for biliary cysts.
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.
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