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Choledochal cyst in infancy: a follow-up study
M T Cheng1, M H Chang, H Y Hsu
1Department of Pediatrics, Surgery, National Taiwan University Hospital, Taipei.
Insights
Early diagnosis of choledochal cysts in infants, especially when associated with biliary atresia, is crucial. Ultrasonography aids detection, and prompt surgery improves outcomes, reducing risks like liver cirrhosis.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Imaging
Background:
- Choledochal cysts are congenital bile duct dilatations, posing risks of complications like biliary cirrhosis and cholangitis.
- Early diagnosis and management are vital for improving long-term outcomes in affected infants.
- The association between choledochal cysts and biliary atresia requires specific consideration in diagnosis and treatment.
Purpose of the Study:
- To analyze the clinical characteristics, diagnostic methods, and treatment outcomes of infants diagnosed with choledochal cysts.
- To investigate the impact of associated biliary atresia on the presentation and prognosis of choledochal cysts.
- To evaluate the effectiveness of ultrasonography in early detection and surgical management strategies.
Main Methods:
- Retrospective review of 19 infants diagnosed with choledochal cysts between January 1980 and February 1997.
- Classification of cysts based on Todani's system and grouping based on the presence or absence of biliary atresia.
- Analysis of diagnostic methods (primarily ultrasonography), surgical interventions (Roux-en-Y, Kasai operation), and follow-up data.
Main Results:
- Ultrasonography diagnosed 79% of cases before 6 months old, including prenatal diagnoses.
- Type Ia choledochal cysts were most common (74%).
- In infants with biliary atresia (37%), jaundice and pale stools were consistent; delayed treatment led to cirrhosis and mortality in some cases. Of 12 infants without biliary atresia, most had good outcomes, though some died due to anomalies or delayed surgery.
Conclusions:
- The potential association of biliary atresia with choledochal cysts warrants careful evaluation as a distinct clinical entity.
- Ultrasonography is a valuable tool for prenatal and infant diagnosis of choledochal cysts.
- Early surgical intervention is critical for infants with persistent jaundice and pale stools to prevent complications like biliary cirrhosis and reduce mortality.
Abstract:
From January 1980 to February 1997, 19 cases, 8 males and 11 females, of choledochal cyst were diagnosed before one year old. The majority of patients were diagnosed by ultrasonography before 6 months old (15/19; 79%), including two diagnosed prenatally. According to Todani's classification, type Ia was the most common (74%), followed by type Ic (26%). Fourteen patients underwent Roux-en-Y choledocho- or hepatico-jejunostomy and cyst excision, 3 patients underwent Kasai operation, and I patient underwent external biliary drainage only. The remaining one patient with Trisomy 18 anomaly refused operation. Four of the 10 patients in whom liver histologic examinations were performed, had liver cirrhosis. The follow-up period of these patients ranged from 6 months to 9 years, with a mean of 4.1 years. We divided these 19 cases into 2 groups, according to the presence or absence of biliary atresia. In the 7 infants with biliary atresia (37%), all presented with jaundice and alcoholic stool. Two patients died due to delayed presentation and surgery, both had liver cirrhosis. One patient is living with liver cirrhosis. Another patient was lost to follow-up, but frequent cholangitis was noted till 8 months old. The remaining 3 patients are living and well. In the 12 without biliary atresia, 9 patients are living and well. Two patients died, one due to Trisomy 18 anomaly and the other with delayed surgery and liver cirrhosis. One case was lost to follow-up. In summary: 1-) a possibility of the association of biliary atresia in infants with choledochal cyst should be carefully searched and considered as a unique group; 2) ultrasonography is a good diagnostic tool in choledochal cyst during prenatal or infancy period; 3) the mortality cases were characterized by prolonged bile stasis, biliary cirrhosis, delayed surgery, or multiple anomalies; 4) surgery should be performed as early as possible for those with persistent jaundice and light colored stools.