J Med Assoc Thai 2014; 97 (11):122

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Clinical Differences between Choledochal Cysts in Infancy and Childhood: An Analysis of 160 Patients
Niramis R Mail, Narumitsuthon R , Watanatittan S , Anuntkosol M , Buranakitjaroen V , Tongsin A , Mahatharadol V , Laorwong S

Background: Choledochal cysts (CDC) can become symptomatic at any age. Clinical features of infants and older children are different and have been documented by many authors.
Objective: The aim of this report was to study the differences in clinical spectrum of CDC between the infants and children treated at our hospital during a 17-year period.
Material and Method: A retrospective study was conducted of 160 patients with CDC treated at Queen Sirikit National Institute of Child Health between 1996 and 2012. The patients were categorized into 2 groups based on age at clinical presentation: an infantile group (up to one year old) and a childhood group (over one year old). Clinical characteristics of patients in the infantile and childhood groups were compared with statistical analysis using the Chi-square and Fisher’s exact test.
Results: Of the 160 patients with CDC, 48 cases (30%) were categorized in the infantile group and 112 cases (70%) in the childhood group. Over three-quarters of the patients in the infantile group presented with jaundice and acholic stool, and this incidence was significantly higher than in the childhood group (77% vs. 46.4%, p = 0.001 and 50% vs. 13.4%, p<0.001). Abdominal pain was the most common symptom of patients in the childhood group, whereas it was noted in only a small number of those in the infantile group (82.1% vs. 8.3%, p<0.001). Average amylase level in CDC content was markedly elevated in the childhood group but much lower in the infantile group (43,630.5+90,234.5 vs. 79+189.9, p<0.001). Only type I and type IV CDC as defined by Todani’s classification were found in our patients, and there were no statistical differences in incidences of type I and type IV in the two groups (79.2% vs. 67.8% and 20.8% vs. 32.2% p>0.05). Neonates and infants with CDC had a significantly higher risk of liver cirrhosis than did the childhood group (25% vs. 8%, p<0.001). However, surviving patients with cirrhosis in both groups were doing well at least 3 years after surgical CDC excision.
Conclusion: Neonates and infants with CDC were more likely to present with jaundice and acholic stool, whereas older children were more likely to present with abdominal pain. Amylase level in CDC content was markedly elevated in the childhood group but at a much lower level in the infantile group. Neonates and infants with CDC tended to develop liver cirrhosis earlier and more often than older children.

Keywords: Choledochal cyst, Infancy, Childhood, Todani’s classification


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