Pediatric Choledochal Cyst Surgery Clinical Presentation

Updated: Nov 03, 2020
  • Author: Gail E Besner, MD; Chief Editor: Harsh Grewal, MD, FACS, FAAP  more...
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History and Physical Examination

Two distinct clinical groups of patients are recognized with regard to age at presentation. [32, 13, 5, 35, 36]  The first group is the infantile group consisting of babies younger than 1 year, with or without obvious hepatomegaly, with obstructive jaundice and acholic stools. This clinical picture is indistinguishable from that of biliary atresia in the absence of a palpable mass in the right side of the abdomen. However, the cystic mass can usually be detected either at clinical examination or on ultrasonography; this finding suggests a diagnosis of choledochal cyst.

In 1995, Todani et al found that 26 of 28 infants younger than 1 year had a mass, whereas only three of eight infants aged 13-24 months had a mass. [35] Other symptoms, such as vomiting, fever, and abdominal pain with hyperamylasemia, are extremely infrequent. [34] In infants with a prenatal diagnosis of choledochal cyst, jaundice often does not manifest until 1-3 weeks after birth. [39, 40]

In contrast, infants older than 1 year, with the so-called adult form of choledochal cyst, generally have one or more components of the classic triad: pain, jaundice, and a palpable mass. The entire triad is present in fewer than 30% of patients. [13] Jaundice is intermittent and often associated with vague abdominal pain. The pain has been described as being similar to that of cholangitis or recurrent mild pancreatitis. Undiagnosed choledochal cysts can lead to choledocholithiasis, cirrhosis with portal hypertension, cyst rupture, or biliary carcinomas.