Choledochal Cyst

 

Authors:
Nicole Zeky, DO; Emily Klepper, MD; and Elizabeth McDonough, MD

Pediatric Residency Program, Our Lady of the Lake Children’s Hospital, Baton Rouge, Louisiana

Citation:
Zeky N, Klepper E, McDonough E. Choledochal cyst [published online October 17, 2019]. Gastroenterology Consultant.

 

A 3-year-old girl presented to the emergency department (ED) with abdominal pain of 1 week’s duration. She also had had nonbloody and nonbilious emesis. She had a history of chronic constipation, so her mother had tried to give the girl an enema. When the enema did not produce a bowel movement and did not seem to help her daughter’s pain, she brought her to the ED.

Physical examination. On initial examination, the girl was in no acute distress. Vital signs were normal, and she was afebrile. She was awake and alert and had moist mucous membranes. She had no scleral icterus. Cardiac examination revealed a normal heart rate and rhythm. Her lungs were clear to auscultation bilaterally. Her abdomen was soft and nondistended with no palpable masses, but she had diffuse abdominal pain without guarding. Her skin was warm and clear of rashes, with normal capillary refill.

Diagnostic tests. Initial laboratory test results showed elevated levels of transaminases, γ-glutamyltransferase (GGT), and lipase (Table). The total bilirubin level was within normal limits, but the direct bilirubin level was minimally elevated at 0.5 mg/dL.


Chart

Further workup was ordered to evaluate for biliary disease. Computed tomography scans of the abdomen showed intrahepatic and extrahepatic dilation of the bile ducts, with a common bile duct width of 2.8 cm—findings that were concerning for choledochal cyst. To better evaluate the cyst, magnetic resonance cholangiopancreatography (MRCP) scanning was performed, the results of which confirmed a type IVa choledochal cyst (Figures 1 and 2). Additional laboratory test results showed an elevated cancer antigen 19-9 (CA 19-9) level but a normal α1-fetoprotein level (Table).

Figure 1
Figure 1. Coronal T2-weighted MRCP image showing diffuse biliary dilation, small stones in the common hepatic and duct and the common bile duct, as well as mild inflammation of the pancreas.

Fig 2
Figure 2. Three-dimensional rotating MRCP image showing severe biliary dilation throughout the liver, including the common hepatic duct and the common bile duct.

Treatment. She was taken to the operating room for excision and Roux-en-Y hepaticojejunostomy. During the procedure, the choledochal cyst was found to encompass the entire length of the extrahepatic biliary tree and bilateral intrahepatic systems, consistent with a type IVa biliary cyst. Tissue was sent for pathology.

NEXT: Discussion

 

Authors:
Nicole Zeky, DO; Emily Klepper, MD; and Elizabeth McDonough, MD

Pediatric Residency Program, Our Lady of the Lake Children’s Hospital, Baton Rouge, Louisiana

Citation:
Zeky N, Klepper E, McDonough E. Choledochal cyst [published online October 17, 2019]. Gastroenterology Consultant.

 

A 3-year-old girl presented to the emergency department (ED) with abdominal pain of 1 week’s duration. She also had had nonbloody and nonbilious emesis. She had a history of chronic constipation, so her mother had tried to give the girl an enema. When the enema did not produce a bowel movement and did not seem to help her daughter’s pain, she brought her to the ED.

Physical examination. On initial examination, the girl was in no acute distress. Vital signs were normal, and she was afebrile. She was awake and alert and had moist mucous membranes. She had no scleral icterus. Cardiac examination revealed a normal heart rate and rhythm. Her lungs were clear to auscultation bilaterally. Her abdomen was soft and nondistended with no palpable masses, but she had diffuse abdominal pain without guarding. Her skin was warm and clear of rashes, with normal capillary refill.

Diagnostic tests. Initial laboratory test results showed elevated levels of transaminases, γ-glutamyltransferase (GGT), and lipase (Table). The total bilirubin level was within normal limits, but the direct bilirubin level was minimally elevated at 0.5 mg/dL.


Chart

Further workup was ordered to evaluate for biliary disease. Computed tomography scans of the abdomen showed intrahepatic and extrahepatic dilation of the bile ducts, with a common bile duct width of 2.8 cm—findings that were concerning for choledochal cyst. To better evaluate the cyst, magnetic resonance cholangiopancreatography (MRCP) scanning was performed, the results of which confirmed a type IVa choledochal cyst (Figures 1 and 2). Additional laboratory test results showed an elevated cancer antigen 19-9 (CA 19-9) level but a normal α1-fetoprotein level (Table).

Figure 1
Figure 1. Coronal T2-weighted MRCP image showing diffuse biliary dilation, small stones in the common hepatic and duct and the common bile duct, as well as mild inflammation of the pancreas.

Fig 2
Figure 2. Three-dimensional rotating MRCP image showing severe biliary dilation throughout the liver, including the common hepatic duct and the common bile duct.

Treatment. She was taken to the operating room for excision and Roux-en-Y hepaticojejunostomy. During the procedure, the choledochal cyst was found to encompass the entire length of the extrahepatic biliary tree and bilateral intrahepatic systems, consistent with a type IVa biliary cyst. Tissue was sent for pathology.

NEXT: Discussion