Anomalous excretion of bile and pancreatic juice in a patient with choledochal cyst

Anomalous Excretion of Bile and Pancreatic in a Patient With Choledochal Cyst ByTakeshi

Abe, Yoshinobu Hata, Fumiaki Sasaki, and Junichi Uchino Sapporo,

l The authors report the findings of endoscopic retrograde cholangiopancreatography in a l-year-old girl with choledochal cyst; contrast dye injected through the common channel was excreted solely through the duct of Santorini. The obstruction to the bile and pancreatic juice caused by marked bending of the common channel was responsible for the abnormal drainage of bile and pancreatic juice. Copyright o 1993 by W.B. Saunders Company INDEX WORDS: Choledochal

Juice

cyst.

I

N PATIENTS with choledochal cyst, there are anatomical configurations such as malconnection of the common bile duct and pancreatic duct, suggesting the presence of developmental anomalies of not only the bile duct but also the pancreatic duct.’ It is possible that these anomalous configurations affect the flow of pancreatic juice; however, there are few subjective studies of the precise anatomy of the pancreatic duct because endoscopic retrograde cholangiopancreatography (ERCP) is difficult with small infants, and intraoperative cholangiographic findings have sometimes been imprecise. This is a report of ERCP in a patient with choledochal cyst; the bile and pancreatic juice were discharged in peculiar fashion through the duct of Santorini rather than through the common channel.

Japan

the common channel could not be seen in the later images. The peripheral pancreatic duct was not dilated (Fig 1). On the 21st day of hospitalization the choledochal cyst was excised, and hepaticojejunostomy was performed in Roux-en-Y fashion. There were no signs of pancreatitis in the biopsy specimen of the pancreas. The patient was discharged after 34 days of hospitalization, and has been living without any bouts of cholangitis or pancreatitis. DISCUSSION The ERCP findings showed that bile and pancreatic juice were excreted solely through the duct of Santorini despite the presence of the common channel. It appears that the marked bending of the common channel played a role in the abnormal excretion of the contrast dye by obstructing the flow of bile and pancreatic juice. Intraoperative cholangiography did not show the common channel, which led

CASE REPORT A l-year-old girl was admitted to our institution because of vomiting. There was no abnormality in the family history. During the clinical examination, slight jaundice and mild dehydration were noted, but no abdominal tumor. Laboratory findings included bilirubin of 4.5 mg/dL (normal, < 1 mg/dL) and serum amylase of 830 IU (normal, < 180 IU). Promptly after fluid supplementation the patient became asymptomatic, and results of laboratory tests became normal. Ultrasonography showed a cystic mass that suggested the presence of choledochal cyst, and ERCP was performed on the 14th day of hospitalization. ERCP showed marked bending of the long common channel, stenosis of the common bile duct just above the common channel, and massive cystic dilatation of the extrahepatic bile duct. Contrast dye injected through the common channel was not excreted through this channel but through the dilated duct of Santorini, and

From the First Department of Surgery, Hokkaido University School of Medicine, Sapporo, Japan. Address reprint requests to Takeshi Abe, First Depatiment of Surgery, Hokkaido University School of Medicine, N-1.5, W-7, Sapporo, Japan. Copyright o 1993 by W. B. Saunders Company 0022-3448/93/2812-0013$03.00/0 1566

Fig 1. ERCP. Top: Contrast dye was injectedthrough the common channel (6). showing marked bending. Bottom: The contrast dye was excreted through the duct of Santorini (S) but not through the common channel. Journal of Pediatric Surgery, Vol28, No 12 (December), 1993: pp 1566-1567

ANOMALOUS

EXCRETION IN CHOLEDOCHAL

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to a misunderstanding of the precise anatomy of this case. There are some reports of pancreatic involvement of choledochal cysts. It can be hypothesized that compression with obstruction to the pancreatic duct by the choledochal cyst is a cause of pancreatitis.2 It is unknown whether the bending of the common channel is the result of compression caused by the massive choledochal cyst or the cause of the reflux of pancreatic juice into the bile duct, leading to the inflammation and dilatation of the common bile duct. In an experimental model, gradual obstruction of

the pancreatic duct over a prolonged period was believed to be of paramount importance in the causation of chronic pancreatitis.3 Another experiment showed that enhancement of secretion of pancreatic juice with incomplete obstruction produced varying degrees of pancreatitis.4 In the present case, the duct of Santorini was a substitute for the obstructed common channel. If there are no such collateral ways to facilitate adequate drainage of the pancreatic juice, the obstruction may continue as a subtle stimulation for the progression of pancreatic damage.

REFERENCES 1. Todani T, Watanabe Y, Fujii T, et al: Cylindrical dilatation of choledochus: A special type of congenital bile duct dilatation. Surgery 98:964-969,1985 2. Altman MS, Halls JM, Douglas AP, et al: Choledochal cyst presenting as acute pancreatitis. Evaluation with endoscopic retro-

grade cholangiopancreatography. Am J Gastroenterol 70:514-519, 1978 3. Floyd CN, Christophersen WM: Experimental chronic pancreatitis. Arch Surg 701-709.1956 4. Hermann RE, Davis JH: The role of incomplete pancreatic obstruction in the etiology of pancreatitis. Surgery 48:318-329.1960