Rare Cause of Obstructive Jaundice in a Young Female

    

    

    Figure 4: Retrograde cholangiogram demonstrating horizontal band like
filling defect in distal CBD.
    

Post ERCP follow up, patient had declining serum bilirubin level with significant improvement in itching and clay stools.

Discussion

Embryologically, the bile ducts in the developing phase become obliterated by epithelial concrescence or proliferation. Later these solid structures become vacuolated, leading to formation of a lumen. Recanalization of the lumen of the biliary tree usually starts at the end of the fifth week of gestation [1]. Congenital CBD webs develop due to incomplete re-canalization of these solid structures.

The described associated abnormalities with these webs include anomalous hepatic duct of the caudate lobe, anomalous junction of the pancreato-biliary ductal system, choledochal cyst or hepatic fibrosis, choledocholithiasis, primary sclerosing cholangitis or idiopathic. Our case represents the first to report EUS characteristics of choledochal web, with no previous reports till date.

Webs may be partial or complete and the degree of obstruction determines whether patient will be symptomatic or not. Treatment is either Endoscopic dilatation or surgical by-pass [2]. Dolar et al. described a congenital web of the CHD in an adult patient, which was not associated with jaundice [3]. Chapoy et al. described a similar case to the one described by Dolar et al. but their patient was only 40 months old.4 The association of CHD ‘diaphragms’ and biliary obstruction has been described by both Fisher et al. and Devanesan et al., who postulated that small perforations in these diaphragms allowed some degree of biliary drainage and thus a delayed presentation of obstructive jaundice [5,6]. Ravi K et al. In their report mentioned about successful endoscopic therapy of choledochal web by Balloon Dilatation [7]. Kim et al described a case of intra-hepatic choledochal web which was treated by balloon dilatation [8]. Gulliver et al. described common bile stones to be associated with the web in a significant number of patients [9]. Our patient did not, however, have bile duct stones, but clearly had a web present in the CBD and had significant improvement in clinical and biochemical parameters post endoscopic treatment.

The physiologic implications of web of the extrahepatic biliary tree are not necessarily the same as for other causes of extrahepatic biliary obstruction. While the web may ultimately cause obstruction, it is likely that forward drainage from the liver will be undisturbed in the vast majority of cases. Initially the patient may be asymptomatic or may present with vague and nonspecific symptoms such as abdominal pain, nausea and vomiting. Early on in the disease process, one may only demonstrate elevations of transaminases and alkaline phosphatase, together with ductal dilatation, but without obstructive jaundice. However, in the setting of inflammation in the region of the porta hepatis, or the passage of a small calculus, the web will almost certainly become obstructed and result in obstructive jaundice with or without the association of cholangitis. These webs are probably congenital and only present later in life due to an associated abnormality.

While the standard imaging studies such as ultrasonogram and computerized tomography may reveal bile duct dilatation, they are unlikely to reveal the presence of a web. Endoscopic Retrograde Cholangiopancreatography may be more successful in adequately delineating the presence of a web. With the increasing usage of Magnetic Resonance Cholangiography, this anatomical abnormality may be more commonly diagnosed in the future. Moreover, role of Endoscopic Ultrasound in choledochal web has not yet been described in world literature. Our case remains first of its kind to describe the same.

References

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2. Siegel JH. Endoscopic retrograde cholangiopancreatography: technique, diagnosis and therapy. New York: Raven Press.1992; 38-39.
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