British Journal of Radiology (2003) 76, 667-669
© 2003 British Institute of Radiology
doi: 10.1259/bjr/19912091
Obstructive jaundice in a case of portal hypertension
A Chawla, MBBS
M Maheshwari, MBBS
and
H Parmar, MD
Department of Radiology, King Edward VII Memorial Hospital, Parel, Mumbai-400012, India
Correspondence: Hemant Parmar
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Introduction
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A 21-year-old man presented with history of progressive jaundice with pruritis for 1 year with a rapid increase in the last month. On presentation the patient had haematemesis with a past history of multiple similar episodes.
On examination the patient was icteric and had moderate splenomegaly. Laboratory investigation demonstrated a raised serum bilirubin (total bilirubin, 5.6 mg%; direct, 3.5 mg%), and the liver enzymes were also raised (alkaline phosphatase, 1014 U l-1, SGOT, 106 U l-1, SGPT, 72 U l-1).
Following are the ultrasound (with colour Doppler) (Figure 1
) and CT–abdomen (plain and contrast) (Figures 2 and 3) images. What is the diagnosis? What is the cause of obstructive jaundice?
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Figure 1. Ultrasound with Doppler shows intrahepatic biliary dilatation containing calculi (arrow). Multiple collateral channels of the portal vein are also present.
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Figure 2. CT of the abdomen reveals moderate dilatation of intrahepatic bile ducts containing debris (arrow).
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Figure 3. CT (contrast enhanced) of the abdomen shows multiple collateral channels (arrow) replacing the portal vein suggestive of portal cavernoma. The common hepatic duct shows abrupt narrowing at the level of portal cavernoma.
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Answer
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Ultrasound of the abdomen shows dilatation of right and left hepatic ducts. Multiple calculi are noted in dilated intrahepatic ducts. There is abrupt narrowing of the common hepatic duct. The common bile duct could not be traced. The portal vein is replaced by multiple collateral channels at the porta hepatis. These collateral channels are seen to surround the common hepatic duct (Figure 1
). Moderate splenomegaly with multiple hyperechoic foci was also noted (not shown). Plain and contrast enhanced CT scans of the abdomen revealed dilation of intrahepatic biliary radicles (IHBR) with calculi and debris within (Figures 2 and 3). In addition, collateral vessels are seen at the gastroesophageal junction and gall bladder wall (not shown).
MR cholangiopancreatography demonstrated short segment narrowing of the common hepatic duct with proximal dilation. The common bile duct is normal (Figure 4).
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Figure 4. MR cholangiopancreatography shows narrowing of the common hepatic duct with normal common bile duct.
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The patient was referred for portal decompression shunt surgery. Following surgery the bilirubin and the liver enzymes returned to normal over a period of 3 weeks.
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Diagnosis
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The diagnosis is of portal biliopathy—abnormalities of the extrahepatic and intrahepatic bile ducts in patients with portal hypertension especially those with extrahepatic portal venous obstruction (EHPVO).
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Differential diagnosis
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The differential diagnosis is of primary sclerosing cholangitis. However, in this condition, there is predominant involvement of small bile ducts with irregular tapering of ducts unlike in portal biliopathy.
Extrahepatic portal venous hypertension is a common cause of portal hypertension accounting for 30% of all cases in all age groups. In children and adolescents it is the most common cause of portal hypertension [1]. Nearly half of all patients with EHPVO experience onset in adulthood [1]. Known childhood causes are omphalitis, umbilical vein catheterization and intra-abdominal sepsis [1]. In adults known causes are myeloproliferative disorders, local tumour invasion and chronic pancreatitis. As many as half of the patients with EHPVO (children and adults) have no predisposing cause [1, 6], as in our patient.
Cavernous transformation of the portal vein due to EHPVO is not infrequent but biliary obstruction in association with this disorder is distinctly uncommon [2]. A few reports have indirectly implicated the presence of a "portal cavernoma", a recanalized portal vein thrombosis due possibly to umbilical sepsis, blunt trauma, intra-abdominal sepsis, coagulopathies and adjacent malignancy or inflammation may be responsible for biliary obstruction and jaundice [2]. In our patient obstructive jaundice occurred because of compression of the common hepatic duct by portal cavernoma.
The extrahepatic bile duct is surrounded by two venous systems. The paracholedochal vein of Perten [4], which runs parallel to the ductal wall, and the epicholedochal plexus of Saint [5], which is on the surface of the bile duct. Dilatation of these veins produces extrinsic impression and irregular mural defects in the common bile duct wall.
Patients with EHPVO are known to have a high frequency of duodenal, rectal and other ectopic varices. Pericholedocheal and periportal varices are also common especially in childhood due to prolonged stimulation of collaterals [3].
Malkan et al studied 42 patients with portal hypertension (EHPVO 20; non-cirrhotic portal fibrosis (NCPF) 11; and cirrhosis 11). In the EHPVO group 85% patients had abnormalities in the extrahepatic or intrahepatic biliary tree. Only one patient with NCPF and three patients with cirrhosis had abnormalities of the biliary systems. This suggests that cholangiopathy associated with portal hypertension is exclusive to patients with EHPVO, implying that the site of portal venous obstruction responsible for portal hypertension is more important in its pathogenesis than the duration and severity of portal hypertension [7].
Khuroo et al studied 21 adult patients with EHPVO, of whom two had extrahepatic cholestasis and one presented with recurrent cholangitis. In this group, liver function tests revealed a raised serum bilirubin in 14 (66.6%) and elevated alkaline phosphatase in 17 (80.9%) patients. Only the three patients with extrahepatic cholestasis and recurrent cholangitis had symptoms and signs of billiary disease. Cholangiography revealed abnormal findings in 17 patients (80.9%) of which the common bile duct was affected in 66.1% and the common hepatic duct in 38.1%. Abnormalities detected included: segmental upstream dilatation, calibre irregularity, stricture, ectasia, extrinsic impression on the bile duct due to collaterals, displacement, angulation and pruning of ducts [6]. Such changes have also been called "pseudocholangiocarcinoma sign" because they mimic a cholangiocarcinoma spreading along the bile duct.
Nagi et al studied 43 patients with an established diagnosis of EHPVO of which eight had symptoms of obstructive jaundice. ERCP revealed cholangiographic abnormalities in 93% patients. Extrahepatic bile ducts were involved in all of them. Cholangiographic abnormalities were very common in EHPVO, even with out manifestations of biliary disease [8].
The pathogenesis of the biliary changes is postulated as due to collaterals causing impressions on the bile duct, with fibrous scarring at the porta causing angulation of the bile duct and ischaemic injury to the bile duct causing stricture formation and calibre irregularity. In some studies bile duct angulation and ectasia did not reverse after decompression shunt surgery indicating fixed obstruction due to fibrosis or ischaemia as their cause [9]. In this case there was narrowing of the common hepatic duct with proximal dilatation.
Elevated alkaline phosphatase levels (after excluding common bile duct calculi) and the presence of gall bladder varices on ultrasound reliably predict the presence of cholangiopathy associated with portal hypertension [7].
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Conclusion
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Obstructive jaundice in association with portal hypertension is rare. In patients presenting with portal cavernoma and clinical feature suggestive of obstructive jaundice the possibility of portal biliopathy should be considered. Raised alkaline phosphatase levels (in the absence of common bile duct calculi) serve as useful markers for the presence of cholangiopathy associated with portal hypertension.
Received for publication March 6, 2002.
Revision received August 14, 2002.
Accepted for publication September 19, 2002.
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References
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- Webb LJ, Sherlock S. The aeitology, presentation and natural history of extrahepatic portal venous obstruction. Q J Med 1979;192:627–39.
- Meredith HC, Vujic I, Schabel SI, O'Brien PH. Obstructive jaundice caused by cavernous transformation of portal vein. Br J Radiol 1978;51:1011–2.[Abstract/Free Full Text]
- Chaudhary A, Dhar P, Sarin SK, Sachdev A, Agarwal AK, Vij JC, et al. Bile duct obstruction due to portal biliopathy in extra hepatic portal hypertension: Surgical Management. Br J Surg 1998;85:326–9.[CrossRef][Medline]
- Petren T. The veins of the extrahepatic biliary system and their pathologic-anatomic significance. Vert Anat Ges 1932;41:139–43.
- Saint JH. The epicholedochal venous plexus and its importance as a means of identifying the common duct during operation on extrahepatic biliary tract. Br J Surg 1961;48:489–98.[CrossRef][Medline]
- Khuroo MS, Yattoo GN, Zargar SA, Javid G, Dar MY, Khan BA, et al. Biliary abnormalities associated with extrahepatic portal venous obstruction. Hepatology 1993;17:807–13.[Medline]
- Malkan GH, Bhatia SJ, Bashir K, Khemnani R, Abraham P, Gandhi MS, et al. Cholangiopathy associated with portal hypertension: Diagnostic evaluation and clinical implications. Gastrointest Endosc 1999;49:344–8.[CrossRef][Medline]
- Nagi B, Kochhar R, Bhasin D, Singh K. Cholangiopathy in extrahepatic portal venous obstruction. Radiological appearances: Acta Radiol 2000;41:612–5.[CrossRef][Medline]
- Dhiman RK, Puri P, Chawla Y, Minz M, Bapuraj JR, Gupta S, et al. Biliary changes in extrahepatic portal venous obstruction: compression by collaterals or ischemic? Gastrointest Endosc 1999;50:646–52.[CrossRef][Medline]
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