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Case Report | Discussion

Case report:

A 21- year -old man presented with history of progressive jaundice with pruritis for the past one year with rapid increase since one month. This was associated with colicky pain and in the right hypochondrium and low grade fever. On presentation, the patient had hemetemesis with a past history of multiple similar episodes. On examination, the patient was icteric. The spleen was moderately enlarged.

Laboratory investigation showed a raised serum bilirubin (total bilirubin-5.6mg%, direct-3.5mg%).The liver enzymes were also raised. Upper gastro-intenstinal scopy showed esophageal and fundic varices.

Ultrasonography of abdomen showed moderate splenomegaly with multiple hyperechoic foci (fig.1); intrahepatic bile duct, right and left hepatic duct dilatation (fig.2). Multiple calculi were noted in dilated intrahepatic ducts (fig.3).There was abrupt narrowing of the common hepatic duct (fig.4). The common bile duct could not be traced. The liver parenchyma was otherwise normal. The portal vein showed thrombosis and was replaced by multiple collateral channels at the porta.. These collateral channels were seen to be surrounding the common hepatic duct (fig.3).

Fig. 1

Fig. 2

Fig. 3

Fig. 4

Computed tomography of abdomen revealed moderate dilation of intrahepatic biliary radicles with debris within (fig.5). The portal vein was replaced by multiple collaterals channels – forming a portal cavernoma (fig.6).The common hepatic duct showed abrupt narrowing at the level of portal cavernoma (fig.7). In addition, collateral vessels were noted at the gastro-esophageal junction and around the gall bladder wall (fig.8).There was moderate splenomegaly with calcific foci (fig.5).

Fig. 5

Fig. 6

Fig. 7

 

Fig. 8

Magnetic resonance cholangio-pancreatography demonstrated a short segment narrowing of the common hepatic duct with proximal dilation. The common bile duct was normal (fig.9).

Fig. 9

Diagnosis- Portal biliopathy –abnormalities of the extrahepatic and intrahepatic bile ducts in a with extra hepatic portal hypertension.

Differential Diagnosis - Sclerosing cholangitis.


Treatement - The patient was taken for biliary-enteric anastomosis but due to excessive bleeding , the procedure was abandoned .Temporary external biliary drainage was done. The patient is now being reevaluated for definitive surgery.

Discussion

Extra hepatic portal venous hypertension is a common cause of portal hypertension accounting for 30% of all cases in all age group. In children and adolescents, it is the commonest cause of portal hypertension. Nearly half of all patients with EHPVO experience onset in adulthood . Known childhood causes are omphalitis , umbilical vein catheterisation and intra abdominal sepsis. In adults, the other known causes are myeloproliferative disorders , local tumour invasion and chronic pancreatitis. As many as half of all patients with EHPVO (children and adults) have no predisposing cause. This was the case in our patient.

Cavernous transformation of the portal vein due to EHPVO is not infrequent; but obstruction in association with this disorder is distinctly uncommon. A few reports have indirectly implicated "portal cavernoma"- a recanalised antepartum or postpartum portal vein thrombosis due possibly to umbilical sepsis, blunt trauma, intra abdominal sepsis , cogalupathies and adjacent malignancy or inflammation can be responsible for biliary obstruction and jaundice. In our patient with, obstructive jaundice had occurred apparently because of compression of common hepatic duct the by portal cavernoma .

Patients with EHPVO are known to have a high frequency of duodenal , rectal , and other ectopic varices. Pericholedocheal and periportal varices are also common specially in youth due to prolonged stimulation for collateral formation.

The extra hepatic bile duct is surrounded by two venous systems. The paracholedochal vein of Perten which run parallel to the ductal wall and the epicholedochal plexus of Saint which is on the surface of bile duct. Dilatation of these vein produces extrinsic impression and irregular mural defects in the common bile duct wall.

The pathogenesis of the biliary changes is postulated as being due to collaterals causing bile duct impression, fibrous scarring at the porta causing angulation of the bile duct and ischemic injury to bile duct causing stricture formation and calibre irregularity .In this patient, there is narrowing of the common hepatic duct with proximal dilatation.

Obstructive jaundice occurring in a case of portal hypertension is a rare association. In a patient presenting with portal cavernoma and clinical feature suggestive of obstructive jaundice possibility of portal biliopathy should be considered.