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Volume: 17 Issue: 1 January 2019 - Supplement - 1


Portal Hypertensive Biliopathy as a Cause of Severe Cholestasis in Children With Congenital Hepatic Fibrosis

Portal hypertensive biliopathy may occur in patients with noncirrhotic hepatic fibrosis. Portal hypertensive biliopathy treatment should be focused on manage-ment of portal hypertension and relief of biliary obstruction. In patients with noncirrhotic portal fibrosis and symptomatic portal hypertensive biliopathy, portal decompression surgery by proximal splenorenal shunt is one successful treatment option.

Key words : Direct bilirubinemia, Icterus, Portal hypertension


Portal hypertensive biliopathy (PHB) is characterized as presence of cholangiopathy in patients with portal hypertension. Although it is most commonly as-sociated with extrahepatic portal vein obstruction (EHPVO), it may also be associated with intrahepatic portal hypertension.1 The rate of incidence of PHB in patients with EHPVO is 81% to 100 %, with 0% to 33% in patients with cirrhosis and with 9% to 40% in patients with noncirrhotic portal fibrosis.2 Most patients with PHB are asymptomatic. Some patients present with biliary colic, fever, jaundice, pruritus, and ductal stones.3

The main theory for the pathogenesis of PHB is compressing portal cavernoma to the bile ducts. In EHPVO, collaterals develop to bypass obstructed segments of the portal vein. These large venous collaterals compress the bile ducts.4 The second theory for PHB is ischemic origin. The causes of ischemia of the bile ducts are prolonged compression by enlarged collaterals or decreased portal blood supply.1 The criterion standard for diagnosis of PHB is endoscopic retrograde cholangiopancreatography (ERCP), with magnetic resonance cholangiopan-creatography (MRCP) usually preferred for initial diagnosis because it is less invasive than ERCP.1,3 Asymptomatic patients do not require treatment.1,3 In symptomatic patients, treatment is aimed at the complications of biliary obstruction and management of portal hypertension.1

Here, we report a child with PHB secondary to congenital hepatic fibrosis who was successfully treated with portosystemic shunt surgery.

Case Report

We obtained permission from the patient/patient’s family for presentation of results.A 16-year-old female patient who had been diagnosed with congenital hepatic fibrosis 4 years previously was referred to our clinic for liver transplant. She had only 1 episode of esophageal variceal bleeding when she was diagnosed. She did not have any symptoms until 1 month earlier. She was hospitalized because of new episodes of variceal bleeding, icterus, and severe pruritus, which had not been responsive to 1 month of medical therapy.

On physical examination, she had icterus, hepatomegaly, splenomegaly, and dermatologic lesions due to scratching. Laboratory tests revealed hemoglobin level of 8.4 g/dL, leukocyte count of 1900/mm3, platelet count of 37 000/mm3, aspartate aminotransferase level of 44 U/L, alanine aminotransferase level of 40 U/L, gamma-glutamyltranspeptidase level of 20 U/L, alkaline phosphatase level of 115 U/L, total bilirubin of 14.3 mg/dL, direct bilirubin of 8.1 mg/dL, albumin level of 3.9 g/dL, and prothrombin time of 10.3 seconds.

Upper gastrointestinal endoscopy showed esophageal and fundic varices (Figure 1). Magnetic resonance cholangiopancreatography showed mild dilatation of the intrahepatic bile ducts (Figure 2). Liver biopsy was reported as congenital hepatic fibrosis and severe bilirubin stasis (Figure 3). Other causes of cholestasis, including infections, autoim-mune and metabolic liver diseases, Caroli disease, ductopenia, and extrahepatic biliary system disorders, were excluded on the basis of appropriate serologic, histopathologic, and radiologic tests. After she was diagnosed with PHB in accordance with clinical and radiologic findings, proximal splenorenal shunt was performed. Within 20 days after the procedure, her clinical and laboratory findings had completely resolved (Figure 4). One year after the surgery, there were no varices in the upper gastrointestinal endoscopy (Figure 5).


The term PHB was defined in 1992 by Sarin and associates; PHB refers to abnormalities of the extrahepatic and intrahepatic bile ducts in patients with portal hypertension. Extrahepatic and intra-hepatic bile duct abnormalities are more common in patients with EHPVO than in patients with noncirrhotic portal fibrosis or cirrhosis of the liver.1,4 Congenital hepatic fibrosis is a rare cause of PHB. In our patient, the cause of PHB was portal hyper-tension due to congenital hepatic fibrosis.

Pathogenesis of PHB is not clear. Compression by dilated collaterals and ischemia resulting from venous thrombosis may have roles in its devel-opment.1 In a study from Dhiman and associates, 55% of the patients had compression due to collaterals to the bile ducts. The remaining patients had no cavernoma compression to the bile ducts. The absence of compression suggests an ischemic origin in these patients.5

Most patients with portal hypertension develop portal cavernoma and biliary changes on cholan-giography, but only 5% to 50% of patients are symptomatic.1 Khuros and associates reported that 38% of patients were symptomatic, with all symptomatic patients in his study being adults.2 Sezgin and associates reported 10 patients with symptomatic PHB, with mean age of patients of 36 years.6 Our patient was symptomatic at 16 years old, and she had icterus and severe pruritus.

Doppler ultrasonography, MRCP, and ERCP are useful for diagnosis of PHB. Doppler ultraso-nography is noninvasive, radioactive-free, and easily available. Therefore, ultrasonography could be the initial imaging method. Although ERCP is the most important imaging method, it is invasive and has many risk factors. Another sensitive and noninvasive imaging method is MRCP and portography.1 Abdominal Doppler ultrasonography and MRCP showed dilated venous collaterals and intrahepatic biliary ductal dilatation in our patient.

Most patients with PHB are asymptomatic and do not require any treatment. A first choice treatment for patients with common bile duct stones is ERCP in those with cholangitis and without a shuntable vein.7 Endoscopic management may result in compli-cations, including injuring the dilated vein complex, causing hemobilia, and worsening the cholestasis.8 Portosystemic shunt surgery is the other treatment modality in patients with noncirrhotic portal hyper-tension and PHB. Esophageal variceal bleeding, symptoms of hypersplenism, and PHB could be controlled by portosystemic shunt surgery. Liver transplant is required in patients with cirrhosis.9 Our patient did not have gallstones and cholangitis, so we did not perform ERCP. Proximal splenorenal shunt surgery was chosen for PHB therapy in our patient because she had esophageal variceal bleeding and hypersplenism with normal liver function.


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Volume : 17
Issue : 1
Pages : 223 - 225
DOI : 10.6002/ect.MESOT2018.P79

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From the 1Division of General Surgery, the 2Division of Pediatric Gastroenterology, and the 3Division of Transplantation Surgery, Gazi University Faculty of Medicine, Ankara, Turkey
Acknowledgements: The authors have no sources of funding for this study and have no conflicts of interest to declare.
Corresponding author: Neslihan Gürcan Kaya, Gazi Üniversitesi Tıp Fakültesi Hastanesi, C blok, 1 kat, Çocuk Gastroenteroloji Polikliniği, Beşevler, Ankara, Turkey
Phone: +90 312 202 4148/+90 505 625 0133