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Congenital hepatic fibrosis leading to cirrhosis and hepatocellular carcinoma: a case report.

Ghadir MR, Bagheri M, Ghanooni AH - J Med Case Rep (2011)

Bottom Line: Advanced cirrhosis was diagnosed and our patient was scheduled for liver transplantation.During preparation for transplant, a hepatic mass was discovered which was found to be hepatocellular carcinoma.Radiofrequency ablation was performed and our patient was referred for transplantation.

View Article: PubMed Central - HTML - PubMed

Affiliation: Qom University of Medical Sciences, Qom, Iran. ghadir@ddrc.ac.ir.

ABSTRACT

Introduction: Congenital hepatic fibrosis is an uncommon cause of portal hypertension. Despite the presence of portal hypertension, hepatocellular and renal function are usually well preserved. Congenital hepatic fibrosis is included in the group of congenital diseases of fibropolycystic disorders. These include a broad spectrum of clinical diseases which are usually accompanied by hepatic involvement.

Case presentation: We report the case of a 27-year-old Iranian woman with congenital hepatic fibrosis leading to cirrhosis and subsequently hepatocellular carcinoma.

Conclusion: Advanced cirrhosis was diagnosed and our patient was scheduled for liver transplantation. During preparation for transplant, a hepatic mass was discovered which was found to be hepatocellular carcinoma. Radiofrequency ablation was performed and our patient was referred for transplantation.

No MeSH data available.


Related in: MedlinePlus

Heterogeneous and cirrhotic liver in CT scan.
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Figure 1: Heterogeneous and cirrhotic liver in CT scan.

Mentions: Our patient was followed regularly without any complications such as abdominal pain, jaundice, hematemesis, tarry stool, or increases in liver enzymes. No history could be elicited of alcohol abuse or previous hepatitis. There was no family history of liver or kidney disease. Markers for hepatitis B and C were negative; urine and serum copper levels were normal; and serum auto-antibodies were negative. The liver size gradually decreased and portal pressure increased as documented by ultrasonography and computed tomography (CT) (Figure 1). Her portal vein diameter was 14 mm, and splenomegaly was observed. Grade 1 lower esophageal varices were reported during an upper gastrointestinal endoscopy. Our patient was put on a waiting list for liver transplantation.


Congenital hepatic fibrosis leading to cirrhosis and hepatocellular carcinoma: a case report.

Ghadir MR, Bagheri M, Ghanooni AH - J Med Case Rep (2011)

Heterogeneous and cirrhotic liver in CT scan.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3105948&req=5

Figure 1: Heterogeneous and cirrhotic liver in CT scan.
Mentions: Our patient was followed regularly without any complications such as abdominal pain, jaundice, hematemesis, tarry stool, or increases in liver enzymes. No history could be elicited of alcohol abuse or previous hepatitis. There was no family history of liver or kidney disease. Markers for hepatitis B and C were negative; urine and serum copper levels were normal; and serum auto-antibodies were negative. The liver size gradually decreased and portal pressure increased as documented by ultrasonography and computed tomography (CT) (Figure 1). Her portal vein diameter was 14 mm, and splenomegaly was observed. Grade 1 lower esophageal varices were reported during an upper gastrointestinal endoscopy. Our patient was put on a waiting list for liver transplantation.

Bottom Line: Advanced cirrhosis was diagnosed and our patient was scheduled for liver transplantation.During preparation for transplant, a hepatic mass was discovered which was found to be hepatocellular carcinoma.Radiofrequency ablation was performed and our patient was referred for transplantation.

View Article: PubMed Central - HTML - PubMed

Affiliation: Qom University of Medical Sciences, Qom, Iran. ghadir@ddrc.ac.ir.

ABSTRACT

Introduction: Congenital hepatic fibrosis is an uncommon cause of portal hypertension. Despite the presence of portal hypertension, hepatocellular and renal function are usually well preserved. Congenital hepatic fibrosis is included in the group of congenital diseases of fibropolycystic disorders. These include a broad spectrum of clinical diseases which are usually accompanied by hepatic involvement.

Case presentation: We report the case of a 27-year-old Iranian woman with congenital hepatic fibrosis leading to cirrhosis and subsequently hepatocellular carcinoma.

Conclusion: Advanced cirrhosis was diagnosed and our patient was scheduled for liver transplantation. During preparation for transplant, a hepatic mass was discovered which was found to be hepatocellular carcinoma. Radiofrequency ablation was performed and our patient was referred for transplantation.

No MeSH data available.


Related in: MedlinePlus