Congenital Hepatic Fibrosis : Report on Two Cases And its Clinicopathological Correlation

  • Neeraj Dhameja institute of medical sciences, bhu
  • Varnika Rai institute of medical sciences, bhu
  • Rajeev Singh Institute of Medical Sciences BHU
  • Vineeta Gupta Institute of Medical Sciences BHU
  • O P Mishra Institute of Medical Sciences BHU
Keywords: Congenital hepatic fibrosis, Caroli’s syndrome, Autosomal Recessive, Polycystic Kidney Disease.


Congenital hepatic fibrosis (CHF) is a relatively rare disease belonging to ductal plate malformations and considered as variant of autosomal recessive polycystic kidney disease with juvenile and young adult presentation.Case 1 was a 13 year male, presented with complaints of abdominal pain and distension with multiple episodes of fever. Case 2 was a two year male patient, presented with complaints of abdominal swelling since birth, fever and nausea, along with palpable liver and non-palpable spleen.Histopathological examinations in both cases showed dense portal fibrosis with preserved hepatocytes. Within the fibrotic portal tract dilated biliary channels were seen with neutrophilic infiltration in first case which was suggestive of superimposed cholangitis. Hepatic parenchyma showed normal arrangement of hepatocytes. Based on the findings diagnosis of CHF was made.Congenital hepatic fibrosis though a rare disease should be considered in the differential diagnosis while dealing with pediatric liver biopsies.

Author Biographies

Neeraj Dhameja, institute of medical sciences, bhu
department of pathology, assistant proffesor.
Varnika Rai, institute of medical sciences, bhu
department of pathology, junior resident.
Rajeev Singh, Institute of Medical Sciences BHU
Department of Radiodiagnosis and Imaging Medicine, Senior Resident
Vineeta Gupta, Institute of Medical Sciences BHU
Department of Pediatrics, Professor.
O P Mishra, Institute of Medical Sciences BHU
Department of Pediatrics, Professor.


1. Shorbagi A, Bayraktar Y. Experience of a single center with congenital hepatic fibrosis: a review of the literature. World journal of gastroenterology 2010;16: 683.

2. Patel J N et al. Congenital Hepatic Fibrosis associated with polycystic kidney disease. Annals of Pathology and Laboratory Medicine 2015;2:187-191.

3. Blyth M, Ockenden BG. Polycystic disease of kidneys and liver. J Med Genet 1971; 8:257-84.

4. Arroyo M, Crawford JM. Pediatric Liver Disease and Inherited, Metabolic, and Developmental Disorders of the Pediatric and Adult Liver. In Odze RD, Goldblum JR, eds. Surgical Pathology Of The GI Tract, Liver, Biliary Tract and Pancreas. 2nd ed. Philadelphia: Saunders Elsevier; 2009.p.1245-1290.

5. Nakanuma Y, Terada T, Ohta G. Caroli’s disease in congenital hepatic fibrosis and infantile polycystic disease. Liver 1982;2:346–54.

6. Summerfield JA, Nagafuchi Y, Sherlock S, et al. Hepatobiliary fibropolycystic diseases. A clinical and histological review of 51 patients. J Hepatol 1986;2:141–56.

7. Portman BC, Roberts EA. Developmental abnormalities and liver disease in childhood. In: Burt AD, Portman BC, Ferrell LD, eds. MacSween’s Pathology of the Liver. 6th ed. London, UK: Churchill Livingstone Elsevier;2012.p.102-156

8. Averback P. Congenital hepatic fibrosis:asymptomatic adults without renal anomaly. Arch Pathol Lab Med 1977;101:260–1.

9. Yonem O, Bayraktar Y. Clinical characteristics of Caroli's syndrome. World J Gastroenterol 2007; 13: 1934-1937

10. Fauvert R, Benhamou JP. Congenital hepatic fibrosis. In: Schaffner F, Sherlock S, Leevy CM, editors. The liver and its diseases. New York: Intercontinental Medical Book; 1974. p. 283–8.

11. Witzleben CL. Cystic diseases of the liver. In: Zakim D, Boyer TD, editors. Hepatology A textbook of liver disease. Philadelphia: WB Saunders; 1990.p. 1395–411.

12. De Vos BF, Cuvelier C. Congenital hepatic fibrosis. J Hepatol 1988;6:222–8.

13. Desmet VJ. What is congenital hepatic fibrosis? Histopathology 1992; 20: 465-477

14. Gocmen R, Akhan O, Talim B. Congenital absence of the portal vein associated with congenital hepatic fibrosis. Pediatr Radiol 2007; 37: 920-924
Case Report