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Congenital Hepatic Fibrosis Treatment & Management

  • Author: Hisham Nazer, MB, BCh, FRCP, , DTM&H; Chief Editor: Carmen Cuffari, MD  more...
Updated: Aug 21, 2015

Medical Care

See the list below:

  • Medical therapy is provided mainly in the presence of cholangitis. Results of the liver biopsy and culture determine medical therapy in congenital hepatic fibrosis (CHF).
  • Portal hypertension with secondary esophageal varices also requires treatment.
    • Some episodes of variceal bleeding may spontaneously resolve. However, persistent hemorrhage that lasts longer than 12 hours or requires blood transfusion warrants the consideration of medical therapy, surgical therapy, or both.
    • Acute management includes intravenous fluid administration, nasogastric tube placement, and, once the patient is stable, an endoscopy.
    • An initial pharmacologic approach with vasopressin, somatostatin, or other vasoconstricting medications is preferred in pediatrics. Each is discussed more thoroughly in the Medication section.
    • In cases of uncontrolled hemorrhage, one may resort to other interventions, including endoscopic sclerotherapy or band ligation, transjugular intrahepatic portosystemic shunting, or surgical shunting.

Surgical Care

Portosystemic shunt surgery is the treatment of choice for these patients because the risk of postoperative hepatic encephalopathy is low. Patients also have a patent portal vein and preserved liver function. External or internal drainage may be required to resolve the refractory hepatobiliary infection.

  • Sclerotherapy is indicated for the treatment of acute hemorrhage from esophageal varices and as a primary therapy for management of recurrent or chronic variceal bleeding.
    • Relative contraindications to the procedure include uncorrectable severe coagulopathy, fever, or compromise of respiratory status.
    • Complications of sclerotherapy include ulcers, strictures, rebleeding, perforations, and bacteremia.
  • A Sengstaken-Blakemore tube may be required in some patients to control massive life-threatening bleeding. However, its current use is very much limited to patients who fail to respond to endoscopic sclerotherapy and in whom band ligation is not possible.
  • Endoscopic variceal ligation is an effective and safe method for early variceal obliteration in children. It is effective in controlling active bleeding and preventing recurrences. Types of surgical shunt include nonselective total portosystemic shunts, nonselective partial portosystemic shunts that maintain some antegrade blood flow to the liver, and selective portosystemic shunts, which decompress the gastroesophageal junction and the spleen through the splenic vein to the left renal vein.
  • Transjugular intrahepatic portosystemic shunts are considered for patients not amenable to sclerotherapy. It is particularly valuable in treating patients with refractory bleeding before liver transplantation.
  • Early shunt surgery with splenorenal or portacaval shunting may be required if repeated endoscopic sclerotherapy fails to arrest the variceal bleeding. Select the type of shunt carefully so that renal or hepatic transplantation remains a future option, with minimal limitations and complications.[16]
  • Liver transplantation is also considered in the management of congenital hepatic fibrosis complicated by recurrent cholangitis or failure to respond to various medical and surgical therapeutic modalities resulting in progressive hepatic dysfunction.[17]


At one stage of the clinical course of congenital hepatic fibrosis, management and follow-up evaluation require consultations with other disciplines, medical and surgical.

  • Pediatric nephrologist - Required in most cases because of frequent association of congenital hepatic fibrosis with autosomal recessive polycystic kidney disease (ARPKD)
  • Pediatric surgeon - Required for biliary drainage procedure and wedge liver biopsy
  • Invasive radiologist - Required for imaging studies, angiography, and splenic portography
  • Vascular surgeon - Required for evaluation of the case with regard to type and timing of shunt surgery
  • Transplant surgeon - Required for liver transplantation, renal transplantation, or both


See the list below:

  • Patients with congenital hepatic fibrosis are usually placed on a regular diet.


See the list below:

  • The activity of children with congenital hepatic fibrosis is not restricted, except in late stages of severe hepatic involvement with progressive bleeding varices, severe renal impairment, and shortly after liver or kidney transplantation.
Contributor Information and Disclosures

Hisham Nazer, MB, BCh, FRCP, , DTM&H Professor of Pediatrics, Consultant in Pediatric Gastroenterology, Hepatology and Clinical Nutrition, University of Jordan Faculty of Medicine, Jordan

Hisham Nazer, MB, BCh, FRCP, , DTM&H is a member of the following medical societies: American Association for Physician Leadership, Royal College of Paediatrics and Child Health, Royal College of Surgeons in Ireland, Royal Society of Tropical Medicine and Hygiene, Royal College of Physicians and Surgeons of the United Kingdom

Disclosure: Nothing to disclose.


Dena Nazer, MD, FAAP Assistant Professor of Pediatrics, Wayne State University School of Medicine; Chief, Child Protection Team, Children's Hospital of Michigan

Dena Nazer, MD, FAAP is a member of the following medical societies: Academic Pediatric Association, American Academy of Pediatrics, American Professional Society on the Abuse of Children, Ray E Helfer Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching.

Additional Contributors

Chris A Liacouras, MD Director of Pediatric Endoscopy, Division of Gastroenterology and Nutrition, Children's Hospital of Philadelphia; Associate Professor of Pediatrics, University of Pennsylvania School of Medicine

Chris A Liacouras, MD is a member of the following medical societies: American Gastroenterological Association

Disclosure: Nothing to disclose.

  1. Lipschitz B, Berdon WE, Defelice AR, Levy J. Association of congenital hepatic fibrosis with autosomal dominant polycystic kidney disease. Report of a family with review of literature. Pediatr Radiol. 1993. 23(2):131-3. [Medline].

  2. Hoyer PF. Clinical manifestations of autosomal recessive polycystic kidney disease. Curr Opin Pediatr. 2015 Apr. 27 (2):186-92. [Medline].

  3. Bristowe F. Cystic disease of the liver associated with similar disease of the kidney. Trans Pathol Soc Lond. 1856. 5:229.

  4. Kerr DN, Harrison CV, Sherlock S, Walker RM. Congenital hepatic fibrosis. Q J Med. 1961 Jan. 30:91-117. [Medline].

  5. O'Brien K, Font-Montgomery E, Lukose L, Bryant J, Piwnica-Worms K, Edwards H, et al. Congenital hepatic fibrosis and portal hypertension in autosomal dominant polycystic kidney disease. J Pediatr Gastroenterol Nutr. 2012 Jan. 54 (1):83-9. [Medline].

  6. Akhan O, Karaosmanoglu AD, Ergen B. Imaging findings in congenital hepatic fibrosis. Eur J Radiol. 2007 Jan. 61(1):18-24. [Medline].

  7. Desmet VJ. Congenital diseases of intrahepatic bile ducts: variations on the theme "ductal plate malformation". Hepatology. 1992 Oct. 16(4):1069-83. [Medline].

  8. Harris PC, Rossetti S. Molecular genetics of autosomal recessive polycystic kidney disease. Mol Genet Metab. 2004 Feb. 81(2):75-85. [Medline].

  9. Losekoot M, Haarloo C, Ruivenkamp C, et al. Analysis of missense variants in the PKHD1-gene in patients with autosomal recessive polycystic kidney disease (ARPKD). Hum Genet. 2005 Nov. 118(2):185-206. [Medline].

  10. Turkbey B, Ocak I, Daryanani K,et al. Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol. 2009 Feb. 39(2):100-11. [Medline].

  11. Hartung EA, Guay-Woodford LM. Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. Pediatrics. 2014 Sep. 134 (3):e833-45. [Medline].

  12. El-Youssef M, Mu Y, Huang L, et al. Increased expression of transforming growth factor-beta1 and thrombospondin-1 in congenital hepatic fibrosis: possible role of the hepatic stellate cell. J Pediatr Gastroenterol Nutr. 1999 Apr. 28(4):386-92. [Medline].

  13. Ozaki S, Sato Y, Yasoshima M, et al. Diffuse expression of heparan sulfate proteoglycan and connective tissue growth factor in fibrous septa with many mast cells relate to unresolving hepatic fibrosis of congenital hepatic fibrosis. Liver Int. 2005 Aug. 25(4):817-28. [Medline].

  14. Abdullah AM, Nazer H, Atiyeh M, Ali MA. Congenital hepatic fibrosis in Saudi Arabia. J Trop Pediatr. 1991 Oct. 37(5):240-3. [Medline].

  15. Ovchinsky N, Moreira RK, Lefkowitch JH, Lavine JE. Liver biopsy in modern clinical practice: a pediatric point-of-view. Adv Anat Pathol. 2012 Jul. 19 (4):250-62. [Medline].

  16. Al-Lawati TT. Fibropolycystic disease of the liver and kidney in Oman. Arab J Gastroenterol. 2013 Dec. 14 (4):173-5. [Medline].

  17. Arikan C, Ozgenc F, Akman SA, et al. Impact of liver transplantation on renal function of patients with congenital hepatic fibrosis associated with autosomal recessive polycystic kidney disease. Pediatr Transplant. 2004 Dec. 8(6):558-60. [Medline].

  18. Dagli U, Atalay F, Sasmaz N, et al. Caroli's disease: 1977-1995 experiences. Eur J Gastroenterol Hepatol. 1998 Feb. 10(2):109-12. [Medline].

  19. Al-Bhalal L, Akhtar M. Molecular basis of autosomal recessive polycystic kidney disease (ARPKD). Adv Anat Pathol. 2008 Jan. 15(1):54-8. [Medline].

  20. Ananthakrishnan AN, Saeian K. Caroli's disease: identification and treatment strategy. Curr Gastroenterol Rep. 2007 Apr. 9(2):151-5. [Medline].

  21. Braga AC, Calheno A, Rocha H, Lourenco-Gomes J. Caroli's disease with congenital hepatic fibrosis and medullary sponge kidney. J Pediatr Gastroenterol Nutr. 1994 Nov. 19(4):464-7. [Medline].

  22. Brancatelli G, Federle MP, Vilgrain V, et al. Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 2005 May-Jun. 25(3):659-70. [Medline].

  23. Gocmen R, Akhan O, Talim B. Congenital absence of the portal vein associated with congenital hepatic fibrosis. Pediatr Radiol. 2007 Sep. 37(9):920-4. [Medline].

  24. Gunay-Aygun M, Font-Montgomery E, Lukose L, Tuchman Gerstein M, Piwnica-Worms K, Choyke P. Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology. 2013 Jan. 144(1):112-121.e2. [Medline].

  25. Gunay-Aygun M, Tuchman M, Font-Montgomery E, et al. PKHD1 sequence variations in 78 children and adults with autosomal recessive polycystic kidney disease and congenital hepatic fibrosis. Mol Genet Metab. 2010 Feb. 99(2):160-73. [Medline].

  26. Ling SC. Congenital cholestatic syndromes: what happens when children grow up?. Can J Gastroenterol. 2007 Nov. 21(11):743-51. [Medline].

  27. McEvoy CF, Suchy FJ. Biliary tract disease in children. Pediatr Clin North Am. 1996 Feb. 43(1):75-98. [Medline].

  28. Miller WJ, Sechtin AG, Campbell WL, Pieters PC. Imaging findings in Caroli's disease. AJR Am J Roentgenol. 1995 Aug. 165(2):333-7. [Medline].

  29. Nakanuma Y, Harada K, Sato Y, Ikeda H. Recent progress in the etiopathogenesis of pediatric biliary disease, particularly Caroli's disease with congenital hepatic fibrosis and biliary atresia. Histol Histopathol. 2010 Feb. 25(2):223-35. [Medline].

  30. Nazer D, Nazer H. Congenital Hepatic Fibrosis. Elzouki, Harfi, Nazer, Stapleton, et al. Textbook of Clinical Pediatrics. 2nd. Berlin-Heidelberg: Springer-Verlag; 2012. 3: 2013-16; Chapter 208.

  31. Poala SB, Bisogno G, Colombatti R. Thrombocytopenia and splenomegaly: an unusual presentation of congenital hepatic fibrosis. Orphanet J Rare Dis. 2010. 5:4. [Medline].

  32. Shorbagi A, Bayraktar Y. Experience of a single center with congenital hepatic fibrosis: a review of the literature. World J Gastroenterol. 2010 Feb 14. 16(6):683-90. [Medline]. [Full Text].

  33. Srinath A, Shneider BL. Congenital hepatic fibrosis and autosomal recessive polycystic kidney disease. J Pediatr Gastroenterol Nutr. 2012 May. 54(5):580-7. [Medline].

  34. Suchy FJ. Liver disease in children. Mosby Yearbook. St Louis, MO: Mosby Year Book Inc; 1994.

  35. Yonem O, Ozkayar N, Balkanci F, et al. Is congenital hepatic fibrosis a pure liver disease?. Am J Gastroenterol. 2006 Jun. 101(6):1253-9. [Medline].

Histopathology of liver biopsy in congenital hepatic fibrosis, which shows a widened portal tract with bands of fibrous tissue that separate areas of normal hepatic parenchyma. Note the multiple irregularly shaped narrow and elongated bile ducts and the absent lobular and portal inflammation.
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