eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology
Congenital Hepatic Fibrosis: Follow-up
Updated: Mar 31, 2008
Follow-up
Further Inpatient Care
- Recurrent episodes of GI bleeding, recurrent cholangitis, and the extent of renal impairment largely influence the course of the disease.
- Congenital hepatic fibrosis (CHF) is also associated with varied clinical conditions that require consultations, resulting in further inpatient care and management (see Consultations).
- With severe bleeding varices, the child may require admission to the intensive care unit.
Further Outpatient Care
- Patients with CHF are usually seen regularly at pediatric gastroenterology, hepatology, and nephrology clinics.
- In complicated cases, other disciplines are involved for regular follow-up assessment, including pediatric infectious disease, vascular surgery, and transplant surgery.
Inpatient & Outpatient Medications
- No specific medication is available for CHF. Medication therapy is usually directed at treatment of complications, such as recurrent cholangitis, sepsis, or renal impairment.
Transfer
- A pediatric gastroenterologist or hepatologist usually provides follow-up care to the child with CHF, in collaboration with a pediatric nephrologist in cases with renal involvement (eg, ARPKD).
- Transfer to other services is indicated only in the presence of complications, especially cholangitis, and particularly with recurrent cholangitis that does not adequately respond to medical management.
- In complicated cases, transfer to a tertiary care centre is recommended to facilitate the consultation and contribution of other services, such as pediatric surgery, vascular surgery, and transplant surgery.
Complications
- Complications in CHF are mainly related to its association with ARPKD, resulting in renal impairment, bleeding varices, and recurrent cholangitis.
- Recognition of cholangitis and prevention of its recurrence by appropriate surgical procedures are important. Transhepatic cholangiography is a safe and direct means of identifying this entity.
- Cholangiocarcinoma and amyloidosis have been reported as late sequelae of CHF.
Prognosis
- Most patients do well. If bleeding from varices can be controlled and renal failure does not occur, the prognosis in CHF is expected to be favorable.
- As many as 25% of patients may eventually succumb to renal failure.
- Renal involvement in neonates and young infants with CHF carries a poor prognosis, with most of these patients dying of renal failure within the first year of life.
- Other major causes of death include sepsis with ascending cholangitis and hepatic failure.
Miscellaneous
Medicolegal Pitfalls
The lack of awareness of congenital hepatic fibrosis (CHF) often leads to its misdiagnosis as cirrhosis.
Recognized associations with CHF-ARPKD include the following:
- Congenital heart disease
- Pulmonary hypertension
- Intestinal lymphangiectasia
- Caroli disease
- Pulmonary fibrosis
- Cerebellar vermis hypoplasia
- Congenital ataxia
- Pancreatic fibrosis
Special Concerns
Most neonates and young infants with CHF who have renal involvement die of renal failure within the first year of life. Coexisting renal involvement may remain asymptomatic until early adulthood. CHF with predominant hepatic involvement may remain undiagnosed for years because of left lobe involvement and normal liver function test results. Percutaneous liver biopsy may fail to reveal the classic histological changes in CHF.
Caroli disease
The original description of this disease was made by Jacques Caroli (1958).13 Caroli disease is characterized by nonobstructive saccular or fusiform dilatation of the intrahepatic bile ducts. The dilatations may involve a part of the liver or the whole liver. Although the classic hepatic fibrosis observed in CHF is not present in Caroli disease, its association with renal involvement is well recognized. Caroli disease is classified into the following 2 types:14
- Caroli disease: Caroli disease is unassociated with CHF and is a rare variety. Originally described by Caroli, this form is characterized by segmental saccular and communicating intrahepatic bile duct ectasia and is frequently associated with cholangitis and stone formation without fibrosis.
- Caroli syndrome: Caroli syndrome is more common and is associated with CHF. Bile duct dilatation is less prominent in this form. Esophageal varices, portal hypertension, and liver failure are recognized complications. Both types are associated with renal abnormalities consistent with ARPKD.
The major clinical feature is recurrent cholangitis, which may be complicated by intrahepatic calculi and hepatic abscess formation. Patients usually present with fever and abdominal pain, with or without jaundice. Hepatosplenomegaly is commonly detected upon physical examination
As in CHF, the disease may be segmental and limited to one lobe, usually the left lobe of the liver. Moreover, renal involvement with cortical cysts and features of medullary sponge kidneys occur in as many as 25% of cases. Recognized associations of Caroli disease include choledochal cysts, medullary sponge kidney,15 and ARPKD.
Caroli disease is being diagnosed more frequently because of improved diagnostic facilities.16 Diagnosis is usually made through endoscopic or percutaneous cholangiography; more recently, MRCP has emerged as the diagnostic modality of choice.
The presence of saccular dilatation in the bile duct results in stagnation of bile, bile sludge formation, and superinfection, usually in the form of repeated cholangitis.
Therapy in Caroli disease is similar to that for CHF with cholangitis. Antibiotic therapy may be sufficient, but measures to obtain sufficient biliary drainage and to relieve symptoms must be implemented. Overall management depends on the clinical features, history of recurrence, results of culture, and severity of hepatic and renal involvement. Ursodeoxycholic acid has been proposed as an adjuvant treatment in patients with lithiasis. In severe cases, lobectomy of the affected lobe may be required. Liver transplantation is considered in patients with recurrent cholangitis and extensive bilateral involvement.
More on Congenital Hepatic Fibrosis |
| Overview: Congenital Hepatic Fibrosis |
| Differential Diagnoses & Workup: Congenital Hepatic Fibrosis |
| Treatment & Medication: Congenital Hepatic Fibrosis |
 Follow-up: Congenital Hepatic Fibrosis |
| Multimedia: Congenital Hepatic Fibrosis |
| References |
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References
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Further Reading
Keywords
congenital hepatic fibrosis, CHF, biliary fibroadenomatosis, hepatic fibroangioadenomatosis, fibrous angio adenomatosis, ductal plate malformation, autosomal recessive polycystic kidney disease, ARPKD, portal hypertension, renal cystic disease, Caroli disease, autosomal dominant polycystic kidney disease, ADPKD, cholangitis, hematemesis, melena, cholestasis, hepatomegaly, cirrhosis, hypersplenism, nephromegaly
