Further Outpatient Care
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Patients with congenital hepatic fibrosis are usually seen regularly at pediatric gastroenterology, hepatology, and nephrology clinics.
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In complicated cases, other disciplines are involved for regular follow-up assessment, including pediatric infectious disease, vascular surgery, and transplant surgery.
Further Inpatient Care
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Recurrent episodes of GI bleeding, recurrent cholangitis, and the extent of renal impairment largely influence the course of the disease.
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Congenital hepatic fibrosis (CHF) is also associated with varied clinical conditions that require consultations, resulting in further inpatient care and management (see Consultations).
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With severe bleeding varices, the child may require admission to the intensive care unit.
Inpatient & Outpatient Medications
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No specific medication is available for congenital hepatic fibrosis.
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Medication therapy is usually directed at treatment of complications, such as recurrent cholangitis, sepsis, or renal impairment.
Transfer
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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).
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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.
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In complicated cases, transfer to a tertiary care center is recommended to facilitate the consultation and contribution of other services, such as pediatric surgery, vascular surgery, and transplant surgery.
Complications
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Complications in congenital hepatic fibrosis are mainly related to its association with autosomal recessive polycystic kidney disease (ARPKD), resulting in renal impairment, bleeding varices, and recurrent cholangitis.
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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.
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Cholangiocarcinoma and amyloidosis have been reported as late sequelae of congenital hepatic fibrosis.
Prognosis
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Most patients do well. If bleeding from varices can be controlled and renal failure does not occur, the prognosis in congenital hepatic fibrosis is expected to be favorable. Respiratory insufficiency in the first month of life and renal insufficiency are primary determinant factors of mortality.
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As many as 25% of patients may eventually succumb to renal failure.
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Renal involvement in neonates and young infants with congenital hepatic fibrosis carries a worse prognosis, with most patients dying of renal failure within the first year of life.
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Other major causes of death include sepsis with ascending cholangitis and hepatic failure.
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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.
