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Gastroenterology1 paper

Fibropolycystic disease of liver

Last edited: 4/16/2026

Overview

Fibropolycystic disease of the liver is characterized by the presence of multiple cysts and fibrous tissue within the liver, often associated with additional congenital anomalies 1.

Diagnosis

  • Presence of multiple hepatic cysts and fibrosis on imaging (ultrasound, CT, MRI) 1.
  • Clinical association with other congenital abnormalities such as neonatal pyloric stenosis, craniostenosis, acromegaly, and neurofibromatosis 1.

    • Management

  • No specific pharmacological treatments mentioned; management is largely supportive and symptomatic 1.
  • Regular monitoring for complications such as portal hypertension and liver function abnormalities 1.

    • Special Populations

  • Pediatrics: Neonatal pyloric stenosis and other congenital anomalies are noted in pediatric cases, requiring multidisciplinary care 1.
  • Comorbidities: Presence of conditions like acromegaly and neurofibromatosis may necessitate additional specialist management 1.

    • Key Recommendations

  • Conduct thorough imaging (ultrasound, CT, MRI) to confirm the presence of hepatic cysts and fibrosis 1. (Evidence: Moderate)
  • Evaluate for associated congenital anomalies, particularly in pediatric patients, to guide comprehensive care 1. (Evidence: Moderate)
  • Implement regular monitoring for liver function and complications due to the progressive nature of the disease 1. (Evidence: Expert opinion)

    • References

    1 Carty JE, Jones CT. Juvenile hepatic fibropolycystic disease and other abnormalities. Postgraduate medical journal 1978. link

    Original source

    1. [1]
      Juvenile hepatic fibropolycystic disease and other abnormalities. — Carty JE, Jones CT Postgraduate medical journal (1978)
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