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Caroli's disease complicating pregnancy

C D Adair1, R Castillo, R W Quinlan

  • 1Baptis Medical Center, Jacksonville, FL, USA.

Southern Medical Journal
|July 1, 1995
PubMed
Summary
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Caroli's disease in pregnancy can lead to severe maternal and fetal complications, including sepsis and fetal distress. This case highlights the critical need for vigilant monitoring and interdisciplinary care during pregnancy with this rare condition.

Area of Science:

  • Hepatology
  • Obstetrics
  • Critical Care Medicine

Background:

  • Caroli's disease, a rare congenital condition characterized by congenital dilatation of the intrahepatic bile ducts, presents unique challenges during pregnancy.
  • Pregnancy in patients with Caroli's disease requires careful consideration due to potential complications affecting both mother and fetus.

Observation:

  • A patient with Caroli's disease experienced an uneventful first pregnancy.
  • The second pregnancy was complicated by an acute episode of Caroli's disease, leading to maternal sepsis, disseminated intravascular coagulopathy, and septic shock.
  • Fetal distress occurred, necessitating a cesarean section for delivery.

Findings:

  • The patient developed acute ascending cholangitis, disseminated intravascular coagulopathy, and septic shock during her second pregnancy.

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  • Cesarean delivery was required due to fetal distress.
  • The mother required prolonged critical care and interdisciplinary consultation post-operatively.

    • Implications:

    • This case represents the first report of Caroli's disease in pregnancy, emphasizing the potential for severe maternal and fetal complications.
    • Management requires a multidisciplinary approach involving hepatologists, obstetricians, and critical care specialists.
    • Increased awareness and proactive management strategies are crucial for improving outcomes in pregnant patients with Caroli's disease.