Polycystic liver disease with lethal abdominal wall rupture: a case report

Area of Science:

• Hepatology
• Nephrology
• Genetics

Background:

• Polycystic liver disease (PLD) is a common manifestation of autosomal dominant polycystic kidney disease (ADPKD).
• While dialysis manages renal failure in ADPKD, liver failure necessitates transplantation, yet it's often not indicated due to preserved liver function.
• Giant hepatic cysts in PLD can significantly impair quality of life and impact mortality.

Observation:

• A 54-year-old woman with PLD and ADPKD presented with abdominal pain due to liver cyst infection and abdominal wall herniation.
• Despite multiple surgeries for liver volume reduction and recurrent incisional hernias, her condition deteriorated with skin ulcers, ascites, and respiratory distress.
• The patient experienced spontaneous abdominal wall ruptures with significant ascites discharge, ultimately succumbing to sepsis from drug-resistant Enterococcus.

Findings:

• This case illustrates the rapid progression of hepatic cyst enlargement in ADPKD, leading to severe complications.
• The patient's quality of life was drastically reduced, and despite the severity, liver transplantation was not pursued.
• Ruptured abdominal wall and sepsis were the terminal events in this complex ADPKD case.

Implications:

• Management of advanced PLD in ADPKD requires careful consideration of surgical interventions and transplantation timing.
• This case underscores the challenges in managing severe PLD when liver function is preserved but cyst burden is high.
• Further discussion on therapeutic strategies, including indications for liver transplantation in PLD, is warranted.