Polycystic liver - A cyst-ridden abdomen.

Area of Science:

• Nephrology
• Gastroenterology
• Medical Imaging

Background:

• Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by cyst formation in the kidneys and often other organs.
• While kidney function may remain stable for years, growing cysts can cause significant morbidity due to mass effect.
• Liver cysts are a common manifestation of ADPKD, contributing to abdominal symptoms.

Purpose of the Study:

• To illustrate the potential for massive cyst volume in autosomal dominant polycystic kidney disease.
• To highlight the abdominal distension and morbidity caused by extensive cyst growth.
• To underscore the ongoing need for effective therapeutic interventions for ADPKD.

Main Methods:

• Case report of a 44-year-old female patient with established ADPKD.
• Clinical evaluation for symptoms including weight loss, early satiety, and abdominal distension.
• Abdominal ultrasonography to identify liver cysts.
• Abdominal magnetic resonance imaging (MRI) to assess the extent and volume of kidney and liver cysts.

Main Results:

• The patient presented with progressive abdominal distension, weight loss, and early satiety.
• MRI revealed a large number of kidney and liver cysts, with the largest measuring 70mm.
• The cysts occupied a significant portion of the abdominal cavity, causing marked distension.

Conclusions:

• Massive cyst volume is a primary cause of morbidity in ADPKD.
• The case demonstrates the extreme abdominal distension achievable by ADPKD-related cysts.
• Novel and effective therapies are crucial for managing the symptomatic burden of ADPKD.