Liver cyst infection in patients with autosomal dominant polycystic kidney disease (ADPKD) is a rare but life-threatening complication. Diagnosis and treatment remain challenging. We report the case of a 64-year-old kidney transplant patient presenting with fever and abdominal pain. The diagnosis of liver cyst infection was made by positron emission tomography scan (PET/CT). Moreover, the course of our patient illustrates the interest of subsequent PET/CT during follow-up as our patient failed on antibiotherapy and required liver cyst drainage.
Potter syndrome (PS) is a term used to describe a typical physical appearance, which is the result of dramatically decreased amniotic fluid volume secondary to renal diseases such as bilateral renal agenesis (BRA). Other causes are abstraction of the urinary tract, autosomal recessive polycystic kidney disease (ARPKD), autosomal dominant polycystic kidney disease (ADPKD) and renal hypoplasia. In 1946, Dr Edith Potter characterized this prenatal renal failure/renal agenesis and the resulting physical characteristics of the fetus/infant that result from oligohydramnios as well as the complete absence of amniotic fluid (anhydramnios). Oligohydramnios and anhydramnios can also be due to the result of leakage of amniotic fluid from rupturing of the amniotic membranes. The case reported below, concerns of stillborn boy with potter syndrome.
A 61-year-old patient with autosomal dominant polycystic kidney disease (Irazabal class 1E) in whom renal function had decreased and kidney size had increased over the past 3 years (change in serum creatinine, 1.3 mg/dL to 1.5 mg/dL; change in total kidney volume, 5632 cm3 to 7301 cm3) was treated with tolvaptan 60 mg/day. After 8 years of tolvaptan treatment, serum creatinine remained at 1.51 mg/dL, and total kidney volume was at 6812 cm3. Adequate salt intake and good weight control may have resulted in good outcomes, even in patients with treatment-resistant giant cystic kidney disease.
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