Inferior Vena Cava (IVC) Compression Secondary to ADPKD: A Diagnostic Dilemma

Introduction: Adult polycystic kidney disease (ADPKD) is one of the most common life-threatening genetic diseases. It is an inherited condition where fluid-filled cysts develop and enlarge in both kidneys and multiple other organs. It is the fourth leading cause of kidney failure, with over 50% of ADPKD patients developing kidney failure by age 50. Interestingly, cysts can enlarge and become symptomatic, with variable symptoms ranging from non-specifiC to mass effect. We present an interesting case of a patient who had symptoms of inferior vena cava (IVC) compression that improved with bilateral nephrectomies. Case Description: A 51-year-old female with a history of ESRD secondary to ADPKD had been receiving hemodialysis (HD) for several years. She was relatively stable on hemodialysis; however, she was usually noted to have bilateral lower limb edema despite having clear chest sounds. The usual thought process was that her target EDW needed to be changed, and more ultrafiltrate needed to be removed. However, every time we tried, her edema persisted. On abdominal examination, we found a distended abdomen.There was suspicion that this might be related to IVC compression. A bedside ultrasound showed large kidneys, leading to the decision to proceed with a CT scan of the abdomen. The CT scan revealed bilateral enlarged kidneys (left: 22x22x12 cm, right:29x19x12 cm) with evidence of IVC compression secondary to mass effect. She was referred to the urology team, who performed bilateral nephrectomies. Her symptoms improved tremendously postoperatively, and she is currently undergoing HD with no active complaints. Discussion: ADPKD remains one of the leading causes of kidney failure. If the kidneys are large enough, they can cause symptoms related to the mass effect. Symptoms related to mass effect compression against surrounding organs are not uncommon. It can compress vascular structures, leading to symptoms of ischemia if compressing the aorta, or symptoms of IVC distention. IVC compression symptoms are mainly related to lower circulatory venous congestion, which can cause edema, congestion, and discomfort. A point of interest is when it causes these symptoms in patients who are in ESRD, making it difficult to judge their volume status. The incidence of IVC compression in ADPKD is not well established, but removal of kidneys appears to result in symptomatic relief.