Epidemiology & Risk Factors

  • Simple Cysts: Common, incidence increases with age (>50% of individuals >50 yo have simple cysts).
  • Autosomal Dominant Polycystic Kidney Disease (ADPKD): Most common inherited kidney disease; PKD1 (chromosome 16, 85%, more severe) or PKD2 (chromosome 4, 15%) mutations.
  • Acquired Cystic Kidney Disease (ACKD): Occurs in pts with ESRD on dialysis (risk increases with duration of dialysis; up to 90% after 10 years).
  • Medullary Sponge Kidney: Congenital ectasia of collecting ducts; associated with nephrolithiasis and recurrent UTIs.

Clinical Features

  • Simple Cysts: Asymptomatic, incidental finding on imaging. Rare flank pain or hematuria if cyst ruptures/infects.
  • ADPKD:
    • Bilateral flank pain, hematuria, progressive renal insufficiency.
    • Extrarenal manifestations: Intracranial berry aneurysms (highest yield association), hepatic cysts, pancreatic cysts, mitral valve prolapse (MVP), colonic diverticula, abdominal/inguinal hernias.
    • Physical Exam: Bilateral palpable flank masses, HTN (due to localized ischemia driving renin-angiotensin activation).
  • ACKD: Often asymptomatic; presents with hematuria, flank pain, or sudden retroperitoneal hemorrhage.

Diagnosis

  • Initial: Renal Ultrasound (US).
    • Simple Cyst Criteria: Anechoic, thin/smooth wall, posterior acoustic enhancement, no septations/calcifications (Bosniak I). No further follow-up required.
    • Complex Cyst Criteria: Thick walls, septations, calcifications, solid components, contrast enhancement (Bosniak II-IV).
  • Confirmatory / Characterization: Contrast-enhanced CT or MRI of abdomen/pelvis (essential for grading complex cysts using the Bosniak Classification to rule out Renal Cell Carcinoma [RCC]).
  • Genetic Testing: Done in atypical ADPKD cases or for family screening if US is equivocal.

Differential Diagnostics

  • Renal Cell Carcinoma (RCC): Diff by solid enhancing components on CT, fever, weight loss, cachexia, and a Bosniak IV classification.
  • Renal Abscess: Diff by systemic infectious symptoms (fever, chills), leukocytosis, and a complex cystic structure with a thick, irregular wall on US.
  • Hydronephrosis: Diff by US showing dilation of the renal pelvis and calyces in a branching pattern, rather than discrete parenchymal cysts.

Management

  • Simple Cyst:
    • Asymptomatic: No treatment or follow-up needed. c
    • Symptomatic (Pain/Obstruction): Percutaneous aspiration with sclerotherapy.
  • Complex Cyst (Bosniak Class IIF - IV):
    • Bosniak II/IIF: Serial imaging follow-up (US or CT).
    • Bosniak III/IV: Surgical resection (partial or total nephrectomy) due to high risk of malignancy.
  • ADPKD:
    • Strict BP Control: Target <130/80 mmHg; ACEi or ARBs are first-line (counters renin-angiotensin-aldosterone system [RAAS] activation).
    • Slowing Cyst Progression: Tolvaptan (Vasopressin V2 receptor antagonist) for rapidly progressing disease.
    • Symptomatic control: Pain management, prompt Abx for cyst infections (lipid-soluble Abx like Ciprofloxacin or Bactrim to penetrate cysts).
    • ESRD: Hemodialysis/peritoneal dialysis or renal transplantation.
    • Screening: Do NOT routinely screen for berry aneurysms unless pt has family history of aneurysms/SAH, high-risk occupation, or symptoms.