Adult polycystic kidney disease

Case contributed by Pranav Sharma
Diagnosis certain

Presentation

Right sided abdominal pain.

Patient Data

Age: 60 years
Gender: Male

Grossly abnormal and enlarged kidneys and liver, with minimal renal parenchyma. Small volume free fluid within the rectovesical pouch and minor right-sided peri-nephric stranding. Both kidneys demonstrate heterogenous hyperdensities within some cysts, which may reflect prior hemorrhagic or calcific material.

Case Discussion

This case is nice a demonstration of pathology. 

This is a gentleman who underwent an open right nephrectomy.

Indication: Bleeding right kidney, hemodynamically unstable and failed conservative management. Background of known polycystic kidney disease. High risk secondary to anti-coagulation given recent AVR and aortic root replacement (previously on warfarin - ceased, now on heparin infusion).

Intraoperative findings: Large right polycystic kidney with overlying hematoma surrounding the upper pole. Hemorrhagic cysts. Nil active bleed.

Histopathology report:

MACROSCOPIC

  • nephrectomy measuring 260x130x160mm
  • specimen weighs 1800g
  • entire renal parenchyma is replaced by variable sized cysts (5-30mm) which contain clear fluid or clotted blood
  • pelvis is severely dilated and contains clotted blood

MICROSCOPIC

  • sections reveal renal parenchyma replaced by multiple variably sized cysts lined by cuboidal to flattened epithelium
  • cysts involve both cortex and medulla
  • cystic dilatation of tubules and glomeruli are noted
  • no epithelial dysplasia or evidence of invasive malignancy

SUMMARY

  • features consistent with adult polycystic kidney disease

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