IS Case 244: Autosomal dominant polycystic kidney disease

A. R. Klekers, MD


Imaging Sciences URMC 2008
Publication Date: 2009-05-26

History

Patient is a 48-year-old female with abdominal pain and history of liver mass on CT scan.

Findings

Bilateral numerous scattered cysts of varying sizes throughout the bilateral kidneys.

Diagnosis

Autosomal dominant polycystic kidney disease

Discussion

Adult polycystic kidney disease (APKD) is an inherited disease passed along from one generation to the next in an autonomic dominant manner. The cysts develop from collecting tubules and nephrons. Additionally, 29-73% of patients have hepatic cysts. Other extra renal manifestations such as pancreatic and splenic cysts are also less often seen. Most patients have renal failure before age 60 which is preceded by hypertension. Complications include infections, hemorrhage, cyst rupture, and renal calculi. Malignancy is rare. Ten percent of patients with APKD die of ruptured intracranial aneurysm.

In APKD MRI is most useful in patients allergic to iodinated contrast and those who have hemorrhagic or complicated cysts, as MR is best for characterization of complex lesions. On MRI patients with autosomal dominant polycystic kidneys demonstrate irregular and distorted kidneys secondary to parenchymal lesions. Uncomplicated cysts are similar to simple cysts in both configuration and signal. These lesions have low T1 and elevated T2 signal with thin walls. Some cysts may be complicated by hemorrhage and have increased signal on all sequences. There should be no contrast enhancement after administration of gadolinium.

References

  1. # Nicolau C, Torra R, Badenas C, et al. Autosomal dominant polycystic kidney disease types 1 and 2: assessment of US sensitivity for diagnosis. Radiology. 1999 Oct;213(1):273-6. [PMID: 10540671]
  2. Gabow PA. Autosomal dominant polycystic kidney disease. N Engl J Med. 1993 Jul 29;329(5):332-42. [PMID: 8321262]
  3. Khan AN, Chandramohan M, MacDonald S. Autosomal dominant polycystic kidney disease. eMedicine, December 23, 2008. http://emedicine.medscape.com/article/376995-overview

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