CASE
STUDY

Further
Reading

Feedback

FURTHER READING

ADULT POLYCYSTIC  KIDNEY DISEASE

This autosomal dominant condition may present in infancy or childhood, but it typically presents in the third or fourth decade.

The kidneys contain numerous cysts in involving all portions of the nephron and therefore involve the cortex and the medulla. The cysts vary in size from microscopic to macroscopic. They compress functioning nephrons, resulting in progressive deterioration in renal function, eventually resulting in renal failure.

Approximately one third of patients have liver cysts, which are usually asymptomatic. Cysts occur less frequently in the spleen, pancreas, and lungs. Between 15%-20% of patients have berry aneurysms of the circle of Willis, resulting in an increased incidence of subarachnoid hemorrhage.

In early stages, the kidneys may be enlarged and contain more cysts than expected for the patient's age. In late stages, the kidneys are large and contain numerous cysts with little or any interviewing normal parenchyma[1]. Sonography easily diagnoses renal polycystic disease by revealing numerous cortical cysts of varying sizes, often with irregular margins. This last feature is distinct from multiple simple cysts, where smooth margins are usual [2]. Inracystic infection or hemorrhage may cause echogenic debris or focal  calcification in the wall (Picture1).

Picture1.

Ultrasound is the preferential modality to establish the diagnosis. When renal polycystic disease is detected, immediate family members who are at risk should be screened with sonography. Affected children may be detected early in this way.

REFERENCES:
[1]Abdominal Ultrasound. E.E.Sauerbrei, K.T.Nguyen, R.L.Nolan. 1992.
2]Sonography of the Abdomen. R.B.Jeffrey, P.W.Rolls. 1995