ROENTGENOLOGIC TRIAD OF CONGENITAL MULTICYSTIC KIDNEY

¹ Associate Professor of Radiology.

Congenital multicystic kidney is the most common cause of an abdominal mass in infancy. The disease may persist into the adult life and be incidentally discovered. Although it became a distinct pathologic entity in 1955, clinical and roentgenologic diagnosis of a congenital multicystic kidney could not be made with certainty. This is because the diagnostic features consisting of a unilateral nonfunctioning kidney with or without a palpable flank mass are also observed in Wilms’ tumor in children and renal cell carcinoma in adults, congenital unilateral hydronephrosis, renal vein thrombosis, renal artery occlusion, renal xanthogranulomatous pyelonephritis and renal agenesis. In view of lack of a diagnostic procedure to make an accurate diagnosis, these patients have been subjected to surgical exploration to confirm the clinical suspicion of congenital multicystic kidney, so as to make certain that one is not dealing with the more serious or malignant renal diseases considered in the differential diagnosis.

Roentgenologic detection of pathologically correlated diagnostic features can be made by employing the intravenous urogram, retrograde pyelogram, and abdominal aortogram, in combination. These procedures bring out selectively functional and pathologic features of congenital multicystic kidney. The diagnostic features each procedure displays complement the other two, to establish the pathognomonic triad of roentgenologic signs for congenital multicystic renal disease which is outlined in Table II.

These diagnostic procedures not only make the primary diagnosis of congenital multicystic kidney possible, but also reveal the nature of the underlying congenital anomaly of the contralateral urinary tract, which occurs in 30 per cent of these patients. Consequently, appropriate therapeutic measures may be undertaken to prevent deterioration of the only functioning kidney.

Emphasis is placed on the roentgenologic recognition and an awareness of the nature and significance of this innocuous renal lesion which, when left alone, is not known to be harmful to the body. If one is cognizant of the diagnostic value of the roentgenologic triad described in this rereport, an accurate diagnosis can be made and consequently surgical intervention for exploration can be averted. This is important since congenital multicystic kidney is the most common cause of abdominal masses in children.

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