CASE REPORTS
Another Variant of the Multicystic
Kidney
By John W. Duckett and R. Bruce Filmer Philadelphia,
Pa.
The multicystic kidney is regarded by some authorsle3 as being an extreme degree of hydronephrosis secondary to congenital obstruction of calyces, pelvis, or ureter. This is a case of pelviureteric obstruction, ureteral atresia, and multicystic renal dysplasia. A l-day-old female was admitted with an abdominal mass and mild respiratory distress syndrome. Physical examination revealed a palpable normal right kidney and a mobile mass 5 x 6 cm in the left lumbar area. Intravenous urogram the day of admission showed a normal right kidney and ureter and a large mass occupying the left renal area. During the intravenous urogram, a definite rim (Fig. 1) was noted, outlining what seemed to be the boundary of the palpable mass. There were no other areas of curvilinear opacification seen during the urogram. A provisional diagnosis of left hydronephrosis was made. Through a left-transverse-anterior upper-abdominal incision the mass was exposed. Dissection revealed a large “cyst” attached to a proximally atretic ureter with patent distal ureter of normal caliber. Capping part of this cyst was renal parenchyma with gross features of multicystic renal dysplasia. The cystic mass and its ureter were excised. The postoperative course was uneventful. Gross study of the specimen failed to show any communication between the large cyst and the adjacent smaller cysts. The proximal ureter was nonpatent. Histologic examination of the wall of the larger cyst and one of the smaller cysts showed flattened epithelium supported by muscle tissue, identical with a hydronephrotic renal pelvis and calyx. Microscopy of the remaining
0 1976 by Grune & Stratton, Inc.
Journal of Pediatric Surgery, Vol. 11, No. 1 (February), 1976
93
94
CASE REPORTS
Fig. 2. (A) lntraoperative and (B) postoperative photographs of the surgical specimen. The arrow indicates the larger cyst (renal pelvis). The proximal ureter is atretic.
tissue was consistent mal renal tissue.
with
multicystic
renal
dysplasia.
There
was complete
absence
of any
nor-
DISCUSSION The rim-like opacification of this renal mass on intravenous urogram lead to the diagnosis of hydronephrosis. Felson and others’*’ have described “calyceal crescents” in multicystic kidneys as seen during intravenous urography. This has been attributed to a corona of glomeruli encircling the cyst. However, our patient only showed opacification of the largest cyst which histologically was consistent with a renal pelvis tissue. We agree with Robson, et al.’ that the multicystic kidney could be at the extreme end of the congenital hydronephrosis spectrum.
REFERENCES 1. Felson B, Cussen LJ: The hydronephrotic type of unilateral congenital multicystic disease of the kidney, Semin Roentgen01 l&l 13-123, 1975 2. Robson WJ, Rudy SM. Johnston JH: Pelviureteric obstruction in infancy, J Ped Surg,
this issue, pp 57-61 3. Filmer RB, Taxy JS: Renal dysplasia and hypoplasia, in Kelalis P, King LR (eds): Pediatric Urology. Saunders (in press) 4. Doberti BS, Escudero G, Cited by Felson and Cussen’