Nephrol Dial Transplant (1999) 14: 2775
© 1999 European Renal Association-European Dialysis and Transplant Association
Letters
The diagnostic dilemma of the unilateral cystic kidney—ADPKD with aplasia of one kidney
Clinic of Nephrology and Haemodialysis, Medical Institute, Pleven, Bulgaria
Sir,
Unilateral cystic kidney disease (UCKD) poses a diagnostic dilemma and several possibilities must be considered [1–3]. We observed one such puzzling case where a surprising explanation was found.
Case.
A 45-year-old white woman was admitted because of symptoms of renal failure. She had no history of renal disease. Physical examination revealed a large mass in the left abdomen. Laboratory data included: Hb 78 g/l, RBC 2.4x1012/l, serum creatinin 550 µmol/l. By ultrasonography the right kidney was not detected in the normal or potentially dystopic locations. The solitary left kidney was enlarged with multiple cysts ranging from 1 to 4 cm. Liver and pancreas were normal. Computed tomography confirmed the absence of renal tissue on the right side (Figure 1
). Upon questioning, the patient reported that her mother suffered from autosomal dominant polycystic kidney disease (ADPKD) and by ultrasonography ADPKD was also later found in one sibling.
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Comment.
ADPKD is characterized by bilateral multiple renal cysts, often accompanied by cyst formation in liver and pancreas. Although the disease is bilateral, renal involvement may be asynchronous and asymmetric [2–6]. Initially, palpable enlargement of one kidney is found in up to 25% of subjects [2].
There are some reports in literature concerning `unilateral polycystic kidney disease', mostly in children [4,6,7]. In the cases of Porch et al. [6] and Strand et al. [4] children had predominantly unilateral involvement, but some cysts were also present in the contralateral kidney and there was a family history of ADPKD. Ohno et al. [7] described, however, a case of a new-born with unilateral cyst formation, normal contralateral kidney and healthy parents. Based on histology, a diagnosis of ADPKD was made.
In adults, unilateral polycystic kidney disease has been rarely described in urological literature [5,8] and the interpretation of some cases remains in doubt (1). Kossow and Meek [3] reported a 79-year-old woman with unilateral cysts, but these were apparently of the multicystic variety. The patient of Lee et al. [5] was aged 72 years, but no family history of ADPKD and multiple cysts in one kidney without cysts in the contralateral kidney. The diagnosis was based upon histological similarity to ADPKD. These pathological descriptions are more reminiscent of multicystic dysplastic kidneys than of ADPKD.
Exclusively unilaterial cyst formation without histological documentation has recently also been reported by Hwang et al. [9].
Our case differs from the above reports by having truly unilateral ADPKD secondary to aplasia of the contralateral kidney. The diagnosis of ADPKD was confirmed by family history. Apparently, the ADPKD phenotype was realized in a solitary kidney.
References
- Cho KJ, Thornbury JR, Bernstein J, Heidelberger KP, Walter JF. Localized cystic disease of the kidney: angiographic-pathologic correlation. AJR 1979; 132: 891–895[Abstract]
- Gabow PA, Grantham JJ. Polycystic kidney disease. In: Schrier RW, Gottschalk CW (eds) Diseases of the Kidney. Little, Brown and Company, 1993: 535–569
- Kossow AR, Meek JM. Unilateral adult polycystic kidney disease. J Urol 1982; 127: 297–299[Medline]
- Strand WB, Rushton HG, Markle BM, Kapur S. Autosomal dominant polycystic kidney disease in infants: asymmetric disease mimickring a unilateral renal mass. J Urol 1989; 141: 1151–1153[Medline]
- Lee JT, McClennan BL, Kissane JM. Unilateral polycystic kidney disease. AJR 1978; 130: 1165–1167[Medline]
- Porch P, Noe HN, Stapleton RB. Unilateral presentation of adult-type polycystic kidney disease in children. J Urol 1986; 135: 744–746[Medline]
- Ohno I, Haruki S, Morita M, Saito K. A case of unilateral renal cystic disease in a newborn. Clin Nephrol 1995; 43: 204–205[Medline]
- Curry NS, Chung CJ. Unilateral renal cystic disease in an adult. Abdom Imaging 1994; 19: 366–368[Medline]
- Hwang DY, Ahn C, Lee JG, Lee JS. Unilateral renal cystic disease in adults. Nephrol Dial Transplant 1999; 14: (in press)
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