Nephrol Dial Transplant (1999) 14: 1999–2003
Nephrology
Dialysis
Transplantation
Case Report
Unilateral renal cystic disease in adults
Dae Yeon Hwang1, Curie Ahn1, Jung Geon Lee1, Seung Hyup Kim2, Ha Young Oh3, Yong Yim
Kim1, E. S. Lee4, Jin Suk Han1, Suhnggwon Kim1 and Jung Sang Lee1
Divisions of 1Nephrology, 2Radiology and 4Urology, College of Medicine, Seoul National University Hospital and 3Division
of Nephrology, College of Medicine, Sungkyunkwan University, Seoul, Korea
Abstract Unilateral renal cystic disease ( URCD) is
morphologically indistinguishable from autosomal
dominant polycystic kidney disease (ADPKD) except
for its unilaterality. Unlike ADPKD, URCD patients
show neither a genetic background nor progressive
deterioration in renal function; thus, the differential
diagnosis of URCD from ADPKD is important. Only
a few cases of URCD have been reported. This study
reports two cases of URCD in adults together with a
literature review. We identified these two cases using
abdominal computerized tomography and family
screening with renal ultrasonography.
Key words: autosomal dominant polycystic kidney disease; unilateral renal cystic disease
Introduction
Unilateral renal cystic disease ( URCD) is a multicystic
disease, characterized by cysts of varying sizes localized
in a diffusely enlarged kidney without forming a distinct encapsulated mass. Except for its unilateral localization, the gross and histological findings of URCD
are indistinguishable from those of autosomal dominant polycystic kidney disease (ADPKD) [1]. However,
unlike ADPKD, URCD patients show neither a genetic
background nor progressive deterioration in renal
function.
Even though the differential diagnosis of URCD
from ADPKD is important, especially from the genetic
and prognosis standpoint, only a few cases of URCD
have been reported. Since an ADPKD clinic was
opened at Seoul National University Hospital, Seoul,
Korea, 83 ADPKD probands have visited the clinic;
among these, two patients (2.4%) with URCD were
identified by computerized tomography (CT ) and
family screening studies. This study reports two cases
of URCD in adults, together with literature reviews.
Correspondence and offprint requests to: Curie Ahn MD, PhD,
Division of Nephrology, Seoul National University Hospital,
28-Yunkun-dong, Chongro-ku, Seoul, 110-744, Korea.
Cases
Case 1
A 31-year-old man visited the ADPKD clinic with
pain in the right flank. He had been experiencing a
mild, lancinating pain in the right flank area for several
years. He experienced a single episode of gross haematuria 1 year prior to visiting the clinic; otherwise, no
other gastrointestinal, respiratory, cardiovascular or
urological symptoms were observed.
Upon physical examination, blood pressure was
measured as 120/80 mmHg. A 15×8 cm hard mass
with a nodular surface was palpated at the right upper
and middle abdomen. No other abnormalities were
found. No family illnesses including renal diseases were
reported.
The urinalysis was normal with SG 1.015. Other
laboratory findings were also within normal limits,
including serum creatinine (1.0 mg/dl ). Abdominal CT
revealed an enlarged right kidney (~18×16×10 cm)
filled with variable sized round, well-marginated multiple cysts with no capsule formation (Figure 1). The
cysts were separated by parenchymal bands, which
were enhanced with contrast media. Two very small
cortical cysts were found in the left kidney. No cysts
were detected in other intra-abdominal organs. The
echocardiography and colon examination were normal.
Renal ultrasound was performed on both of the
patients, the parents and his siblings and none of them
was observed to have cystic renal disease.
During the follow-up period of 18 months, the
subject maintained a good renal function.
Case 2
A 44-year-old man with mild to moderate left flank
pain for 5 months was seen at the clinic. Upon
examination, blood pressure was measured as
135/100 mmHg. A 13×8 cm sized mass was palpated
at the left flank area. Except for the mass, no other
abnormalities were noted. Serum creatinine was
1.1 mg/dl, and urinalysis was normal. An excretory
urogram (IVP) showed a normal right kidney and a
© 1999 European Renal Association–European Dialysis and Transplant Association
2000
D. Y. Hwang et al.
Fig. 1. Case no. 1. Abdominal CT shows an enlarged right kidney filled with variable sized round, well-marginated multiple cysts with no
capsule formation. The cysts were separated with parenchymal bands, which were enhanced with contrast media.
markedly enlarged left kidney with stretched appearance of the left pelvocaliceal system (Figure 2).
Abdominal CT showed multiple variable sized cysts
separated by contrast-enhanced band-like septa in the
markedly enlarged (18×11×15 cm) left kidney. Some
residual renal parenchyma was seen in the upper and
lower pole. The right kidney was entirely normal.
Other intra-abdominal organs appeared to be normal
except for a very small simple cyst in the liver. The
echocardiography and colon examination were normal.
His parents were both dead; his father died of liver
cirrhosis at the age of 45 years and his mother had
died at age 75 years. None of the other family members
had a history of any renal diseases including cysts or
genetic syndromes. None of his three siblings screened
with abdominal USG showed any cysts.
Fig. 2. Case no. 2. An excretory urogram depicted a markedly enlarged left kidney with stretched appearance of the left pelvocaliceal
system. Note that abdominal CT findings are the same as those of case no. 1 except affected side. The right kidney was entirely normal.
Unilateral renal cystic disease in adults
2001
During the course of the follow-up of 15 months,
the subject’s renal function was well maintained.
Discussion
In the late 1970s, several studies reported that URCD
might be different from ADPKD [1–3]. Levine et al.
[4] proposed the term ‘unilateral renal cystic disease
( URCD)’ as a distinct disease entity in 1989. The
clinical importance of URCD is to make a differential
diagnosis from ADPKD. As this study shows, URCD
has at least three aspects different from ADPKD
(i) unilateral localization, (ii) negative family history
and (iii) no progression to chronic renal failure.
The pathological findings of URCD are not different
from those of ADPKD [2], showing numerous epithelial cell-lined cysts interposed among patches of noncystic renal parenchyma. However, grossly, those cysts
were localized exclusively in one kidney. In earlier
studies, the unilaterality was confirmed by examining
nephrectomized specimens or autopsy tissues [6,7]. As
more imaging techniques improve, it will become easier
to confirm the unilateral localization of lesions of
URCD. Since CT became popular, the surgical confirmation of URCD is no longer needed if patients
have characteristic CT findings in combination with
genetic and clinical manifestations [4,5]. The frequency
of 2.4% in our patients is higher than that of western
countries. This estimation of the frequency might be
higher than the actual prevalence of URCD in general
Korean populations, as our hospital is a third referral
hospital in Korea.
Usually, URCD patients have no cysts in the
unaffected kidney or in other intra-abdominal organs.
However, two small cortical cysts of 0.7 and 0.5 cm in
the unaffected kidney in case 1 and a small liver cyst
of 0.7 cm in case 2 were observed. These cysts were
very likely to be the simple cysts that are commonly
present in many adults. The co-existence of simple
renal cysts in the unaffected kidney in URCD has been
reported in another study [5]. However, the presence
of simple cysts in other organs may be ominous,
especially in children. In children, ADPKD may manifest as URCD [8,9]. In some children, careful family
history taking and radiological studies of their parents
Table 1. Clinical features of URCD patients reported
Reference
Age/Sex
Family
history
Involved
kidney
Radiological
study
Clinical findings
Outcome
Bergman et al.,
1964 [17]
57 years/
M
No
Right
IVP
Nephrectomy
Sellas et al.,
1972 [15]
37 years/
M
No
Left
IVP
Angiography
Lee et al.,
1978 [2]
72 years/
M
No
Left
Cho et al.,
1979 [3]
59 years/
M
54 years/
M
No
Left
UNK
Right
CT, IVP
USG
Angiography
IVP
Angiography
IVP
USG
Angiography
UNK
Right
No
Right
No
by USG
No
Left
Curry et al.,
1994 [5]
Present case,
1998
79 years/
F
30 years/
M
14 years/
M
57 years/
M
31 years/
M
Left
IVP, CT
Angiography
CT
Angiography
CT, IVP
Angiography
CT, IVP
Abdominal mass,
dyspepsia, hypertension,
proteinuria, MH
Flank pain,
renal stone,
gross haematuria
Lower abdominal pain,
abdominal mass,
azotaemia (Cr 1.9)
Hypertension, MH,
azotaemia (Cr 1.4)
Hypertension,
albuminuria,
MH; liver cirrhosis,
proliferative GN
Abdominal mass,
flank pain
Flank pain
No
Right
CT, USG
No
Left
CT, IVP
Middlebrook et al.,
1992 [16 ]
44 years/
M
10 months/
F
No
by USG
Right
CT, USG
Cho et al.,
1979 [3]
3 years/
F
No
Right
IVP
Kossow et al.,
1982 [1]
Levine et al.,
1989 [4]
Gross haematuria,
flank pain
Hypertension
Flank pain,
abdominal mass,
gross haematuria
Flank pain,
abdominal mass
Hypertension,
urinary tract infection,
abdominal mass
Hypertension,
abdominal mass,
gross haematuria
Nephrectomy
Nephrectomy
Nephrectomy
Autopsy
Nephrectomy
Nephrectomy
No change in
7 years
No change in
30 years
No change in
18 months
No change in
15 months
Nephrectomy
Nephrectomy
Patients included: patients with unilateral renal involvement diagnosed by imaging techniques with negative family history or patients with
unilateral renal involvement confirmed with nephrectomy.
MH, microscopic haematuria; IVP, intravenous pyelography; CT, computerized tomography; UNK, unknown; USG, ultrasonography.
2002
have revealed their parents as having ADPKD
[10,11,13]. In others, the initial unilateral disease
evolved into an asymmetric bilateral disease in a longterm follow-up period [10,12,13]. Therefore, asymmetric evolution of ADPKD should be excluded either by
phenotype screening of family members or by a long
period of follow-up.
Besides CT, family screening is an important tool
for the diagnosis of URCD. However, most of the
previous reported cases took family history only
( Table 1). Phenotypic analysis of family members using
renal ultrasound was performed in our study. Both
parents and siblings of case 1 showed no cystic diseases.
Therefore, it is concluded that URDC is not a hereditary trait. In case 2, although family screening was not
complete due to the parents’ death, no evidence of
familial disease was found either by an intensive history
taking or by renal ultrasound of his siblings. Longterm follow-up is necessary because the development
of ADPKD by a new mutation might be observed.
This study was limited due to the short duration of
the follow-up period to prove non-progressive renal
function or new cyst formations in the contralateral
kidney. However, the study was expanded to seek
extrarenal manifestations: no mitral valve prolapse or
colonic diverticulum were found, which are known to
be associated with ADPKD [14].
Only a few cases of URCD have been reported.
Recently, Curry et al. [5] summarized seven adult
URCD cases including one of their patients. Thirteen
URCD cases from the present study and previous
studies are summarized in Table 1. None of the family
members of these 13 patients were documented as
having renal disease. The initial clinical profiles of
URCD patients were not very different from those of
ADPKD. Ten out of 13 patients were males who were
diagnosed between the ages of 3 and 79 years. Among
them, hypertension was found in six, flank or abdominal pain in seven, abdominal mass in seven, and gross
haematuria in three cases. A renal stone was found in
one case and UTI in one case [15,16 ]. The clinical
manifestations of our two cases such as abdominal
mass, flank pain and gross haematuria were consistent
with other reported cases.
In the literature, several patients who had both
possible URCD and neoplastic disease have been
reported. Posso et al. [18] and Heyward [19] reported
patients with unilateral polycystic kidney and renal cell
carcinoma. Cole et al. [20] reported a case of URCD
associated with dual adenocarcinomas; Wells et al. [21]
reported a case with intra-cystic papillomas.
Regrettably, except for the case of Cole et al., no study
reported the description of family history; the study
by Cole et al. reported a suspicious hereditary renal
disease. It might be worthwhile to note that the case
reported by Posso et al. might have had multiloculated
cystic renal neoplasm; and that by Cole, ADPKD.
Although the possibility of misdiagnosis of other cystic
renal diseases exists in these cases, the possibility of
co-existing renal cell carcinoma in URCD should also
be considered.
D. Y. Hwang et al.
Besides ADPKD, multilocular cystic renal neoplasms, cystic dysplasia and multiple simple cysts are
other categories of cystic diseases. These cystic diseases
must also be differentiated from URCD. Multilocular
cystic renal neoplasms usually form discrete, encapsulated masses that are well demarcated from the adjacent
renal parenchyma [22]. Also, a cystic neoplasm grows
slowly and expands to displace normal renal parenchyma and does not contain the islands of enhancing
parenchyma on CT, which are found in URCD. A
multicystic dysplastic kidney is usually diffusely cystic,
severely dysplastic and non-functioning due to
ureteropelvic occlusion. It is usually easily distinguished from URCD with sonography, CT and radionuclide studies. The collecting system draining the
dysplastic segment is atretic or obstructed and, therefore, is not usually opacified on contrast-enhanced CT,
whereas the collecting system in URCD shows only
the displacement [23]. Multiple simple cysts may be
difficult to distinguish from URCD when confined to
one kidney. However, they are not as numerous as
in URCD.
Acknowledgements. We thank Ms Bina Choi for her excellent secretarial service.
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Received for publication: 13.1.99
Accepted in revised form: 26.3.99