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Journal of Pediatric Urology (2015) xx, 1.e1e1.e6

Ultrasound classification of solitary renal


cysts in children
B. Karmazyn a, A. Tawadros b, L.R. Delaney a, M.B. Marine a,
M.P. Cain c, R.C. Rink c, S.G. Jennings d, M. Kaefer c

a
Department of Radiology and Summary In 20.2% (18/89) of the children, the cysts
Imaging Sciences, Indiana increased in size.
University School of Medicine, Introduction A definitive diagnosis was obtained in 8.5% (18/
Riley Hospital for Children, 705 Solitary renal cysts are typically incidentally found 212) of the children. A cystic tumor (multilocular
Riley Hospital Drive, Room 1053, in children who have undergone renal ultrasound cystic nephroma) was found in one child (Figure)
Indianapolis, IN 46202, USA (US). The main concern is a cystic tumor. There is no with a complex cyst (graded III by both radiologists).
US-based grading system for children to guide
b
Department of Radiology, management. Discussion
Memorial Hospital of South The use of a modified Bosniak classification system
Bend, 615 N. Michigan Street, Objective to grade renal cysts was found to have good inter-
South Bend, IN 46601, USA
To evaluate a US-based, modified Bosniak grading observer variability (kappa Z 0.65) in differenti-
c
system in order to differentiate between simple ating between simple and complex renal cysts. Using
Department of Urology, Indiana (grade I or II) and complex (grade II or IV) renal cysts this classification, few (<4%) renal cysts were clas-
University School of Medicine,
and guide management in children. sified as complex. Cystic tumors are rare and the
Riley Hospital for Children, 705
Riley Hospital Drive, Room 4230,
only cystic tumor (multilocular cystic nephroma) was
Indianapolis, IN 46202, USA Study design classified as complex renal cysts by the two radiol-
This was a retrospective (2003e2011) study of 212 ogists. Growth of simple, solitary renal cyst is com-
d
Department of Radiology and
children (114 females), age range one day to 17 mon (20.2%) and, therefore, if not associated with
Imaging Sciences, Indiana years (mean 8.4 years), with solitary renal cysts other imaging findings, is not an indication for a
University School of Medicine, diagnosed by US. Two radiologists, who were inde- cystic tumor.
950 W. Walnut Street, Room pendent and blinded to clinical information, graded There were limitations inherent in the retro-
E124, Indianapolis, IN 46202, USA the cysts using the modified Bosniak classification spective nature of the study and because only one
system. In children with more than one year of child had a cystic tumor.
Correspondence to: B. Karmazyn, follow-up US, the change (>10%) in cyst diameter
Department of Radiology and was evaluated. Inter-observer variability (Kappa) Conclusion
Imaging Sciences, Indiana was calculated. The modified Bosniak classification system demon-
University School of Medicine, strated good inter-observer agreement, and identi-
Riley Hospital for Children, 705 Results fied the single tumor as a complex cyst. The vast
Riley Hospital Drive, Room 1053,
Radiologists one and two saw simple renal cysts in majority of solitary renal cysts in children are simple
Indianapolis, IN 46202, USA. Tel.:
317 948 6305
96.2e96.6% (204205/212) of the children. Ten and if asymptomatic, they require no other imaging
children had complex renal cysts, as rated by either evaluation. Complex renal cysts are uncommon and
bkarmazy@iupui.edu of the radiologists. There was good inter-observer should be evaluated with a pre-intravenous and
(B. Karmazyn) agreement (kappa Z 0.65) for simple versus com- postintravenous contrast CT scan to exclude a
tawadros.alex@gmail.com plex cysts. tumor.
(A. Tawadros)
ldelaney@iupui.edu
(L.R. Delaney)
mbshelto@iupui.edu
(M.B. Marine)
mpcain@iupui.edu (M.P. Cain)
rrink@iupui.edu (R.C. Rink)
sajennin@iupui.edu
(S.G. Jennings)
mkaefer@iupui.edu
(M. Kaefer)

Keywords
Ultrasound; Children; Solitary
renal cysts; Simple cysts; Com-
plex cysts

Received 15 January 2015


Accepted 9 March 2015
Available online xxx

http://dx.doi.org/10.1016/j.jpurol.2015.03.001
1477-5131/ 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

Please cite this article in press as: Karmazyn B, et al., Ultrasound classification of solitary renal cysts in children, Journal of Pediatric
Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.03.001
+ MODEL
1.e2 B. Karmazyn et al.

Figure Longitudinal color doppler US of a complex (grade III by both radiologists) left renal cyst in a 7.2 year-old boy. The child
presented with gross hematuria. Ultrasound shows a septated cystic mass with vascularity of the septations (arrowheads) and cyst
wall (arrow). Radical nephrectomy was performed with pathologic diagnosis of multiloculated cystic nephroma.

Introduction abscess, prior renal tumor, or syndromes associated with


renal cysts (polycystic kidney disease, tuberous sclerosis,
Only a few small series have evaluated solitary renal cysts von Hippel-Lindau syndrome).
in children [1e5]. In adults, the Bosniak classification All follow-up renal US studies until December 2012 were
system of renal cysts is used to assess cystic renal cell retrieved. Medical charts were reviewed for demographic
carcinoma risk and to guide complex renal cyst manage- information, underlying urologic diseases, and symptoms.
ment [6e8]. This classification is based on computerized Other available renal imaging studies, including CT, MRI,
tomography (CT) [8]; however, in children, most solitary and VCUG, were also reviewed. The VUR side was noted, as
renal cysts are discovered using ultrasound (US), and was ureteral reimplant or Deflux injection side, if
cystic renal tumors are rare [9e11]. performed.
A modified Bosniak classification system for renal cysts,
which is based on US, has been developed for children. Review of renal ultrasound
Solitary renal cysts have now been retrospectively classified
using the new system and its reproducibility and ability to Two fellowship-trained pediatric radiologists, blinded to
identify cystic tumors has been determined. the clinical information, independently reviewed the renal
US studies. One radiologist excluded children with multiple
cysts, and then measured the maximum cyst diameter on
Material and methods the initial and available follow-up studies. Each radiologist
independently graded the renal cysts using a
Patient population US-based modification of the Bosniak classification system
(Table 1).
The institutional review board approved this Health Insur- For children with follow-up studies, change in grading
ance Portability and Accountability Act compliant study and cyst size (either absolute size change or ratio of renal
with waiver of informed consent. From the radiology in- cyst diameter to renal length) was evaluated. Any change
formation system at a tertiary care childrens hospital, all by at least 10% of the renal size was considered to be
children younger than 18 years, who underwent a renal US positive.
study, with keyword of cyst on the report, between 2003 For children with a modified Bosniak grade III or IV on
and 2011 were retrospectively identified. initial US, a different fellowship-trained pediatric radiolo-
Exclusion criteria were children with: multiple renal gist graded their available CT, or MRI studies were graded
cysts, previous renal surgery, a transplanted kidney, renal using the Bosniak classification system.

Table 1 The modified Bosniak classification system for renal cysts, based on ultrasound findings.
Grade Shape Wall Septations Calcification Content
Thickness Nodules Doppler flow Number Thickness Nodules Doppler flow
I Round 1 mm No No 0 N/A No No Noa Anechoic
II Lobulated 1 mm No No Few 1 mm No No No Debris
III N/A 1 mm No Yes Multiple 1 mm No Yes Yes N/A
IV N/A N/A Yes N/A N/A N/A Yes N/A N/A Soft tissue
a
Not including movable cyst stone.

Please cite this article in press as: Karmazyn B, et al., Ultrasound classification of solitary renal cysts in children, Journal of Pediatric
Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.03.001
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Solitary renal cysts in children 1.e3

A definitive diagnosis of renal cysts was determined by Ultrasound findings


pathology reports, or imaging demonstrating a cyst
and collecting system communication (calyceal Renal cyst grades
diverticulum). Most cysts (96.2% and 96.7% by radiologists one and two,
respectively) were simple (grade I or II). Most simple cysts
were grade I: 86.3% (183/212) by radiologist one and 74.1%
Statistics (157/212) by radiologist two.
Complex renal cysts (grade III and IV) were seen in 3.8%
Laterality of VUR and reimplant/Deflux injection was (8/212) and 3.3% (7/212) of children by radiologists one and
compared with the presence of renal cysts by the Chi- two, respectively (Fig. 1). Only radiologist one categorized
squared test. The grading system and each of the one cyst as grade IV. There were up to four septations in the
morphological features of the renal cysts were examined as cysts that were classified by either of the two radiologists
a predictor of renal tumor or development of polycystic as having few septations, and at least six in cysts classified
renal disease during the follow-up period using Chi-squared as having multiple septations.
tests. Weighted kappa was calculated to assess inter- Of the 10 renal cysts graded as grade III or IV by either
observer agreement. Analyses were performed using SAS radiologist, three children had surgical resection of the
version 9.3 (SAS Institute, Cary NC) and significance was cysts (one multilocular cystic nephroma, two simple cysts),
defined as P < 0.05. five children had CT and one had an MRI that demonstrated
simple cysts (two grade I, three grade II, one grade IIF). The
child with the grade IIF cyst had a stable cyst on US at the
Results two-year follow-up. One child did not attend follow-up US.
Other findings in the ipsilateral kidney included hydro-
Patient population nephrosis in 8% (21/212) and parenchymal echogenicity in
10% (21/212) of children.
A total of 212 (prevalence of 1.0%, 212/20,349) children
with solitary renal cysts were identified; 114 (54%) were Inter-observer variability
females and 98 (46%) were males, with a mean age of 8.4 Radiologists one and two agreed on the cyst grade classi-
years (range one day to 17 years, median 8.1 years). The fication (kappa Z 0.48) in 83% of children. When classifying
most common (>5% prevalence) indications for US by simple (grades IeII) versus complex (grades IIIeIV)
included: VUR (22.6%, 48/212); follow-up of known renal cysts, the radiologists agreed in 98% of children
cysts (14.2%, 30/212); dysfunctional voiding (13.2%, 28/ (kappa Z 0.65).
212); UTI (12.7%, 27/212); hydronephrosis (6.6%, 14/212);
neurogenic bladder (6.1%, 13/212); and abdominal pain
(5.7%, 12/212). Follow-up renal ultrasound
There was no statistical significance between side of
VUR and the presence of renal cysts (ipsilateral 62.5% (30/ Of the 212 children (42.0%), 89 had at least one year of
48), contralateral 60.4% (29/48), P Z 0.83). Thirty-eight follow-up US. The cysts resolved (40.4%, 36/89) or
children had a reimplant (n Z 16) or Deflux injection decreased in size (6.7%, 6/89) in 47.2% (42/89) of these
(n Z 2) ipsilateral to the cysts; 20 children had a contra- children. A total of 32.6% (29/89) of the cases had stable
lateral reimplant (n Z 15) or Deflux injection (n Z 5). The cysts. In 20.2% (18/89) of the children, the cysts increased
surgical procedure location and the presence of renal cysts in size; using the ratio of cyst to renal length, the relative
were not associated (P Z 0.56). size increased in 15 (16.9%) children.

Figure 1 Images of a complex left renal cyst (grades IV by one radiologist and III by the other radiologist) in a 7.4 year-old boy.
The child presented with macroscopic hematuria. a) Transverese ultrasound view of the right kidney shows a complex cystic mass
with an echogenic material. b) An axial post-intravenous-contrast CT scan of the abdomen was performed that demonstrated a
right kidney lesion with high density thought to represent a solid mass. A non-contrast CT was not performed. Radical nephrectomy
was performed. Pathology diagnosis was epithelial cyst filled with blood.

Please cite this article in press as: Karmazyn B, et al., Ultrasound classification of solitary renal cysts in children, Journal of Pediatric
Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.03.001
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1.e4 B. Karmazyn et al.

Four (three and one, by radiologists one and two, Discussion


respectively) simple cysts changed to complex cysts at
follow-up. In two of the four children, no long-term follow- Solitary renal cysts are uncommon in children, with a re-
up was performed, as the cysts were reported as stable and ported prevalence of 0.22e2% [1e5]. In the present series,
simple in the original report, one child had a CT scan the prevalence was 1%. The true prevalence, however, may
demonstrating simple (grade II) renal cysts and one other be higher as asymptomatic children were not screened.
child had a resection of the cysts, which were diagnosed as There is a challenge in providing imaging criteria to
benign on pathology. consistently and reliably differentiate between simple and
In five of the 212 children (2.4%) there was an increase in complex renal cysts. When discovered, complex renal cysts
the number of renal cysts on follow-up. The additional cysts can pose a challenge to both the physician taking care of
appeared 1.6e6.8 years (mean 3.7 years) after the initial the child and the radiologist [1,5]. In adults, the Bosniak
study. In four of the five children, the number of cysts classification system is a well-established grading system
increased to four. In one child with a single kidney following that guides management of renal cysts based on imaging
resection of multicystic dysplastic kidney, follow-up US characteristics on CT scan [6,8].
demonstrated numerous (n Z 20) cysts in the kidney that However, there are several differences between chil-
were compatible with polycystic renal disease. dren and adults in the natural history of renal cysts. The
main risk in adults is cystic renal cell carcinoma (RCC)
Definitive diagnosis of the renal cysts [6,8,12e14]. Yet, malignant transformations of renal cysts
A definitive diagnosis was obtained in 18 of the 212 children into renal cell carcinoma, Wilms tumor, or multilocular
(8.5%, Table 2). Calyceal diverticulum was diagnosed in cystic renal carcinoma are very rare in children. They have
nine children by delayed intravenous (IV) contrast abdom- been reported in a few case reports and in a small series of
inal CT (n Z 7), retrograde nephrostogram (n Z 1), and complex renal cysts [9,10,14e17].
technetium 99m MAG3 scan (n Z 1). Prior reports of solitary renal cysts in children have been
Nine children had surgical resection of the cyst. In- from selected groups of children with either simple or
dications for surgical procedures included: complex cyst complex renal cysts [14,16]. The present study is the first to
(n Z 5), flank pain (n Z 2), and increased size of the cyst evaluate all consecutive simple and complex solitary renal
(n Z 1). One child had end-stage renal disease and the right cysts in children.
lower pole artery was used for anastomosis of the trans- The CT-based Bosniak classification system for renal
planted kidney. The pathology diagnosis was benign renal cysts guides surgical treatment versus conservative man-
cysts in eight children; one had a cluster of cysts. One child agement. Because approximately 25% of grade IIF or in-
had multilocular cystic nephroma (MLCN). Overall, surgical termediate-grade cysts (multiple septa and/or minimal
intervention for renal cysts was performed in thirteen septal thickening) found on CT scan are malignant [18,19],
children (Table 2). these children require follow-up CT. However, US imaging

Table 2 Details of the children with a specific diagnosis of renal cysts (n Z 18).
Gender Age (years) Indicationa Diagnosis Cyst grade Procedure
(Radiologists 1 and 2)
Male 4.3 Complex cyst Benign cyst II, II Upper pole nephrectomy
Female 3.0 Flank pain Benign cyst II, II Fulguration
Female 8.7 Increased size Benign cyst II, II Fulguration
Female 11.8 Flank pain Epithelial cyst I, II Fulguration
Male 15.4 Complex cyst, HT Benign cyst II, II Partial nephrectomy
b
Male 2.3 Benign cyst I, I Nephrectomy
Male 7.4 Complex cyst Epithelial cyst with blood IV, III Radical nephrectomy
Female 7.2 Complex cyst A cluster of benign cysts III, III Upper pole nephrectomy
Male 7.2 Complex cyst MLCN III, III Radical nephrectomy
Male 12.8 Cyst CD I, I e
Male 4.7 UTI CD I, I e
Female 14.8 Cyst vs DK CD I, II e
Female 9.5 Stone, pain, UTI CD with stone I, II Fulguration
Male 2.1 Cyst vs DK CD I, II e
Female 4.7 Stone, UTI CD with stone I, II Excision of the cyst
Male 14.1 Stone, HT CD with stone I, I Fulguration
Male 1 day UTI CD I, I Fulguration
Female 8.6 Cyst vs DK CD I, I e
HT, hypertension; CD, calyceal diverticulum; MLCN, multilocular cystic nephroma; DK, hydronephrotic upper pole of a duplex kidney.
a
Indication for further imaging work-up or surgery of the cyst.
b
End-stage renal disease, right lower pole artery was used for anastomosis of the transplanted kidney.

Please cite this article in press as: Karmazyn B, et al., Ultrasound classification of solitary renal cysts in children, Journal of Pediatric
Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.03.001
+ MODEL
Solitary renal cysts in children 1.e5

helps to select the few children with complex renal cysts A definitive diagnosis of the renal cysts was obtained in
that require further evaluation with CT. Therefore, the 18/212 (8.5%) children; calyceal diverticulum was diag-
modified Bosniak classification system is for US, so that nosed in 50% (9/18), and benign renal cysts in 44% (8/18).
imaging characteristics of CT-grade IIF cysts are included in Only one child (6%, 1/18) had a tumor (MLCN).
grade III. Only four categories (I to IV) of cysts are used that The indications for intervention for renal cysts in chil-
define simple (grade I and II) and complex (grade III and IV) dren include complications of simple renal cysts such as
cysts. Based on the present study results, it is suggested bleeding, stone, superimposed infection, and/or pain [2].
that multiple septations be defined as >4 septations. In complex renal cysts, the indication for surgery is a risk of
It was found that the vast majority of renal cysts were tumor. In the present study, 12 children underwent surgical
simple and none of the children were found to have a renal intervention, five were due to complex renal cysts and risk
tumor. Very few children (3.8%, 8/212 and 3.3%, 7/212, by of tumor (one also had hypertension) and seven were due to
radiologists 1 and 2, respectively) had complex (grade III or IV) cyst stones (n Z 3), hypertension (n Z 1) and/or symptoms
renal cysts. Only one of these complex renal cysts was found (pain (n Z 3) and recurrent UTIs (n Z 3)). Another child had
to have a benign tumor (multilocular cystic nephroma, MLCN). the kidney removed at the time of renal transplant, but not
The Bosniak classification system has previously been because of symptoms.
reported in children in two highly selective groups of chil- Based on experience, a management algorithm based on
dren [14,16]. One study included 22 children who had sur- clinical presentation and grade of the cyst is suggested. The
gical resection of their cystic masses [16] and the other modified Bosniak classification system provides consistent
series included 39 children with complex renal cysts [14]. and well-defined criteria for simple (grade I and II) and
All children with malignant tumors had either Bosniak grade complex (grade II and IV) cysts. In asymptomatic simple renal
III or IV. In these two studies, the authors included the US cysts, no other imaging workup is required. In symptomatic
characteristics of grade IIF in the grade II category. This children, if surgical intervention is considered, a single-
resulted in the inclusion of MLCN tumors as grade II and the phase, delayed, post-contrast CT scan is suggested to
authors suggestion for follow-up of grade II cysts. differentiate between calyceal diverticulum (potential for
In the present study, inter-observer agreement of the either percutaneous or retrograde fulguration) and non-
modified Bosniak classification system was 83% communicating cysts. In complex cysts, a non-contrast CT of
(kappa Z 0.48). However, when the population was divided the kidneys followed by IV CT scan in the corticomedullary
into simple (grade I and II) and complex (grade III and IV) (arterial) and nephrographic phase (120 seconds) is sug-
cysts, there was better agreement (98% agreement, gested. In one of the present cases, a radical nephrectomy
kappa Z 0.65). In children, inter-observer variability has was performed in a child with hemorrhagic simple renal
been evaluated in one other study [17]. The authors found cysts. The CT scan was performed as a single, post-contrast
higher inter-observer agreement on both CT and US phase and the high-density cyst appeared as a solid tumor.
(kappa Z 0.86 and 0.83, respectively). However, this study The addition of a non-contrast phase may have shown non-
included a highly selective small series of children with enhancement of the mass and suggested a high-density cyst.
approximately 80% grade III or IV renal cysts [16]. In adults, Using this algorithm and US cyst grade, approximately 96% of
inter-observer agreement varied between studies [20,21]. the present children would not have needed additional im-
The management of renal cysts in children depends on aging. The other children would have needed a dedicated CT
the clinical presentation and imaging. The clinician may scan for further characterization of the mass.
decide to perform follow-up US, other imaging, or an inter- The present study had some limitations that were
vention. In the present study, 89 children had follow-up US inherent to the retrospective nature of the study. Only 42%
for more than a year later, 73 children had an abdominal CT, children had follow-up for more than a year. Additionally,
and 14 had an MRI. Surgery was performed in 13 children. only one child was found to have a tumor, which limited the
A study on 41 children that were followed for at least evaluation of the grading of renal cysts to predict renal
one year found that 49% (20/41) of the cysts grew in size tumors. However, this reflects the overall low risk of cystic
[4]. In the present study, the cysts increased in size in 18 of renal tumors in children.
the 89 children (20%). Using the ratio of cyst to renal
length, there was an increase in relative size in 17% (15/89)
of the children. Conclusions
Very few (three and one, by radiologists one and two,
respectively) cysts progressed from simple to complex The risk of a renal tumor in children with solitary renal cysts
cysts; none contained tumors. is extremely low. No children with grade I and II cysts had a
The risk for development of polycystic kidney disease renal tumor. The use of a modified Bosniak classification
(PCKD) is another concern in children with solitary renal system designed for US can save unnecessary imaging work-
cysts. During the follow-up period of the present study, five up and lead to more consistent classification and manage-
out of 89 (6%) children had an increase in the number of ment of renal cysts. Prospective studies based on this
renal cysts; four children had up to four cysts and only one classification would be necessary to confirm these results.
child with prior resection of multicystic kidney disease and
numerous (n Z 20) cysts was diagnosed with a polycystic
renal disease. However, to evaluate the long-term risk for Conflict of interest
PCKD, follow-up is required until the fifth decade of life,
which is when most PCKDs are discovered; this is beyond None of the authors of this manuscript has a conflict of
the scope of the present study. interest related to this manuscript.

Please cite this article in press as: Karmazyn B, et al., Ultrasound classification of solitary renal cysts in children, Journal of Pediatric
Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.03.001
+ MODEL
1.e6 B. Karmazyn et al.

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755e8.
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Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.03.001

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