Skip to main content
Top
Published in: European Radiology 9/2019

01-09-2019 | Magnetic Resonance Imaging | Urogenital

Multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney: CT and MRI findings and clinical characteristic

Authors: Kotaro Yoshida, Naoki Takahashi, Bernard F. King, Akira Kawashima, Peter C. Harris, Lynn D. Cornell, Emilie Cornec-Le Gall, Dai Inoue, Ichiro Mizushima, Mitsuhiro Kawano, Eric Thervet, Paul René de Cotret, Vicente E. Torres

Published in: European Radiology | Issue 9/2019

Login to get access

Abstract

Purpose

The aim of this study was to clarify the radiologic and clinical characteristics of multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney.

Method

Fourteen patients with unique and characteristic multiple hemorrhagic subcapsular cortical cysts of the kidney, not categorized in any existing renal cystic diseases, were retrospectively reviewed. The clinical information including age, sex, symptom, family history of renal or renal cystic disease, and laboratory data were collected. CT and MRI findings including distribution, number and size of cysts, and CT attenuation and signal intensity on T1- and T2-weighted MRI of cysts were analyzed.

Results

All patients except one were young and none had a family history of renal or renal cystic disease. Common clinical symptoms were flank or abdominal pain and hematuria. In all cases, only the left kidney was involved at initial presentation. Cysts were small (median cyst size, 4–15 mm), numerous, and distributed mainly along the subcapsular cortex of the kidney. Cysts were hyper-attenuated on unenhanced CT, extremely hypointense on T2-weighted MRI, and mildly hyperintense on T1-weighted MRI. All patients except one had normal renal function. Imaging follow-up revealed stable or mildly progressive disease in seven patients. Two patients developed several hemorrhagic subcapsular cortical cysts in the right kidney at follow-up. Three of five patients with a renal pathology specimen showed concurrent IgA nephropathy.

Conclusion

We have identified a unique renal cystic disease with multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney that has a characteristic manifestation both radiologically and clinically.

Key Points

• Multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney is a unique non-familial renal cystic disease with a characteristic manifestation both radiologically and clinically.
• Most cases of multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney are stable or slowly progressive, and do not require invasive intervention.
Literature
1.
go back to reference Grantham JJ, Torres VE (2016) The importance of total kidney volume in evaluating progression of polycystic kidney disease. Nat Rev Nephrol 12:667–677CrossRefPubMedPubMedCentral Grantham JJ, Torres VE (2016) The importance of total kidney volume in evaluating progression of polycystic kidney disease. Nat Rev Nephrol 12:667–677CrossRefPubMedPubMedCentral
2.
go back to reference Leonhard WN, Happe H, Peters DJ (2016) Variable cyst development in autosomal dominant polycystic kidney disease: the biologic context. J Am Soc Nephrol 27:3530–3538CrossRefPubMedPubMedCentral Leonhard WN, Happe H, Peters DJ (2016) Variable cyst development in autosomal dominant polycystic kidney disease: the biologic context. J Am Soc Nephrol 27:3530–3538CrossRefPubMedPubMedCentral
3.
go back to reference Ong AC, Devuyst O, Knebelmann B, Walz G, ERA-EDTA Working Group for Inherited Kidney Diseases (2015) Autosomal dominant polycystic kidney disease: the changing face of clinical management. Lancet 385:1993–2002 Ong AC, Devuyst O, Knebelmann B, Walz G, ERA-EDTA Working Group for Inherited Kidney Diseases (2015) Autosomal dominant polycystic kidney disease: the changing face of clinical management. Lancet 385:1993–2002
4.
go back to reference Hoyer PF (2015) Clinical manifestations of autosomal recessive polycystic kidney disease. Curr Opin Pediatr 27:186–192CrossRefPubMed Hoyer PF (2015) Clinical manifestations of autosomal recessive polycystic kidney disease. Curr Opin Pediatr 27:186–192CrossRefPubMed
5.
go back to reference Bergmann C (2015) ARPKD and early manifestations of ADPKD: the original polycystic kidney disease and phenocopies. Pediatr Nephrol 30:15–30CrossRefPubMed Bergmann C (2015) ARPKD and early manifestations of ADPKD: the original polycystic kidney disease and phenocopies. Pediatr Nephrol 30:15–30CrossRefPubMed
6.
go back to reference Bleyer AJ, Kidd K, Živná M, Kmoch S (2017) Autosomal dominant tubulointerstitial kidney disease. Adv Chronic Kidney Dis 24:86–93 Bleyer AJ, Kidd K, Živná M, Kmoch S (2017) Autosomal dominant tubulointerstitial kidney disease. Adv Chronic Kidney Dis 24:86–93
7.
go back to reference Eckardt KU, Alper SL, Antignac C et al (2015) Autosomal dominant tubulointerstitial kidney disease: diagnosis, classification, and management--a KDIGO consensus report. Kidney Int 88:676–683CrossRefPubMed Eckardt KU, Alper SL, Antignac C et al (2015) Autosomal dominant tubulointerstitial kidney disease: diagnosis, classification, and management--a KDIGO consensus report. Kidney Int 88:676–683CrossRefPubMed
8.
go back to reference Nguyen KA, Syed JS, Shuch B (2017) Hereditary kidney cancer syndromes and surgical management of the small renal mass. Urol Clin North Am 44:155–167CrossRefPubMed Nguyen KA, Syed JS, Shuch B (2017) Hereditary kidney cancer syndromes and surgical management of the small renal mass. Urol Clin North Am 44:155–167CrossRefPubMed
9.
go back to reference Adeniran AJ, Shuch B, Humphrey PA (2015) Hereditary renal cell carcinoma syndromes: clinical, pathologic, and genetic features. Am J Surg Pathol 39:e1–e18CrossRefPubMed Adeniran AJ, Shuch B, Humphrey PA (2015) Hereditary renal cell carcinoma syndromes: clinical, pathologic, and genetic features. Am J Surg Pathol 39:e1–e18CrossRefPubMed
11.
go back to reference Irazabal MV, Rangel LJ, Bergstralh EJ et al (2015) Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials. J Am Soc Nephrol 26:160–172CrossRefPubMed Irazabal MV, Rangel LJ, Bergstralh EJ et al (2015) Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials. J Am Soc Nephrol 26:160–172CrossRefPubMed
12.
go back to reference Kim B, King BF Jr, Vrtiska TJ, Irazabal MV, Torres VE, Harris PC (2016) Inherited renal cystic diseases. Abdom Radiol (NY) 41:1035–1051CrossRef Kim B, King BF Jr, Vrtiska TJ, Irazabal MV, Torres VE, Harris PC (2016) Inherited renal cystic diseases. Abdom Radiol (NY) 41:1035–1051CrossRef
13.
go back to reference Kim DJ, Kim MJ (2003) Localized cystic disease of the kidney: CT findings. Abdom Imaging 28:588–592CrossRefPubMed Kim DJ, Kim MJ (2003) Localized cystic disease of the kidney: CT findings. Abdom Imaging 28:588–592CrossRefPubMed
14.
go back to reference Slywotzky CM, Bosniak MA (2001) Localized cystic disease of the kidney. AJR Am J Roentgenol 176:843–849CrossRefPubMed Slywotzky CM, Bosniak MA (2001) Localized cystic disease of the kidney. AJR Am J Roentgenol 176:843–849CrossRefPubMed
15.
go back to reference Storey P, Thompson AA, Carqueville CL, Wood JC, de Freitas RA, Rigsby CK (2007) R2* imaging of transfusional iron burden at 3T and comparison with 1.5T. J Magn Reson Imaging 25:540–547CrossRefPubMedPubMedCentral Storey P, Thompson AA, Carqueville CL, Wood JC, de Freitas RA, Rigsby CK (2007) R2* imaging of transfusional iron burden at 3T and comparison with 1.5T. J Magn Reson Imaging 25:540–547CrossRefPubMedPubMedCentral
16.
go back to reference Schreuder MF (2011) Unilateral anomalies of kidney development: why is left not right? Kidney Int 80:740–745CrossRefPubMed Schreuder MF (2011) Unilateral anomalies of kidney development: why is left not right? Kidney Int 80:740–745CrossRefPubMed
17.
go back to reference Maeda Y, Fukui H, Tomida H, Kuyama T (2016) Unilateral renal cystic disease with an impaired renal function. Intern Med 55:2115–2116CrossRefPubMed Maeda Y, Fukui H, Tomida H, Kuyama T (2016) Unilateral renal cystic disease with an impaired renal function. Intern Med 55:2115–2116CrossRefPubMed
18.
go back to reference Lee JK, McClennan BL, Kissane JM (1978) Unilateral polycystic kidney disease. AJR Am J Roentgenol 130:1165–1167CrossRefPubMed Lee JK, McClennan BL, Kissane JM (1978) Unilateral polycystic kidney disease. AJR Am J Roentgenol 130:1165–1167CrossRefPubMed
19.
go back to reference Cho KJ, Thornbury JR, Bernstein J, Heidelberger KP, Walter JF (1979) Localized cystic disease of the kidney: angiographic-pathologic correlation. AJR Am J Roentgenol 132:891–895CrossRefPubMed Cho KJ, Thornbury JR, Bernstein J, Heidelberger KP, Walter JF (1979) Localized cystic disease of the kidney: angiographic-pathologic correlation. AJR Am J Roentgenol 132:891–895CrossRefPubMed
20.
go back to reference Levine E, Huntrakoon M (1989) Unilateral renal cystic disease: CT findings. J Comput Assist Tomogr 13:273–276CrossRefPubMed Levine E, Huntrakoon M (1989) Unilateral renal cystic disease: CT findings. J Comput Assist Tomogr 13:273–276CrossRefPubMed
22.
go back to reference Katabathina VS, Kota G, Dasyam AK, Shanbhogue AK, Prasad SR (2010) Adult renal cystic disease: a genetic, biological, and developmental primer. Radiographics 30:1509–1523CrossRefPubMed Katabathina VS, Kota G, Dasyam AK, Shanbhogue AK, Prasad SR (2010) Adult renal cystic disease: a genetic, biological, and developmental primer. Radiographics 30:1509–1523CrossRefPubMed
23.
go back to reference Jeon A, Cramer BC, Walsh E, Pushpanathan C (1999) A spectrum of segmental multicystic renal dysplasia. Pediatr Radiol 29:309–315CrossRefPubMed Jeon A, Cramer BC, Walsh E, Pushpanathan C (1999) A spectrum of segmental multicystic renal dysplasia. Pediatr Radiol 29:309–315CrossRefPubMed
24.
go back to reference Truong LD, Choi YJ, Shen SS, Ayala G, Amato R, Krishnan B (2003) Renal cystic neoplasms and renal neoplasms associated with cystic renal diseases: pathogenetic and molecular links. Adv Anat Pathol 10:135–159CrossRefPubMed Truong LD, Choi YJ, Shen SS, Ayala G, Amato R, Krishnan B (2003) Renal cystic neoplasms and renal neoplasms associated with cystic renal diseases: pathogenetic and molecular links. Adv Anat Pathol 10:135–159CrossRefPubMed
25.
go back to reference Watanabe T, Yamazaki A, Kurabayashi T, Hanaoka J (2005) Familial multicystic dysplastic kidney. Pediatr Nephrol 20:1200CrossRefPubMed Watanabe T, Yamazaki A, Kurabayashi T, Hanaoka J (2005) Familial multicystic dysplastic kidney. Pediatr Nephrol 20:1200CrossRefPubMed
26.
go back to reference Taxy JB, Filmer RB (1976) Glomerulocystic kidney. Report of a case. Arch Pathol Lab Med 100:186–188PubMed Taxy JB, Filmer RB (1976) Glomerulocystic kidney. Report of a case. Arch Pathol Lab Med 100:186–188PubMed
27.
go back to reference Bernstein J (1993) Glomerulocystic kidney disease--nosological considerations. Pediatr Nephrol 7:464–470CrossRefPubMed Bernstein J (1993) Glomerulocystic kidney disease--nosological considerations. Pediatr Nephrol 7:464–470CrossRefPubMed
28.
go back to reference Oliva MR, Hsing J, Rybicki FJ, Fennessy F, Mortelé KJ, Ros PR (2003) Glomerulocystic kidney disease: MRI findings. Abdom Imaging 28:889–892 Oliva MR, Hsing J, Rybicki FJ, Fennessy F, Mortelé KJ, Ros PR (2003) Glomerulocystic kidney disease: MRI findings. Abdom Imaging 28:889–892
29.
go back to reference Lennerz JK, Spence DC, Iskandar SS, Dehner LP, Liapis H (2010) Glomerulocystic kidney: one hundred-year perspective. Arch Pathol Lab Med 134:583–605PubMed Lennerz JK, Spence DC, Iskandar SS, Dehner LP, Liapis H (2010) Glomerulocystic kidney: one hundred-year perspective. Arch Pathol Lab Med 134:583–605PubMed
30.
go back to reference Senger C (2000) Pathologic quiz case: infant girl with unilateral nephromegaly. Arch Pathol Lab Med 124:327–329PubMed Senger C (2000) Pathologic quiz case: infant girl with unilateral nephromegaly. Arch Pathol Lab Med 124:327–329PubMed
Metadata
Title
Multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney: CT and MRI findings and clinical characteristic
Authors
Kotaro Yoshida
Naoki Takahashi
Bernard F. King
Akira Kawashima
Peter C. Harris
Lynn D. Cornell
Emilie Cornec-Le Gall
Dai Inoue
Ichiro Mizushima
Mitsuhiro Kawano
Eric Thervet
Paul René de Cotret
Vicente E. Torres
Publication date
01-09-2019
Publisher
Springer Berlin Heidelberg
Published in
European Radiology / Issue 9/2019
Print ISSN: 0938-7994
Electronic ISSN: 1432-1084
DOI
https://doi.org/10.1007/s00330-019-06057-3

Other articles of this Issue 9/2019

European Radiology 9/2019 Go to the issue