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| Year : 2010 | Volume
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| Issue : 6 | Page : 1122-1126 |
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| Localized renal cystic disease: Report of a rare case |
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Shilpi Singh Gupta1, Onkar Singh1, Sumit Shukla1, Sangeet Chowdhary2
1 Department of Surgery, MGM Medical College & MY Hospital, Indore, India
2 Department of Radiology, Wellspring Radio-diagnostic Centre, Indore, India
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| Date of Web Publication | 4-Nov-2010 |
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 Abstract | | |
We report a case of a 32-year-old female who presented with right flank pain. Ultrasonography done for hematuria 10 years ago indicated the presence of renal cysts in the right kidney, but she had been asymptomatic since then. This history attracted our attention to the possibility of "localized renal cystic disease" (LRCD). Diagnosis was confirmed by computerized tomogram, negative renal ultrasound of one of the parents and absence of family history of renal disease. She is still being followed up for the last 3 years and is doing well. LRCD has been given various names since it was first recognized as a distinct clinical entity but the term LRCD appears to be the most accurate. Here, we present a brief discussion of clinical significance, diagnosis and differentials of this rare condition.
How to cite this article:
Gupta SS, Singh O, Shukla S, Chowdhary S. Localized renal cystic disease: Report of a rare case. Saudi J Kidney Dis Transpl 2010;21:1122-6 |
How to cite this URL:
Gupta SS, Singh O, Shukla S, Chowdhary S. Localized renal cystic disease: Report of a rare case. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2020 Oct 26];21:1122-6. Available from: https://www.sjkdt.org/text.asp?2010/21/6/1122/72303 |
| Introduction | |  |
"Localized renal cystic disease" (LRCD) is an uncommon kidney disease of unknown etiopathogenesis, which involves only one kidney at a time and has recently earned clinical significance because of its completely non progressive nature. [1],[2],[3],[4],[5] The condition is typically nonfamilial, does not cause renal functional impairment and does not require specific management, thus warranting its differentiation from other cystic diseases, most frequently autosomal dominant polycystic kidney disease. Diagnosis of this condition is made by computerized tomography (CT) scan. Usual treatment offered in the past was nephrectomy. But the condition needs follow-up only and no surgical intervention. We report a case of LRCD along with a brief discussion. Diagnosis was confirmed by computerized tomogram, negative renal ultrasound of one of the parents and absence of family history of renal disease. The patient has been followed up for three years and is doing well.
| Case Report | | |
We report a case of a 32-year-old female who came to our hospital in April 2005, with dull ache in right flank. She had one episode of hematuria at 22 years of age, which warranted ultrasonography (US) that showed multiple cystic lesions in the right kidney. Patient remained absolutely well since then and did not get herself evaluated further. Her urine analysis did not show any abnormality and her renal function tests were within normal range. Her serum electrolyte levels and ECG were also normal. Repeat ultrasound now showed multiple cysts in mid and upper portions of the right kidney. Intravenous peylogram demonstrated normal uptake and enhancement of bilateral kidneys with collection of contrast at lower pole in the right kidney [Figure 1]. Abdominal computerized tomography (CT) scan revealed replacement of mid and upper portions of the right kidney by multiple round, variable sized, wellmarginated cysts, the largest being 3.8 cm in diameter, without distinct capsule formation, and normal left kidney. Enhancement with contrast revealed normally enhancing renal parenchyma at lower pole and in between the cysts [Figure 2] A and B. Also, normal excretion of contrast by both the kidneys was noted along with a non-obstructive small 0.4 ± 0.3 cm sized calculus in posterior calyx mid polar region. There were no hepatic or pancreatic cysts. Renal USG of her mother did not reveal any lesion. Her parental screening could not be done completely as her father had died three years back in a road traffic accident, but did not have any symptoms related to kidney disease. She and her mother denied any sort of complaint or disease related to kidneys in her father. Similarly, her five siblings had no renal symptoms, and ultrasound in both her brothers and one of the sisters was normal. On the basis of non-progressive clinical course of long duration, imaging features, negative screening of family members and absence of family history of renal disease, the diagnosis of LRCD was made. No further evaluation or treatment was done; only periodic follow-up was planned. Findings in CT scans done at different intervals during a follow-up of three years were the same as in CT scans conducted in April 2005. | Figure 1 :Intravenous peylogram of a 32-year-old female, showing normal uptake and enhancement of bilateral kidneys with collection of contrast at lower pole in right kidney.
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 | Figure 2 :Abdominal CT scan of a 32-year-old female done in April 2005, revealing mid and upper portions of the right kidney replaced by multiple and variable sized cysts without distinct capsule formation, and unaffected left kidney.
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Last CT was done in July 2008 [Figure 3]A and B and the patient is still being followed clinically and radiographically. | Figure 3 :Abdominal CT scan of same female done in July 2008, showing same findings as in Figure 2.
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| Discussion | | |
LRCD is one of the renal cystic diseases which is uncommon and poorly understood, as only few cases have been documented in the literature, describing this condition. [1],[2],[3],[4],[5] Initially considered as a form of autosomal dominant polycystic kidney disease (ADPKD) with unilateral presentation, LRCD has now been accepted as a distinct disease entity. [2],[3],[4],[6],[7],[8],[9],[10],[11],[12],[13],[14] LRCD is typically defined as "unilateral, non-familial, and non-progressive multicystic disease involving kidney, characterized by the replacement of part or most of one kidney by multiple cysts of varying sizes, scattered diffusely throughout the renal parenchyma or localized to one or more portions of one kidney, without formation of a distinct encapsulated renal mass". [1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14] Replacement of kidney tissue by cysts can occur in two ways: either diffuse scattering of cysts throughout renal parenchyma [1],[2],[4],[6],[8],[11],[13] or a more localized or segmental involvement of one region of the involved kidney. [1],[2],[6],[7],[9],[10],[12] Opposite kidney is not involved and other organs such as liver and pancreas are free from cysts. [6]
The pathogenesis of LRCD is not known but it may possibly be an acquired condition. [1],[15] As inferred from cases described in the literature, men have been involved more often and majority are affected in sixth and seventh decades of life. Clinical presentation of LRCD is characterized by multiple cysts in only one kidney without the involvement of other intraabdominal organs. [6] Renal functions are preserved. [16] Slywotzky and Bosniak [1] reported incidentally found LRCD to be the most common way of presentation. Symptoms, if present, usually include flank pain, flank mass, hematuria and hypertension. [6] James et al [17] described the only case of LRCD in literature, associated with secondary polycythemia, which resolved after nephrectomy. Histologically, the cysts are dilated ducts and tubules, lined by epithelial cells, and varying in size from millimeters to several centimeters. [14] In between these cysts are patches of non-cystic normal or atrophic renal parenchyma. [1],[4]
US shows multiple cysts that are not enclosed in a capsule but separated by thick septae in whole of or a portion of one kidney. [4],[9] CT gives similar findings along with clear visualization of renal tissue in between the cysts as enhancing bands, if contrast is given. [2],[4],[9],[10],[11] Also, CT is more sensitive for detecting small cysts than USG. [6] Magnetic resonance imaging (MRI) also reveals similar diagnostic features of LRCD as those revealed in CT. [1] CT and MRI can confirm the diagnosis of LRCD if specific imaging findings are seen, along with negative family history for renal cystic disease and absence of clinical manifestations of deterioration of renal functions. Surgical confirmation or renal biopsy is no longer required if this combination (typical radiological findings, and negative family history/ absence of deterioration of renal functions) is appreciated. [6],[12],[18]
It is clinically important to differentiate LRCD from ADPKD, the most common renal cystic disease, i.e. multicystic dysplastic kidney (MCDK), multilocular cystic renal neoplasms, and simple cysts, especially because of the genetic and prognostic reasons. [3],[5] LRCD is indistinguishable from ADPKD in morphological and histological aspects except for unilateral localization and non familiarity associated with the former. [4],[5],[6],[7] Other important difference is progressive deterioration in renal function seen with ADPKD but not with LRCD. [5],[16] Absence of cysts in other organs such as liver and pancreas also supports the diagnosis of LRCD. [6] Asymmetrically evolving ADPKD needs to be excluded, especially in children and younger patients, either by screening of parents with renal ultrasonogram, along with presence or absence of family history of renal cystic disease, or by a long duration follow-up of the patient. [1],[3],[5],[19] Differentiation of MCDK from LRCD is easy with the help of US, contrast enhanced CT scan and radionuclide studies because of diffusely cystic, severely dysplastic phenotype and non-functioning state of kidney associated with the former. [5] But sometimes when MCDK is segmental, differentiation from LRCD becomes difficult. [14] However, the number of cysts is less in MCKD and it is usually associated with ureteral atresia or agenesis. [5],[16] Simple renal cysts may involve single kidney, and when multiple, lead to a diagnostic challenge. Notably, these are mostly scattered throughout the kidney and rarely localized. [2]
Apart from the above mentioned renal cystic diseases, other differentials include multilocular cystic renal neoplasms such as multilocular cystic nephroma and cystic renal cell carcinoma. Multilocular cystic renal tumors are frequently seen as discrete, encapsulated lesions which are very well demarcated from the adjacent renal parenchyma. [14] Therefore, normal renal parenchymal tissue intervening in between the cysts is not seen like in LRCD. [2],[5] Moreover, whatever the type of involvement is, diffuse or segmental, distinct capsule formation is never seen in LRCD. [1],[6]
In conclusion, we discuss the occurrence of LRCD in a female and its differential in detail. LRCD usually does not require any specific management unless it causes hypertension or polycythemia.
| Acknowledgements | | |
We are thankful to Prof. Dr. R.K. Mathur, Head of the Department, and Prof. Dr. D.K. Jain, Professor of Surgery, MGM Medical College & MY Hospital, Indore, India, for their incessant encouragement and moral support which has always inspired us during the pro cess of review of literature.
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Correspondence Address:
Shilpi Singh Gupta
VPO- Sangowal, Tehsil- Nakodar, District- Jalandhar, Punjab, 144041
India
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PMID: 21060185 
[Figure 1], [Figure 2], [Figure 3] |
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