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Year : 2011  |  Volume : 54  |  Issue : 2  |  Page : 437-438
Unilateral multicystic dysplasia in an ectopic kidney


Department of Pathology, MGM Medical College and Hospital, Kamothe, Navi Mumbai, India

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Date of Web Publication27-May-2011
 

How to cite this article:
Sahu SS, Maheshwari UM, Borkar D B, Sachdeva HS. Unilateral multicystic dysplasia in an ectopic kidney. Indian J Pathol Microbiol 2011;54:437-8

How to cite this URL:
Sahu SS, Maheshwari UM, Borkar D B, Sachdeva HS. Unilateral multicystic dysplasia in an ectopic kidney. Indian J Pathol Microbiol [serial online] 2011 [cited 2019 Dec 12];54:437-8. Available from: http://www.ijpmonline.org/text.asp?2011/54/2/437/81629


Sir,

Renal dysplasia has increasingly become recognized as an important clinical entity. Unilateral renal dysplasia in the most common abdominal mass in the newborn infant and is the most common cystic disorder of the kidney in children. [1] Dysplasia of the kidney is a form of renal cystic disease characterized by disturbed differentiation of nephrogenic tissue with persistence of structures inappropriate to gestational age of the patient. [2] The process may involve one or both kidneys; it may involve the entire kidney (total), only a part of it (segmental), or there may be scattered foci of dysplasia throughout the kidney (focal). Renal dysplasia has been differentiated from familial forms of polycystic disease of the kidney and has been designated as a Type II polycystic kidney. The prognosis differs in the various types of cystic disease of the kidney, and the individual clinical course varies among the types of renal dysplasia. [3]

We present a case of a 1-year-old male child who presented initially with acute abdomen. He presented with no other symptoms. Computed tomography (CT) scan finding revealed a multicystic kidney with mild partial hydroureter present ectopically in the left pelvic region [Figure 1]. The right kidney pelvicalyceal system and ureter were normal. We received the nephrectomy specimen of the left ectopic kidney along with ureter [Figure 2]. Kidney measured 2.5 × 2 × 1 cm and was whitish in color. Cut section showed multiple cysts in the periphery with solid areas toward medulla.
Figure 1: CT scan of patient showing a cystic mass in left pelvis

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Figure 2: Gross specimen of ectopic kidney with dilated ureter. Externally bosselated due to multiple cysts

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Microsections from the kidney revealed peripherally placed large cystic structures lined by flattened epithelium. Lobules of renal tissue comprised proximal convoluted tubules, distal convoluted tubules, immature glomeruli and bits of fibrocollagenous tissue. There are primitive ducts with fibromuscular cuffing inside the lobules [Figure 3]. Intrinsically enmeshed within this is mesenchyme including cartilage [Figure 4]. A diagnosis of multicystic renal dysplasia was made on histopathology.
Figure 3: Photomicrograph showing primitive tubules surrounded by a collar of fibromuscular stroma (H and E, ×400)

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Figure 4: Photomicrograph showing atrophied glomeruli and an island of cartilage (H and E, ×400)

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Recent follow-up revealed the patient surviving well on the normally functioning right kidney.

Multicystic dysplastic kidney (MCDK) is a congenital renal dysplasia with variable number of cysts. Non-communicating cysts and the non-patent proximal ureter replace the renal parenchyma. The incidence of contralateral genitourinary tract anomalies for patients with MCDK disease ranged from 20 to 80% in previous studies. Vesicoureteral reflux (VUR) is the most common contralateral genitourinary tract anomaly, while ureteropelvic junction (UPJ) obstruction is the second one. Since the MCDK is usually dysfunctional, the preserved function of the contralateral kidney thus becomes very important.

Previous report has revealed that MCDK lesions involute with time. Also, compensatory contralateral renal growth occurs before birth in MCDK patients. Previous studies indicated that the affected kidney shrinks in the first two years of life. In most cases, the natural history of the contralateral renal function is usually normal as a consequence of progressive compensatory hypertrophy. However, some exceptional complications of MCDK, such as hypertension, renal insufficiency, and malignant degeneration, have been found. Though in our case contralateral kidney is normally functioning, it is important to follow-up on the remaining renal function of the normal kidney on a regular basis. [2]


   Acknowledgment Top


Dr. A. K. Singhal, Lecturer, Department of Urosurgery, MGM Medical College, Navi Mumbai, is acknowledged.

 
   References Top

1.Longino LA, and Martin LW. Abdominal masses in newborn infant. Pediatrics 1958;21:596-604.  Back to cited text no. 1
    
2.Chan-Yao WU, Chiou YH, Sung PK. Left Multicystic dysplastic kidney with contralateral ectopic kidney and ureterovesicle junction obstruction. Chinese Med J (Taipei) 2001;64:129-32.  Back to cited text no. 2
    
3.Laufer I, Griscom NT. Compensatory renal hypertrophy: Absence in utero and development in early life. Am J Roentgenol Radium Ther Nucl Med 1971;113:464-7.  Back to cited text no. 3
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Correspondence Address:
Shilpi S Sahu
1201-A, Tiara Residential Complex, Sector-13, Kharghar, Navi Mumbai-410 210
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.81629

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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