Multicystic Renal Dysplasia Workup

  • Author: Agnieszka Swiatecka-Urban, MD, FASN; Chief Editor: Craig B Langman, MD   more...
 
Updated: Nov 9, 2011
 

Laboratory Studies

  • A urine for dipstick and microscopic analysis should be obtained in patients with suspected multicystic dysplastic kidney (MCDK). Urine should be sent for culture as needed.
  • Blood tests for creatinine, urea, and electrolytes should be performed.
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Imaging Studies

  • Renal ultrasonography
    • Renal ultrasonography is the recommended preliminary diagnostic imaging study.
    • Ultrasonography reveals a kidney that contains multiple cysts of variable size that are randomly arranged and are separated by little or no echogenic parenchyma.
    • No renal pelvis is identifiable.
  • Voiding cystourethrography (VCUG)
    • VCUG should be performed to look for vesicoureteral reflux (VUR). Current recommendations for patients with unilateral multicystic dysplastic kidney include evaluation of the contralateral kidney for VUR by VCUG.
    • Although, the necessity of VCUG, an invasive technique, has been increasingly questioned by many authors because of the following observations:
      • VUR has been reported in 4-19% of contralateral kidneys; it is usually low grade and resolves in early life.[19, 12, 13]
      • Repeat ultrasonography examinations of the contralateral kidney are able to detect significant number of renal anomalies.[19]
      • No difference in the incidence of urinary tract infections (UTIs) or renal scaring was observed between children with or without VUR in the contralateral kidney.[12]
    • Routine VCUG may not be necessary for evaluation of unilateral multicystic dysplastic kidney. Two successive normal neonatal renal ultrasonography scans in infants with antenatally diagnosed multicystic dysplastic kidney clinically rules out significant contralateral anomalies, thereby rendering neonatal VCUG unnecessary. Furthermore, VCUG may be deferred, unless abnormal ultrasonography findings in the contralateral kidney or ureter and/or a history of UTI are noted.
  • Dimercaptosuccinic acid (DMSA) renal scanning
    • DMSA renal scanning may be necessary if ultrasonography does not reveal the classic features of multicystic dysplasia of the kidney.
    • DMSA renal scanning demonstrates absence of function in the kidney with multicystic dysplastic kidney.
  • Technetium Tc 99m diethylenetriamine pentaacetic acid (DTPA) or technetium Tc 99m mercaptoacetyltriglycine (MAG-3) renal scanning
    • When ultrasonography reveals a pelvocaliceal system or a dominant central cyst, severe UPJO and renal dysplasia secondary to obstruction may be difficult to differentiate. In these cases, technetium Tc 99m DTPA or technetium Tc 99m MAG-3 renal scanning with furosemide washout is important.
    • In cases of severe hydronephrosis, a peripheral rim of activity might be observed, followed by an increase in excretion noted on delayed images.
    • Delayed images also reveal retention of the DTPA or MAG-3 in the pelvocaliceal system with little or no washout with furosemide.
    • With renal dysplasia secondary to obstruction, degrees of decreased function vary.
  • Intravenous pyelography (IVP): This is not usually necessary; however, if multicystic dysplastic kidney involves only the upper or lower segment of a duplicated system, an IVP might reveal a small but functional ipsilateral kidney with an incomplete and possibly dilated calyceal system.
  • Cystoscopy
    • Cystoscopy is usually unnecessary.
    • If performed, the ipsilateral ureteral orifice and hemitrigone might be absent.
    • When the ureteral orifice is present, retrograde ureterography usually demonstrates a ureter that terminates blindly below the ureteropelvic junction.
  • Cyst puncture: In the rare situation in which severe UPJO cannot be differentiated from multicystic dysplastic kidney by ultrasonography and DTPA renal scanning, a diagnostic puncture of a cyst with instillation of radiographic contrast material might help distinguish these 2 disorders. The presence of cysts connected by tubular structures and the absence of a collecting system is diagnostic of multicystic dysplastic kidney.
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Other Tests

  • An annual assessment of blood pressure should be performed.
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Histologic Findings

  • The kidney with multicystic dysplastic kidney is enlarged, abnormally shaped, and often resembles a bunch of grapes. The numerous and irregularly sized cysts range from less than 1 mm to several centimeters in diameter.
  • In a study of 48 patients, fewer than 5 cysts were noted in 34% of patients, and 5 or more cysts were noted in 66% of patients.[20]
  • The cysts contain a clear or yellow fluid and are connected by a fibrous tissue stroma. The usual reniform configuration of the kidney is obscured by the cysts and by a lack of pelvocaliceal differentiation. Rudimentary lobes are sometimes grossly identifiable. Nodular masses are present in a medial and central location in some kidneys and might represent pelvic atresia.
  • Multicystic dysplastic kidney might be present in only the upper or lower pole of a duplicated collecting system.
  • Ureteral or ureteropelvic atresia is always present.
  • The atretic portion of the ureter varies in length from 1-5 cm.
  • The ipsilateral renal artery is absent or hypoplastic.
  • Primitive epithelial ducts and nests of metaplastic cartilage are the principal microscopic criteria for a diagnosis of multicystic dysplastic kidney.
  • Histopathologic examination reveals abnormal ductal differentiation and only rudimentary corticomedullary differentiation. Thick-walled or thin-walled cysts with smooth inner linings are present throughout the kidney. Normal renal parenchyma is usually absent. The loose connective tissue that surrounds the cysts might range from thin strands to extensive areas of fibrosis.
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Contributor Information and Disclosures
Author

Agnieszka Swiatecka-Urban, MD, FASN  Assistant Professor, Department of Pediatrics, Cell Biology and Physiology, University of Pittsburgh School of Medicine; Assistant Professor, Department of Nephrology, Children's Hospital of Pittsburgh

Agnieszka Swiatecka-Urban, MD, FASN is a member of the following medical societies: American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, and Women in Nephrology

Disclosure: Nothing to disclose.

Specialty Editor Board

Laurence Finberg, MD  Clinical Professor, Department of Pediatrics, University of California, San Francisco, School of Medicine and Stanford University School of Medicine

Laurence Finberg, MD is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Frederick J Kaskel, MD, PhD  Director of the Division and Training Program in Pediatric Nephrology, Vice Chair, Department of Pediatrics, Montefiore Medical Center and Albert Einstein School of Medicine

Frederick J Kaskel, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Pediatric Society, American Physiological Society, American Society of Nephrology, American Society of Pediatric Nephrology, American Society of Transplantation, Eastern Society for Pediatric Research, Federation of American Societies for Experimental Biology, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Renal Physicians Association, Sigma Xi, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Howard Trachtman, MD  Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine

Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD  The Isaac A Abt, MD, Professor of Kidney Diseases, Northwestern University, The Feinberg School of Medicine; Division Head of Kidney Diseases, Children's Memorial Hospital

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology

Disclosure: NIH Grant/research funds None; Raptor Pharmaceuticals, Inc Grant/research funds None; Alexion Pharmaceuticals, Inc. Grant/research funds None

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author William Lane M Robson, MA, MD, FRCP, FRCP(Glasg), to the original writing and development of this article.

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Kidney, ureter, and bladder (KUB) images of an infant with a right multicystic dysplastic kidney demonstrate displacement of bowel loops away from the right abdomen.
 
 
 
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