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Multicystic Dysplastic Kidney: Imaging

Author: John S Wiener, MD, FACS, FAAP, Clinical Assistant Professor, Division of Urology, University of North Carolina at Chapel Hill; Associate Professor of Surgery and Associate Residency Program Director, Division of Urologic Surgery, Associate Professor of Pediatrics, Duke University School of Medicine
Coauthor(s): Michaella E Maloney, Duke University School of Medicine; Ana Maria Gaca, MD, Assistant Professor, Division of Pediatric Radiology, Duke University Medical Center
Contributor Information and Disclosures

Updated: Feb 19, 2008

Radiography

Findings

Incidental findings on kidney, ureter, and bladder (KUB) images include displacement of the bowels when the affected kidney is enlarged (see Image 1). Also, ringlike calcifications of the cyst walls may be seen on plain images.

Retrograde pyelography may demonstrate an atretic or absent ureter.

Computed Tomography

Findings

MCDK can be an incidental finding, but CT studies are not part of the diagnostic investigation. They show the typical multicystic appearance of MCDK with little or no parenchyma (see Images 8-9). Cyst wall calcification can be seen.

If a contrast-enhanced CT is performed, there is no excretion seen.

Magnetic Resonance Imaging

Findings

MCDK can be an incidental finding but these MRIs are not part of the diagnostic investigation. MRIs show the typical multicystic appearance of MCDK with little or no parenchyma (see Images 8-9).

Ultrasonography

Findings

Prenatal imaging (see Images 2-3) findings include hypoechoic cysts of variable sizes and shapes, interfaces between cysts, a nonmedial location of large cysts, the absence of an identifiable renal sinus, a lack of communication between cysts on sonograms, and minimal surrounding parenchyma. Only 20% of MCDKs have an identifiable reniform shape (compared with 90% of hydronephrotic kidneys). Bilateral MCDK may occur with oligohydramnios as a result of poor urine production. The cysts of MCDK may become enlarged, may shrink, or may involute during fetal life.

Postnatal sonograms are shown in Images 4-5. The diagnostic criteria for postnatal sonography are the same as those for prenatal sonography.

Degree of Confidence

Ultrasonography is an excellent diagnostic test for MCDK, with a high degree of confidence. An obstructive uropathy with little renal parenchyma can mimic MCDK, but radionuclide studies can provide confirmation of the diagnosis. Autosomal recessive PCKD is not usually mistaken for MCDK, as the cysts in PCKD are too small to be visualized on sonograms, and the parenchyma is generally homogeneously hyperechoic. Other cystic diseases typically appear with some functional parenchyma.

False Positives/Negatives

The greatest source of false-positive errors occurs in the setting of hydronephrosis or UPJO. A reniform shape and/or a large cystic structure in the medial portion of the kidney are more indicative of hydronephrosis than MCDK. Unlike in hydronephrosis, where there is communication with the central renal pelvis, the cysts of the classic form of MCDK do not communicate.

Regarding false-negative results, renal agenesis with non-renal cystic structures in the retroperitoneum (eg, an adrenal cystic mass) could potentially be mistaken for MCDK.

Nuclear Imaging

Findings

Nuclear renograms are used to evaluate the perfusion, function, and drainage of the kidneys, as there is uptake of the radiotracer into a functioning kidney and excretion into the renal pelvis, ureter, and bladder. In the area of the MCDK, a photopenic region is present that represents displaced tissue with background activity only (see Image 7).

Degree of Confidence

Nuclear medicine studies are the best imaging studies for differentiating between MCDK and hydronephrosis; however, severe hydronephrosis with poor renal function may still be difficult to distinguish from MCDK. The similarity may not be clinically relevant because reconstructive surgery is usually not indicated in the setting of poor (ie, <10-15%) differential function.

MAG-3, diethylenetriamine-pentaacetic acid (DTPA), and dimercaptosuccinic acid (DMSA) are the preferred agents. Tests with these agents are sensitive for detecting renal function, but the anatomic resolution is relatively poor. Other forms of imaging are required to visualize the specific anatomy of the kidney.

False Positives/Negatives

On a nuclear renogram, anything that impairs the renal circulation, such as a renal artery stenosis or a renal vein thrombosis, appears as a nonfunctioning kidney; nevertheless, the renal parenchyma should appear relatively normal with ultrasonography. In addition, renal agenesis may mimic the nonfunctioning kidney of MCDK, but the tissue is absent on sonograms.

False-negative results may arise in the setting of severe hydronephrosis. In a poorly functioning kidney, the intervention is the same as in MCDK; therefore, discriminating between these 2 disorders may not be imperative.

Angiography

Findings

Angiography is not indicated for the evaluation of MCDK; however, if catheterization, CT scanning, or MRA is performed, the ipsilateral renal artery is atretic or absent.

More on Multicystic Dysplastic Kidney

Overview: Multicystic Dysplastic Kidney
Imaging: Multicystic Dysplastic Kidney
Follow-up: Multicystic Dysplastic Kidney
Multimedia: Multicystic Dysplastic Kidney
References

References

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  3. Spence HM. Congenital unilateral multicystic kidney: an entity to be distinguished from polycystic kidney disease and other cystic disorders. J Urol. Dec 1955;74(6):693-706. [Medline].

  4. Beck AD. The effect of intra-uterine urinary obstruction upon the development of the fetal kidney. J Urol. Jun 1971;105(6):784-9. [Medline].

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  6. Chang LW, Chang FM, Chang CH, Yu CH, Cheng YC, Chen HY. Prenatal diagnosis of fetal multicystic dysplastic kidney with 2-dimensional and 3-dimensional ultrasound. Ultrasound Med Biol. Jul 2002;28(7):853-8. [Medline].

  7. Cambio AJ, Evans CP, Kurzrock EA. Non-surgical management of multicystic dysplastic kidney. BJU Int. Jan 8 2008;[Medline].

  8. Weinstein A, Goodman TR, Iragorri S. Simple multicystic dysplastic kidney disease: end points for subspecialty follow-up. Pediatr Nephrol. Jan 2008;23(1):111-6. [Medline].

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  10. Belk RA, Thomas DF, Mueller RF, Godbole P, Markham AF, Weston MJ. A family study and the natural history of prenatally detected unilateral multicystic dysplastic kidney. J Urol. Feb 2002;167(2 Pt 1):666-9. [Medline].

  11. Minevich E, Wacksman J, Phipps L, Lewis AG, Sheldon CA. The importance of accurate diagnosis and early close followup in patients with suspected multicystic dysplastic kidney. J Urol. Sep 1997;158(3 Pt 2):1301-4. [Medline].

  12. Wiener JS. Multicystic dysplastic kidney. In: Belman AB, King LR, Kramer SA, eds. Clinical Pediatric Urology. 4th ed. London: Taylor & Francis; 2002:633-45.

Further Reading

Keywords

classic multicystic dysplastic kidney, classic MCDK, hydronephrotic multicystic dysplastic kidney, hydronephrotic MCDK, multicystic dysplasia of the kidney, MCDK, multicystic kidney, multicystic renal dysplasia, solid cystic dysplasia, renal dysplasia

Contributor Information and Disclosures

Author

John S Wiener, MD, FACS, FAAP, Clinical Assistant Professor, Division of Urology, University of North Carolina at Chapel Hill; Associate Professor of Surgery and Associate Residency Program Director, Division of Urologic Surgery, Associate Professor of Pediatrics, Duke University School of Medicine
John S Wiener, MD, FACS, FAAP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Urological Association, Society for Fetal Urology, Society for Pediatric Urology, and Society of University Urologists
Disclosure: Nothing to disclose.

Coauthor(s)

Michaella E Maloney, Duke University School of Medicine
Disclosure: Nothing to disclose.

Ana Maria Gaca, MD, Assistant Professor, Division of Pediatric Radiology, Duke University Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Lori Lee Barr, MD, FACR, FAIUM, Clinical Associate Professor of Radiology, University of Texas Health Science Center in San Antonio; Clinical Assistant Professor of Radiology, University of Texas Medical Branch at Galveston; Member, Board of Directors, Austin Radiological Association; Consulting Staff, Seton Health Network, Columbia/St David's Healthcare System, Healthsouth Rehabilitation Hospital of Austin, Georgetown Hospital, St Mark's Medical Center, Cedar Park Regional Medical Center
Lori Lee Barr, MD, FACR, FAIUM is a member of the following medical societies: American Association for Women Radiologists, American College of Radiology, American Institute of Ultrasound in Medicine, American Roentgen Ray Society, American Society of Pediatric Neuroradiology, Association of University Radiologists, Radiological Society of North America, Society for Pediatric Radiology, Society of Radiologists in Ultrasound, Southern Medical Association, Texas Radiological Society, and Undersea and Hyperbaric Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Kieran McHugh, MBBCh, Honorary Lecturer, The Institute of Child Health; Consultant Pediatric Radiologist, Department of Radiology, Great Ormond Street Hospital for Children, London, UK
Kieran McHugh, MBBCh is a member of the following medical societies: American Roentgen Ray Society and Royal College of Radiologists
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Eugene C Lin, MD, Consulting Radiologist, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine
Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, and Society of Nuclear Medicine
Disclosure: Nothing to disclose.

 
 
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