History

Most cases of multicystic dysplasia of the kidney (MCDK) are detected during fetal ultrasonography and are reported as early as 15 weeks' gestation. Prior to fetal ultrasonography, an abdominal mass in the flank of an otherwise healthy newborn was the most common clinical presentation of unilateral multicystic dysplastic kidney.

In prenatally diagnosed multicystic dysplastic kidney, the abnormal kidney is palpable in only 13-22% of patients. The mass is usually mobile, ballotable, irregular in shape, nontender, and might transilluminate.

Physical Examination

Multicystic dysplastic kidney is usually asymptomatic and can remain undetected into adulthood. Abdominal or flank pain and respiratory distress are uncommon symptoms because of the pressure effect of the abnormal kidney.

Multicystic dysplastic kidney might be discovered during an investigation for urinary tract infection (UTI), voiding dysfunction, or hypertension. Multicystic dysplastic kidney may also be discovered when diagnostic imaging studies are performed to investigate a nonurinary problem.

Bilateral multicystic dysplastic kidney usually results in stillbirth or death within the first few days of life; however, an infant with bilateral multicystic dysplastic kidney who survived for 17 days was reported. Bilateral multicystic dysplastic kidney is usually associated with oligohydramnios, amnion nodosum, pulmonary hypoplasia, and Potter facies. Infants with bilateral multicystic dysplastic kidney who survive have renal failure from birth and require dialysis from the first day of life.

Workup

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Media Gallery

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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Author

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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 Association for the Advancement of Science, Eastern Society for Pediatric Research, Renal Physicians Association, American Academy of Pediatrics, American Pediatric Society, American Physiological Society, American Society of Nephrology, American Society of Pediatric Nephrology, American Society of Transplantation, Federation of American Societies for Experimental Biology, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Sigma Xi, The Scientific Research Honor Society, 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, The Ann and Robert H Lurie Children's Hospital of Chicago

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

Disclosure: Received income in an amount equal to or greater than $250 from: Alexion Pharmaceuticals; Horizon Pharmaceuticals); ; Dicerna Pharmaceuticals<br/>Incyte Pharmaceuticals; Eli Lilly for: Federation bio.

Additional Contributors

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.

Acknowledgements

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.

Multicystic Renal Dysplasia Clinical Presentation

History

Physical Examination

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