Multicystic Renal Dysplasia Follow-up

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

Further Outpatient Care

  • Blood pressure should be checked at least once each year in patients with multicystic dysplastic kidney (MCDK), and hypertension should be treated. Ambulatory blood pressure monitoring should be considered to screen children for hypertension.
  • Preventative antibiotics are only necessary in those children with multicystic dysplasia of the kidney and vesicoureteral reflux (VUR). Annual voiding cystourethrography (VCUG) is not indicated.
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Deterrence/Prevention

  • During pregnancy, a woman with a history of Voiding cystourethrography should have her blood pressure followed closely, her urine screened for infection, and her kidney function periodically assessed.
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Complications

Complications of multicystic dysplastic kidney might be categorized as those due to multicystic dysplastic kidney and those due to associated urinary malformations.

  • Abdominal or flank pain
  • Urinary tract infection (UTI)
    • The Multicystic Kidney Registry reported UTIs in 12 (5%) of 260 patients who were observed for 5 years.[9]
    • Pyelonephritis, when present, is almost always on the contralateral side and is often associated with VUR.
    • UTI in patients with multicystic dysplastic kidney is rare because the ureteral atresia presumably prevents ascending infection; however, Hartman et al reported abscess formation in multicystic dysplastic kidney, and Reitelman et al reported an individual with a primarily infected kidney with multicystic dysplastic kidney.[27, 28] The latter authors presumed an ascending infection developed when bacteria traversed the microlumen of an atretic ureteric segment.
  • Hypertension
    • The Multicystic Kidney Registry reported mild hypertension in 4 (1.5%) of 260 individuals with multicystic dysplastic kidney who were observed for 5 years.[9]
    • A systematic review of 29 studies reported 6 cases of hypertension in 1115 eligible children with multicystic dysplastic kidney. The mean probability of a child with unilateral multicystic dysplastic kidney developing hypertension was 5.4 cases per 1000.[29] Although the risk of hypertension in multicystic dysplastic kidney is low, the results of this study did not allow firm recommendations on the frequency and duration of blood pressure measurement follow-up for children with multicystic dysplastic kidney. Thus, prospective cohort study with a long follow-up is needed.
    • Seeman et al performed ambulatory blood pressure monitoring on 25 children with multicystic dysplastic kidney.[30] Five (20%) children had blood pressures greater than the 95th percentile, 2 had combined daytime and nighttime hypertension, and 3 had isolated nighttime hypertension. Hypertension was more common in children with contralateral urinary abnormalities.
    • Ambrose et al identified 4 adults with hypertension that did not improve following nephrectomy of the kidney with multicystic dysplastic kidney.[31]
    • Hypertension that resolved after nephrectomy of an multicystic dysplastic kidney–affected kidney has been reported in 4 individuals. In one case, the plasma renin activity was elevated before nephrectomy and normalized after nephrectomy.[32]
    • Most patients with multicystic dysplastic kidney have no radiologically demonstrable blood flow. A nonfunctional bloodless kidney is an unlikely cause of hypertension in infancy and early childhood. However, the presence of a contralateral congenital urinary abnormality such as ureteropelvic junction obstruction (UPJO) or renal dysplasia, the development of a pyelonephritic scar in a contralateral kidney with VUR, or the effects over time of hyperfiltration of the contralateral kidney are potential causes of hypertension.
  • Renal malignancy
    • Nephroblastoma (Wilms tumor) in multicystic dysplastic kidney has been reported in 7 children with an average age of 7 months.
    • Renal cell carcinoma was reported in 13 patients at an average age of 39 years, and an embryonal tumor was reported in a 68-year-old patient.[21]
    • Nodular renal blastema is reported in 0.25-0.5% of the general population, 3-6.7% of individuals with multicystic dysplastic kidney, and 12-40% of patients with Wilms tumor.[22]
    • The increased incidence of nodular renal blastema in multicystic dysplastic kidney might confer a higher risk for Wilms tumor.
    • The intervening stroma of a kidney affected by multicystic dysplastic kidney may not undergo complete involution and may provide a focus for malignant degeneration. However, no increased risk of Willms tumor has been noted in multicystic dysplastic kidney. A systemic review of 26 studies demonstrated no cases of Willms tumor in 1041 children with unilateral multicystic dysplastic kidney.[23] These data are consistent with an earlier study by the American Multicystic Kidney Disease Registry that found no cases of renal neoplasia in 260 patients with multicystic dysplastic kidney.[24]
    • Beckwith noted that nodular renal blastema is present in as much as 1% of the general population and that Wilms tumor develops in approximately 1 in 8000 children.[22] Approximately 1 in 80 infants with nodular renal blastema develops Wilms tumor. The incidence of nodular renal blastema in multicystic dysplastic kidney is considered to be approximately 5%; therefore, 20 multicystic dysplastic kidney–affected kidneys would need to be removed to ablate one with nodular renal blastema and 1600 kidneys with multicystic dysplastic kidney would need to be removed to prevent one case of Wilms tumor. Because the cure rate for Wilms tumor is approximately 90%, 16,000 multicystic dysplastic kidney–affected kidneys would need to be removed to save one life. Based on this analysis, Beckwith does not recommend nephrectomy to prevent the development of Wilms tumor in a patient with multicystic dysplastic kidney.
  • Gastric outlet obstruction and respiratory depression: Gastric outlet obstruction with feeding difficulties and respiratory depression due to elevation of the hemidiaphragm are rare reported complications.
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Prognosis

  • Prognosis depends on whether the involvement is unilateral or bilateral and on the presence and severity of associated anomalies.
  • Most individuals with isolated unilateral multicystic dysplastic kidney do not experience any problems or complications as a consequence of this congenital abnormality.
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Patient Education

  • All patients with multicystic dysplastic kidney should be counseled on the lifetime implications of the presence of only one functional kidney.
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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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