Multicystic Renal Dysplasia Treatment & Management

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

Medical Care

The role of nephrectomy in multicystic dysplasia of the kidney (MCDK) is controversial. Prior to the availability of modern ultrasonography, nephrectomy was often required to establish the diagnosis. A survey by Bloom et al revealed that 65% of pediatric urologists recommended nephrectomy for multicystic dysplastic kidney, compared with more recent data that indicate only 40% of pediatric urologists recommend nephrectomy.[17, 21]

  • The traditional reasons to consider nephrectomy are to treat or prevent abdominal or flank pain, urinary tract infection (UTI), hypertension, or renal malignancy.
  • If a child has significant abdominal or flank discomfort that can be attributed to the pressure effects of multicystic dysplastic kidney, the abnormal kidney should be removed.
  • If a child has evidence of a UTI, consider a nephrectomy only if the UTI involves the kidney with multicystic dysplastic kidney.
  • The presence of ipsilateral vesicoureteral reflux (VUR), pyelonephritis in the contralateral kidney, or recurrent episodes of cystitis are not sufficient justification for a nephrectomy in multicystic dysplastic kidney.
  • If a child has hypertension, consider a nephrectomy only if the hypertension can be shown to be due to the kidney with multicystic dysplastic kidney.
  • The presence of a renal malignancy is an indication for nephrectomy.
  • The main controversy regarding the indications for nephrectomy is whether this procedure prevents a renal malignancy.
    • Determining whether a renal malignancy is present in individuals with multicystic dysplastic kidney might be difficult. The retroperitoneal location precludes physical detection of a small mass, and the lack of urine output from the affected kidney does not allow for cytologic examination.
    • The evidence for the presence of a renal malignancy must be based on diagnostic imaging changes, and because cysts can be noted in renal malignancy, differentiation might be difficult.
  • Beckwith's mathematical analysis does not recommend nephrectomy based on the calculated incidence of Wilms tumor, but the calculation does not consider the incidence of renal carcinoma (see Complications).[22] Whether the risk of renal malignancy in those individuals who show involution is different compared with the risk in those who do not is unknown.
  • Some concern surrounds the possibility that the intervening stroma of a kidney affected by multicystic dysplastic kidney might not undergo complete involution and might provide a focus for malignant degeneration. However, no increased risk of Wilms tumor has been demonstrated in patients with multicystic dysplastic kidney. A systemic review of 26 studies demonstrated no cases of Wilms 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]
  • Some authors recommend nephrectomy only in patients who do not show involution over a defined period, such as the first 5 years of life. Because the peak incidence of Wilms tumor is in infancy and declines after age 5 years, this approach seems less scientific.
  • A poll of insurance companies revealed that only 15% issue life insurance if the multicystic dysplastic kidney–affected kidney is left in situ, compared with 70% if the kidney is removed.
  • The role of laparoscopic nephrectomy for multicystic dysplastic kidney is controversial.
  • Individuals with multicystic dysplastic kidney should undergo renal ultrasonography every 6-12 months until age 5 years or until involution is noted.
  • Individuals with multicystic dysplastic kidney and contralateral VUR should receive antibiotic prophylaxis during infancy and early childhood, the ages during which the risk for scarring due to pyelonephritis is highest. Miller et al reported on 75 children with multicystic dysplastic kidney.[25] Nineteen (26%) had VUR. After a median follow-up of 4.4 years, spontaneous resolution occurred in 89% of patients with grades I and II VUR, compared with only 50% of patients with grades III and IV VUR (P = .14). The presence of VUR did not affect the sonographically measured growth of the contralateral kidney.
  • Lifetime follow-up is required whether or not involution has occurred or a nephrectomy has been performed because long-term studies of patients with a single kidney report hypertension in 27-47% of patients, proteinuria in 23-47% of patients, and renal insufficiency in 3-13% of patients.
    • Sporadic clinical reports have documented the development of proteinuria and focal glomerulosclerosis in patients with a single kidney.
    • Follow-up should include routine and ambulatory measurement of the blood pressure, urinalysis, and periodic assessments of kidney filter function.
    • The presence of associated urinary and nonurinary abnormalities might require specific follow-up.
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Surgical Care

  • Nephrectomy for multicystic dysplastic kidney is conventionally done as an inpatient procedure. However, outpatient nephrectomy has been reported through a 2.5-cm incision without the need for overnight hospitalization.
  • The role of laparoscopic nephrectomy for multicystic dysplastic kidney is controversial.[26]
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Consultations

  • Consultations with a pediatric nephrologist and a pediatric urologist should be obtained.
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Diet

  • A diet that minimizes salt and protein to the recommended daily requirements for age is recommended for all patients with only one functional kidney.
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Activity

  • Lifestyle recommendations for patients with only one functional kidney include maintaining a thin body habitus and daily aerobic exercise.
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Proceed to Medication
 
 
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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