Multicystic Renal Dysplasia Follow-up

Updated: Aug 22, 2016
  • Author: Agnieszka Swiatecka-Urban, MD, FASN; Chief Editor: Craig B Langman, MD  more...
  • Print
Follow-up

Further Outpatient Care

See the list below:

  • 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 who have vesicoureteral reflux (VUR). Annual voiding cystourethrography (VCUG) is not indicated.
Next:

Deterrence/Prevention

See the list below:

  • During pregnancy, a woman with a history of multicystic dysplasia of the kidney should have her blood pressure followed closely, her urine screened for infection, and her kidney function periodically assessed.
Previous
Next:

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. [11]
    • 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. [31, 32] 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. [11]
    • 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. [33] 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. [34] 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. [35]
    • 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. [36]
    • 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. [13]
    • 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. [26]
    • 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 Wilms tumor has been noted in multicystic dysplastic kidney. A systemic review of 26 studies demonstrated no cases of Wilms tumor in 1041 children with unilateral multicystic dysplastic kidney. [27] 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. [28]
    • 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. [26] 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.
    • In 2015, the Canadian Urological Society updated their guidelines following a 2014 literature review to obtain up-to-date information regarding the medical implications of having a multicystic dysplastic kidney, including complications and appropriate follow up to reduce the risk of complications. [37]
  • 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.
Previous
Next:

Prognosis

See the list below:

  • 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.
Previous
Next:

Patient Education

See the list below:

  • All patients with multicystic dysplastic kidney should be counseled on the lifetime implications of the presence of only one functional kidney.
  • Women with fetal multicystic dysplastic kidney may benefit from genetic counselling, supported by recent findings that a substantial proportion of patients with multicystic dysplastic kidneys have abnormal microarray analysis. [38, 39]
Previous