Multicystic Renal Dysplasia

Multicystic Renal Dysplasia 

Updated: 10/05/2020

© Jun Wang, MD, PhD

General features
  • AKA multicystic dysplastic kidney
  • Congenital
  • Most common pediatric cystic renal disease

  • Usually sporadic but may be familial
  • Bilateral involvement usually results in stillbirth or death within the first few days of life 
Pathogenesis
  • Abnormal induction of metanephric mesenchyme by ureteral bud
Clinical features
  • No significant clinical problem if unilateral
  • Abdominal mass: Most common cause of abdominal mass in the newborns
  • Commonly associated with ureteropelvic obstruction, ureteral agenesis, atresia or reflux
  • May presents with urinary tract infection, voiding dysfunction, or hypertension
Radiologic findings
  • Sonographic findings: Multiple cysts
  • Voiding cystourethrography: Vesicoureteral reflux
  • Dimercaptosuccinic acid (DMSA) renal scanning: Loss of renal function, used if sonographic examination inconclusive
Pathological findings
Diagnosis
  • Antenatal sonography
  • Image studies
  • Biopsy
Management
  • Observation 
  • Treat complications, such as infection
  • Nephrectomy: Controversial



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