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Updated: Apr 28 2022

Horseshoe Kidney

  • Overview
  • Snapshot
    • A 2-year-old boy is referred by his pediatrician to a nephrologist for recurrent urinary tract infections. He was born at 39 weeks and appeared healthy at birth; however, he has developed significantly more urinary tract infections than expected for a child of his age. Physical exam reveals increased fullness in the abdomen and an MRI scan is obtained. Urine cultures are obtained and empirical antibiotics are started pending culture results.
  • Introduction
    • Clinical definition
      • congenital malformation of the renal system resulting in fused kidneys
    • Epidemiology
      • common malformation found in 1/400 live births
      • twice as common in males
    • Pathogenesis
      • fusion of the lower poles of the kidneys (most common)
      • trapping of the fused kidneys under the inferior mesenteric artery
      • arrest of the normal retroperitoneal ascent of the kidneys
    • Associated conditions
      • Turner syndrome
      • trisomy 18 (Edwards syndrome)
    • Prognosis
      • typically very good
      • most patients remain asymptomatic
  • Presentation
    • Symptoms
      • asymptomatic (most common)
      • increased rate of urinary tract infections
      • hydronephrosis due to
        • ureteropelvic junction obstruction
      • renal stones
    • Physical exam
      • abdominal fullness upon palpation
  • Imaging
    • Kidney ultrasound
      • indications
        • initial evaluation of symptomatic patients
    • Voiding cysturethrogram
      • indications
        • further evaluation of abnormal anatomy
      • findings
        • fusion of kidney poles trapped under the inferior mesenteric artery
    • Intravenous pyelogram
      • indications
        • for evaluation of symptomatic kidney stones
      • findings
        • filling defect at the level of the obstruction
  • Differential
    • Unilateral renal agenesis
    • Posterior urethral valves
    • Multicystic dysplastic kidney
    • Duplex collecting system
  • Treatment
    • Conservative
      • no treatment for asymptomatic patients
    • Medical
      • antibiotics
        • indication
          • urinary tract infections
        • outcomes
          • very good
  • Complications
    • Postrenal kidney failure due to kidney stones
      • rare since most cases are asymptomatic
      • treatment
        • removal of kidney stones
    • Hydronephrosis if obstruction is left untreated
    • Anomalous original of multiple renal arteries may complicate surgery
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