American Journal of Kidney Diseases
Volume 49, Issue 3 , Pages A49-A51, March 2007

Quiz Page March 2007

Article Outline

 

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Clinical Presentation 

A 6-year-old boy had an episode of painless gross hematuria. There was no recent history of infection or skin rash. Family history is significant for kidney stones on the father’s side. He was initially seen by his primary care physician and a urine dipstick showed a large amount of blood and more than 0.3 g/dL (3 g/L) of protein. Microscopy showed red blood cells too numerous to count. No cast or dysmorphic red blood cells were found. Random urine for calcium to creatinine ratio was 0.3. Intravenous pyelogram (Fig 1) was performed.

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■What is your diagnosis?

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Discussion 

What is your Diagnosis? 

The intravenous pyelogram shows left side duplex collecting systems. There is a stone of 1 cm diameter on the right side (Figure 2, Figure 3). The axes of both sides of the kidney are rotated. The upper and lower calyces on the left side are projected medially and anteriorly, respectively. The calyx on the right side is elongated in shape and is opening anteriorly (Fig 4). There is no hydronephrosis or ureteral obstruction observed in the intravenous pyelogram. Both the ureters are normal in caliber. Ultrasound (Fig 5) was done after the patient was seen and it shows a large horseshoe kidney with lower poles connected anterior to the aorta.

A horseshoe kidney consists of two functioning kidneys on each side of the midline. The kidneys are connected at the lower poles by either an isthmus of functioning parenchyma or fibrous tissues. The collecting systems are often rotated anteriorly. The incidence of horseshoe kidney is about 1 case in 400 to 600 persons worldwide.1 Although 90% of pediatric patients with horseshoe kidney are asymptomatic, they are more prone to have kidney stones, hydronephrosis, hematuria, and infection.2 The patient in this vignette also has positive family history of stones and hypercalciuria. The proteinuria is probably secondary to blood contamination in concentrated urine. Subsequent urinalysis showed negative protein.

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Final Diagnosis 

Horseshoe kidney with a double calyces on the left side and a stone on the right side.

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References 

  1. Weizer AZ, Silverstein AD, Auge BK, et al. Determining the incidence of horseshoe kidney from radiologic data at a single institution. J Urol. 2003;170:1722–1766
  2. Wilson C, Azmy AF. Horseshoe kidney in children. Br J Urol. 1986;58:361–363

 Case Provided By Keith K. Lau, MD, Lavjay Butani, MD, and Maha N. Haddad, MD, Department of Pediatrics, University of California, Davis

 Support: None.

 Potential conflicts of interest: None.

PII: S0272-6386(06)01700-8

doi:10.1053/j.ajkd.2006.11.029

American Journal of Kidney Diseases
Volume 49, Issue 3 , Pages A49-A51, March 2007