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Macroscopic hematuria caused by congenital portosystemic shunt and concomitant nutcracker syndrome
Author(s) -
Lee Sang Hyub,
Lee DongGi
Publication year - 2015
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12671
Subject(s) - medicine , nutcracker syndrome , concomitant , asymptomatic , portosystemic shunt , left renal vein , shunt (medical) , macroscopic hematuria , abnormality , inferior vena cava , radiology , gross hematuria , microscopic hematuria , renal vein , surgery , portal hypertension , kidney , gastroenterology , cirrhosis , psychiatry , proteinuria
Nutcracker syndrome (NCS) is an uncommon vascular abnormality that causes a variety of symptoms that range from asymptomatic microscopic hematuria to severe pelvic congestion. Congenital portosystemic shunt (CPSS) is an extremely rare anomaly that causes serious complications. Many cases of NCS and CPSS that have presented separately have been reported, but no cases of concomitant NCS and CPSS have been reported. We present a case of intermittent macroscopic hematuria in a patient with both NCS and CPSS. We diagnosed NCS on pressure gradient between the left renal vein (LRV) and the inferior vena cava. The presence of CPSS, which emerged from the LRV and connected to the extrahepatic portal vein, was confirmed on computed tomography. The interaction between NCS and CPSS resulted in mild intermittent macroscopic hematuria only, rather than the more common symptoms that occur when NCS or CPSS present separately.