Nutcracker Syndrome Associated with Non-glomerular Hematuria in Childhood / 대한신장학회잡지

ABSTRACT

PURPOSE: This study was designed to aid the diagnosis and to predict the outcorne by understanding the clinical course of nutcracker syndrome in childhood. METHODS: The clinical, laboratory, radiological and cystoscopic data from the medical records of eleven children who were diagnosed as nutcracker syndrome by gross hematuria and pressure gradient criteria (>3mrnHg) were studied retrospectively and analyzed. RESULTS: Sex ratio of the cases was 74, and the median age of onset was 12.8 (3-14.3) years. Six cases showed persistent and 5 cases manifested interrnittent, exercise induced hematuria. Left flank pain (64%), abdominal pain (18%), left varicocele (9%) were associated in some of the children, but hematuria was the only symptom in 36Yo. Left renal vein entrapment was documented in 10 cases by ultrasonography. Out of the 5 cases studied by renal Doppler ultrasonography, 4 and 5 cases showed higher (>5) mean left renal vein diameter ratio (Distal/ Aortomesenteric portion) and mean peak velocity ratio respectively. Unilateral bleeding from left ureteral orifice was documented in 7 of the 9 cases at cystoscopy. The mean pressure gradient between proximal left renal vein and inferior vena cava was 4.4+/-1.6 (3-7) mmHg. Hematuria of 25% and 57% of the cases disappeared spontaneously in 3 and 5 years after onset respectively. Proteinuria disappear- ed in 3 of the 5 initial proteinuric cases. CONCLUSION: Nutcracker syndrome must be considered in the differential diagnosis of non-glomerular, especially gross hematuria in childhood, and Doppler ultrasonography can aid diagnosis non-invasively. The renal function remained stable, but 4396 of the cases continued to show hematuria still 5 years after onset.