Korean Journal of Nephrology 1998;17(5):702-707.

소아의 비사구체성 혈뇨와 연관된 Nutcracker 증후군 (Nutcracker Syndrome Associated with Non-glomerular Hematuria in Childhood)

백경훈, 민재홍, 박경미, 김정수, 하일수, 정해일, 최용, 김우선, 김인원 (Kyung Hoon Paek, Jae Hong Min, Kyung Mi Park, Jung Sue Kim, Il Soo Ha, Hae Il Cheong, Yong Choi, Woo Sun Kim and In One Kim)

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 7:4, 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 consi- dered 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.