ESPN 50th Annual Meeting

ESPN 2017


 
Nutcracker Syndrome (NCS) and its variants: three cases report.
SIMONA SPADARELLA 1 GABRIELE MALGIERI 1 FRANCESCA NUZZI 1 PATRIZIA DI MATTEO 1 FRANCESCO ESPOSITO 2 ALFONSO FERRETTI 1 BRUNO MINALE 1 ANGELA DE LUCA 1 CARMINE PECORARO 1

1- UNIT OF NEPHROLOGY AND DIALYSIS, CHILDREN HOSPITAL SANTOBONO, NAPLES, ITALY
2- UNIT OF RADIOLOGY, CHILDREN HOSPITAL SANTOBONO, NAPLES, ITALY
 
Introduction:

 NCS refers to compression of the left renal vein between the aorta and the superior mesenteric artery (anterior NCS) or between the aorta and the vertebral body (posterior NTS), with impaired blood outflow often accompanied by distention of the distal portion of the vein. The most common symptoms are gross hematuria, left abdominal pain, varicoceles, proteinuria. It could be asintomatic too.

Material and methods:

 Case 1: 4 years old girl, presented with several recurrent episodes of gross hematuria associated with left abdominal pain; renal function, urinary metabolites and urinary tract US were normal. The abdomen magnetic resonance (acronimo in inglese) showed an entrapment of a retro-aortic left renal vein between the aorta and the vertebral column.

Case 2: 11 years old boy, with a history of 4 episodes of gross hematuria associated with lumbar pain; laboratory tests and urinary US were normal. The CT-angiography showed a dilation of  initial tract of left renal vein, with a reduced caliber in the portion interposed between abdominal aorta and superior mesenteric artery.

Case 3: 14 years old boy, with mild proteinuria and gross hematuria. Kidney function tests were normal. Abdominal US and CT-angiography showed a dilated left renal vein, with a reduced aorto-mesenteric caliber angle. 

Results:

 Because of the variability of symptoms and absence of consensus on diagnostic criteria, the exact prevalence of NCS is unknown. 

Conclusions:

 NCS represents a cause of non-glomerular gross hematuria always be taken into account, after excluding the most frequent causes, especially if associated with abdominal left side pain, but that may present themselves, as in our case, with asymptomatic proteinuria. In these patients it’s essential renal vascular district study to confirm or rule out the suspected diagnosis.