Left renal vein entrapment syndrome: nutcracker syndrome!

Abstract

Nutcracker syndrome (NCS) is a rare vascular compression disorder that involves compression of the left renal vein most commonly between the aorta and the superior mesenteric artery (SMA), although variations exist. It is associated with the formation of the left renal vein from the aortic collar during the 6th–8th week of gestation and abnormal angulation of the SMA from the aorta. Collateralisation of venous circulation including mainly the left gonadal vein and the communicating lumbar vein are the most significant effects. It has a female predilection occurring in the third to fourth decade and it tends to be diagnosed earlier in men. Affected individuals may present with a myriad of symptoms such as haematuria, left flank pain and proteinuria. As patients often present with these non-specific symptoms to primary care, knowledge of NCS is essential. The diagnosis can be rendered with Doppler ultrasonography, retrograde venography, CT angiography, intravascular ultrasound and magnetic resonance angiography. The authors describe the case of a 39-year-old woman with a low body mass index (BMI) presenting with generalised abdominal and flank pain as well as chronic microcytic anaemia. Physical examination findings were suggestive of biliary or renal colic. Laboratory investigations confirmed her anaemia (haemoglobin 88 g/L, mean corpuscular volume (MCV) 72 fL), but were otherwise unremarkable. Urinalysis showed proteinuria and haematuria. However, ultrasonography was unremarkable with a normal gallbladder and no evidence of calculi. Her CT scan showed marked compression of the left renal vein between the aorta and the SMA (nutcracker phenomenon), with upstream left renal, left gonadal and left lumbar vein dilatation. She was managed conservatively. This paper provides an overview of the aetiology, embryology, clinical manifestations, imaging modalities and management of NCS.

Associations of left renal vein entrapment with IgA nephropathy and Henoch–Schönlein purpura nephritis

“…NCS could be differentiated clinically into two subtypes as follows: renal presentations and non-renal presentations [ 2 ]. Hematuria, orthostatic proteinuria with or without flank pain is the common renal clinical presentations [ 3 ]. The non-renal presentations include abdominal pain, varicocele, dyspareunia, dysmenorrhea, fatigue, and orthostatic intolerance and so on [ 4 ].…”

Nutcracker syndrome: a case report

“…A rarer condition is posterior nutcracker, when the LRV is compressed between the aorta and the vertebral body. The term 'nutcracker syndrome', first described by De Schepper in 1972, is preferred over phenomenon if the vascular compression becomes symptomatic 2,3 .…”

“…N utcracker syndrome (NCS) is a condition caused by compression of the left renal vein (LRV), most often, between abdominal aorta and superior mesenteric artery (SMA) 1,2 . It may cause many symptoms in both children and adults, such as intermittent hematuria, proteinuria, flank pain, pelvic congestion in females, and varicocele in male patients.…”

Nutcracker syndrome due to aorto-mesenteric compression in adults: Case report and literature review

“…This is a rare situation whose prevalence is unknown [ 4 ]. It has been described as more common in women in the third or fourth decade, although a later study showed equal prevalence between men and women [ 5 ] with a peak prevalence in adults between 20 and 30 years old probably related to the rapid development of vertebral bodies during puberty, causing a narrowing of the angle between the abdominal aorta and the superior mesenteric artery [ 6 ] compression of the left renal artery is most often anterior, between the abdominal aorta and the superior mesenteric artery, more rarely compression is posterior when the left renal vein passes between the abdominal aorta and the spine, then we speak of a posterior nutcracker syndrome [ 7 ] the other etiologies: Pancreatic tumors, para-aortic adenopathy, retroperitoneal tumors, abdominal aortic aneurysm, duplication of the left renal vein, left renal ptosis, lordosis and decrease of retroperitoneal and mesenteric adipose tissue [ 8 ] the nutcracker syndrome can be clinically differentiated into 2 subtypes as follows: typical presentation (or renal presentation) and atypical presentation (or urological presentation). The typical clinical presentation includes hematuria, orthostatic proteinuria with or without flank pain.…”