Case ReportRight nutcracker syndrome associated with left-sided inferior vena cava, hemiazygos continuation and persistant left superior vena cava: a rare combination☆
Introduction
Nutcracker phenomenon is a well described anatomic anomaly which is usually associated with left renal vein (LRV) compression between the superior mesenteric artery (SMA) and aorta. Nutcracker phenomenon is called “nutcracker syndrome” when the compression of the renal vein becomes symptomatic as a result of renal venous hypertension, hilar and ureteral varices, and disruption of the small renal veins into the collecting system. The most commonly related symptoms are hematuria, proteinuria, flank pain, varicocele and chronic fatigue syndrome [1], [2], [3].
As well as its classical form there are also uncommon causes that result in compression of LRV, such as posterior nutcracker phenomenon (compression between aorta and vertebral column), compression between SMA and right renal artery or compression of a left-sided inferior vena cava (IVC) between SMA and aorta (Fig. 1A-D) [4], [5], [6]. On the other hand, not only the LRV but also the right renal vein (RRV) can be compressed and result in the right nutcracker syndrome. In English literature, there are a few case reports of right nutcracker syndrome; one was associated with pregnancy and two of them were associated with a left-sided IVC [1], [2], [7]. Related to the type of IVC anomaly-associated renal vein compression may be on either side; right or left (Fig. 1D-F) [1], [2], [6].
We reported here a case of a child with right nutcracker syndrome associated with left-sided IVC and hemiazygos continuation diagnosed by Doppler ultrasonography (US) and magnetic resonance angiography (MRA) to avoid risk of radiation in this age group.
Section snippets
Case report
A 12-year-old girl was admitted to the hospital for the evaluation of microscopic hematuria. The patient had been followed up for intermittent microscopic hematuria episodes for the previous 7 years. She had been examined with renal sonography 6 times with normal abdominal findings. Past medical history was unremarkable. There was no history of gross hematuria, trauma, urinary tract infection, arthralgias, or skin rashes. Family history was negative for hematuria, deafness, rheumatologic
Discussion
In normal anatomical formation, IVC lies on the right side of aorta and comprise four segments: hepatic, suprarenal, renal, and infrarenal. Embryogenesis of the IVC is a complex process which may result in different variations with renal vein anomalies. In the embryonic life during the sixth to eighth week of gestation, three pairs of fetal veins—posterior cardinal, subcardinal, and supracardinal veins—develop, regress, and form anastomoses between each other in an order and finally form the
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2018, Radiology Case ReportsCitation Excerpt :The diagnosis with Doppler ultrasound is done when the ratio of systolic peak velocity between the site of the compression and the vein at the renal hilum is >4.7 [1]. This finding has a sensitivity of 100% and a specificity of 90% [1,3]. Although this finding was described in the original nutcracker, we assume that it may be extrapolated.
The nutcracker syndrome: The usefulness of different MRI sequences for diagnosis and follow-up
2019, Clinical ImagingCitation Excerpt :Compression of the LRV between the abdominal aorta and superior mesenteric artery (SMA) is known as anterior NCS, while compression of the LRV between the aorta and vertebral column is known as posterior NCS [2,3]. In the case of left-sided inferior vena cava (IVC), the right renal vein (RRV) is compressed and this is referred to as right NCS [4]. NCS may present with symptoms such as flank pain, hematuria, proteinuria, pelvic congestion, and left-sided varicocele [5,6].
Persistent Left Superior Vena Cava: Why is Prenatal Diagnosis Important?
2022, Fetal and Pediatric PathologyLeft renal vein entrapment syndrome: Nutcracker syndrome!
2019, BMJ Case ReportsA rare variation of the hemiazygos vein draining into the persistent left superior vena cava
2019, Anatomical Science International
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No funding was received from any source. The authors declare no conflicts of interest.