Nutcracker syndrome in children presenting with recurrent

gross hematuria

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Case Report

Nutcracker syndrome in children presenting withrecurrent gross hematuria

Alkarani T. Patil a,*, K.S. Sanjay b, M. Govindraj b

a Associate professor of Pediatrics, Department of Pediatrics & Pediatric Nephrology, Indira Gandhi Institute of Child

Health, Bangalore, Karnataka, Indiab Department of Pediatrics, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India

a r t i c l e i n f o

Article history:

Received 11 January 2015

Accepted 21 February 2015

Available online xxx

Keywords:

Nutcracker syndrome

CT angiography

Left renal vein

Superior mesenteric artery

Abdominal aorta

* Corresponding author. Incharge PediatricIndia.

E-mail address: alkaranipatilurs@gmail.chttp://dx.doi.org/10.1016/j.apme.2015.02.0180976-0016/Copyright © 2015, Indraprastha M

Please cite this article in press as: Patil ATApollo Medicine (2015), http://dx.doi.org/

a b s t r a c t

Nutcracker syndrome is a rare cause of hematuria. Two children who presented to us with

recurrent gross hematuria were evaluated. Renal parenchymal disease and abnormalities

in the urinary tract were ruled out. CT angiography revealed a compressed left renal vein

with dilatation and hence a diagnosis of nutcracker syndrome was made. A high index of

suspicion is required for diagnosis of nutcracker syndrome.

Copyright © 2015, Indraprastha Medical Corporation Ltd. All rights reserved.

1. Introduction

The term of nut cracker syndrome (NCS) is used for patients

with clinical symptoms associated with nut cracker anatomy.

Nut cracker phenomenon (NCP) refers to compression of the

left renal vein (LRV), commonly between abdominal aorta (AA)

and superior mesenteric artery (SMA), leading to stenosis of

the aorto mesenteric portion of the LRV and dilatation of the

distal portion. The terms nut cracker phenomenon and nut

cracker syndrome are used as synonym in the literature. NCP

refers to anatomic and hemodynamic abnormalities, NCS re-

fers to clinical manifestations of the abnormality.1 This phe-

nomenon was first noticed in 1950 by El-Sadr and Mina2 and

Nephrology, Indira Gandh

om (A.T. Patil).

edical Corporation Ltd. A

, et al., Nutcracker synd10.1016/j.apme.2015.02.

later in 1972, the Belgian physician De Schepper3 referred to

the disorder as “nut cracker syndrome”. It is also called as LRV

entrapment syndrome and can be divided into two types.

Anterior NCS refers to compression of a normally situated LRV

by the aorta and the SMA and accounts for most of the NCS

cases. Posterior NCS, accompanied by a retroaortic LRV, is

usually attributed to a small space between the aorta and the

vertebral column.

Prevalence of NCS is unknown, though it may occur from

childhood to old age. Most symptomatic patients are in their

second and third decade of life, and is slightly more prevalent

in females.4 A low body mass index (BMI) has been shown to

correlate positively with NCS.5 Theories of causes of NCP

include posterior renal ptosis, an abnormally high course of

i Institute of Child Health, Dharmaram college post. Bangalore,

ll rights reserved.

rome in children presenting with recurrent gross hematuria,018

a p o l l o m e d i c i n e x x x ( 2 0 1 5 ) 1e32

the LRV, and an abnormal SMA branching from the aorta.6 The

LRV compression leads to renal vein hypertension, leading to

rupture of the thin-walled vein into the renal calyceal fornix

with presentation of intermittent gross or microscopic he-

maturia. Collateral venous circulation formation such as

prominent left ovarian vein or testicular vein with its associ-

ated symptoms, such as vulvar varices in females or varico-

cele in males has been observed. Other symptoms include left

flank pain, orthostatic proteinuria, chronic fatigue syndrome

and gastrointestinal symptoms.7 Here we report two cases

with nut Cracker syndrome in our pediatric nephrology unit.

2. Case report

The first child was a 9 year old female child hospitalized in our

pediatric nephrology unit for intermittent hematuria and

recurrent left flank pain of 2 years duration. The patient

continued to have non colicky left flank and lower abdominal

pain, aggravated by change in position. The second childwas 8

year old boy who came to our unit with similar complaints of

recurrent hematuria since 2 months, no significant past

medical history or examination findings were observed in

both the children. Urine red cell morphology showed

isomorphic red cells in both children. There was no evidence

of proteinuria in the early morning or day time urine sample

which was tested by dipstick method. 24 hrs urinary proteins

were 140 mg in the first child and 128 mg in the second child.

Urine calcium/creatinine ratio was 0.1 in both the children.

BMI was 14.34 kg/m2 and 12.62 kg/m2 respectively, which is

low in both the children. Renal ultrasonography and renal

doppler were found to be within normal limits. Computerized

tomography angiography (CTA) revealed acute angulation of

the origin of superiormesenteric artery from the aorta in both.

The angle between SMAand aortawas found to be less than 21

degrees in the first child (Fig. 1A) and 18 degrees in the second

child (Fig. 2A). The distal third of left renal vein was seen to be

significantly compressed between superior mesenteric artery

and aorta (Figs. 1B and 2B). These findings were characteristic

of nutcracker syndrome. The 9 year old female underwent

stenting of the left renal vein, and is on follow up with no

recurrence of hematuria. The second child received no surgi-

cal treatment and has remained stable over the subsequent

two years.

Fig. 1 e A: Coronal section of the CT- angiography shows

angulation between the abdominal aorta (AA) and superior

mesenteric artery (SMA) is < 21 degrees. B: Axial CT Image

showing compression of left renal vein between aorta and

superior mesenteric artery.

3. Discussion

NCS is rare but treatable condition.8 If a patient has symptoms

of hematuria and pelvic congestion,the association of left

sided flank pain, pelvic discomfort, pelvic and vulvar varices

in the female and varicocele in the male, constitutes a strong

basis for the diagnosis. Imaging, such as Doppler ultrasound,

computerized tomographic angiography (CTA), magnetic

resonance angiography (MRA) is required to diagnose NCS.

CTA and MRA can demonstrate the precise LRV compression

point together with peri renal and/or gonadal varices. Retro-

grade phlebography and cine video angiography with reno

caval pressure gradient determination is accepted as the gold

standard in the final diagnosis of NCS.8The normal SMA

Please cite this article in press as: Patil AT, et al., Nutcracker syndApollo Medicine (2015), http://dx.doi.org/10.1016/j.apme.2015.02.

originates behind the neck of the pancreas at the level of the

first lumbar vertebra, and usually creates an acute angle at its

origin from the aorta. Mean SMA angles in children are

45.8 ± 18.2 degrees for boys and 45.3 ± 21.6 degrees for girls.

Mean SMA-aorta distances in children are 11.5 ± 5.3 mm for

boys and 11.5 ± 4.5 mm for girls.9 The angle between the aorta

and SMA in our report was found to be 21 and 18 degrees by

CTA. Both SMA angle and SMA distance correlate with BMI.

One of the presenting symptoms of NCS is weight loss and

most patients have low BMI at presentation.5 In both the

children BMI was low.

Conservative treatment has been suggested for mild he-

maturia. Surgical or radiological interventions are indicated

rome in children presenting with recurrent gross hematuria,018

Fig. 2 e A: Sagittal section of the CT- angiography shows

angulation between the abdominal aorta (AA) and superior

mesenteric artery (SMA) is < 18 degrees. B: Axial CT Image

showing compression of left renal vein between aorta and

superior mesenteric artery.

a p o l l o m e d i c i n e x x x ( 2 0 1 5 ) 1e3 3

for severe pain, significant hematuria and renal functional

impairment or if symptoms are not relieved after more than

two years of conservative treatment. Current open surgery

technique includes LRV transposition, renal auto trans-

plantation, SMA transposition, gonadocaval bypass and

external stent implantation. LRV transposition is the most

frequent and most effective technique. The advantages are

shorter period of renal ischemia and fewer anastomosis,

although there is a risk of LRV thrombosis.10 Renal

Please cite this article in press as: Patil AT, et al., Nutcracker syndApollo Medicine (2015), http://dx.doi.org/10.1016/j.apme.2015.02.

autotransplantation is a more invasive technique with excel-

lent results. Placement of an external stent to the LRV is

another approach.1 Endovascular surgery (EVS) has defini-

tively become more appealing than traditional open surgery.8

Anticoagulant and antiplatelet treatment is recommended to

lower the risk of thrombosis.

In conclusion, any child presenting with intermittent he-

maturia not attributed to renal pathology or renal calculi

should be considered for a Doppler ultrasound and CTA to rule

out the presence of nut cracker syndrome. NCS can be

managed conservatively if mild hematuria is present. Surgical

or intravascular interventions are reserved for severe symp-

toms, and EVS is the primary treatment option for patients

requiring surgery.

Conflicts of interest

All authors have none to declare.

r e f e r e n c e s

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2. El-Sadr AR, Mina E. Anatomical and surgical aspects in theoperative management of varicocele. Urol Cut Rev.1950;54:257e262.

3. De Schepper A. Nutcracker phenomenon of the renal veincausing left renal vein pathology. J Belge Radiol.1972;55:507e511.

4. Mahmood SK, Oliveira GR, Rosovsky RP. An easily misseddiagnosis: flank pain and nutcracker syndrome. BMJ Case Rep.2013;37:415e418.

5. Ozkurt H, Cenker MM, Bas N, et al. Measurement of thedistance and angle between the aorta and superiormesenteric artery:normal values in different BMI categories.Surg Radiol Anat. 2007;29:595e599.

6. Fu WJ, Hong BF, Xiao YY, et al. Diagnosis of the nutcrackerphenomenon by multislice helical computed tomographyangiography. Chin Med J (Engl). 2004;117:1873e1875.

7. Scholbach T. From the nutcracker phenomenon of the leftrenal vein to the midline congestion syndrome as a cause ofmigraine, headache, back and abdominal pain and functionaldisorders of pelvic organs.Med Hypotheses. 2007;68:1318e1327.

8. Waseem M, Upadhyay R, Prosper G. The nutcrackersyndrome: an under recognized cause of hematuria. Eur JPediatr. 2012;171:1269e1271.

9. Arthurs OJ, Mehta U, Set PA. Nutcracker and SMA syndromes:what is the normal SMA angle in children? Eur J Radiol.2012;81:e854ee861.

10. Said SM, Gloviczki P, Kalra M, et al. Renal Nutcrackersyndrome:surgical options. Semin Vasc Surg. 2013;26:35e42.

rome in children presenting with recurrent gross hematuria,018

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