Available Online at http://www.recentscientific.com International Journal of CODEN: IJRSFP (USA) Recent Scientific International Journal of Recent Scientific Research Research Vol. 11, Issue, 01(C), pp. 36836-36838, January, 2020 ISSN: 0976-3031 DOI: 10.24327/IJRSR CASE REPORT NUTCRACKER SYNDROME: A RARE ETIOLOGY OF VARICOCELE Abdelhalim Mahmoudi*, Khalid Khattala and Youssef Bouabdallah Department of Pediatric Surgery, CHU Hassan II, Faculty of Medicine and Pharmacy, Sidi Mohamed Ben Abdellah University, Fez, Morocco DOI: http://dx.doi.org/10.24327/ijrsr.2020.1101.5005 ARTICLE INFO ABSTRACT Article History: Nutcracker syndrome (NS) describes left renal vein compression between the superior mesenteric Received 14th October, 2019 artery and the aorta. NS is a rare entity. We report a case of nutcracker syndrome diagnosed with Received in revised form 29th varicocele. November, 2019 A 15 -year-old man with good past health presented with a left varicocele and intermittent left Accepted 05th December, 2019 flank pain. He had no urinary symptoms and no haematuria. Doppler ultra sonography an MRI Published online 28th January, 2020 showed a grossly distended left renal vein, and left varicocele secondary to the nutcracker syndrome The patient underwent laparoscopic varicocelectomy and conservative treatment was adopted for the left renal vein compression. The evolution is favorable. Key Words: NS is a rare entity. The treatment options are ranged from surveillance to nephrectomy. Treatment Nutcracker syndrome, Left renal vein decision should be based on the severity of symptoms and their expected reversibility with regard to hypertension, varicocele, children. patient’s age and the stage of the syndrome. Copyright © Abdelhalim Mahmoudi, Khalid Khattala and Youssef Bouabdallah, 2020, this is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution and reproduction in any medium, provided the original work is properly cited. INTRODUCTION venousout flow obstruction of the LRV. We report a case of nutcracker syndrome diagnosed with varicocele. Nutcracker syndrome (NS) also known as left renal vein(LRV) entrapment syndrome, was first defined anatomically by Grant in 1937 as follows: “. the left renal vein, as it lies between the aorta and superior mesenteric artery, resembles anuty between the jaws of a nutcracker”1. The nutcracker phenomenon was first described by de Schepper2 in 1972. Compression of the left renal vein between the superior mesenteric artery (SMA) and the aorta causes left renal vein hypertension2. The presence of symptoms and signs related to LRV outflow obstruction, including abdominal and left flank pain, macroscopic and microscopic hematuria, proteinuria and orthostatic proteinuria, and varicocele.3The diagnosis of NS requires a high index of suspicion. It is extremely rare, and it represents A diagnos is of exclusion. Doppler ultra sonography is recommended as a first-line study. CT Scan and MRI can A also be obtained, and they can beused to demonstrate Figure 1 A- Doppler ultra sonography showed left varicocele. compression of the LRV, gonadal vein distention, and pelvic congestion.3 Patients presenting with symptoms or signs and anatomic compression of the left renal vein (LRV) can be considered for intervention. Open, laparoscopic, and endovascular techniques have been developed to decrease the *Corresponding author: Abdelhalim Mahmoudi Department of Pediatric Surgery, CHU Hassan II, Faculty of Medicine and Pharmacy, Sidi Mohamed Ben Abdellah University, Fez, Morocco International Journal of Recent Scientific Research Vol. 11, Issue, 01(C), pp. 36836-36838, January, 2020 findings suggestive of nutcracker are present without clinical symptoms. Term of NS is used for patients with clinical symptoms associated with nutcracker anatomy. The exact prevalence of NCS is unknown, Patients can present at any age from child hood to the seventh decade, with prevalence peaking in young (second or third decade) and middle aged adults.6 Left renal ptos is, lordos is and decreased retroperitoneal and mesenteric fat tissue may cause to NS6,7. Right-sided NS is a more rare condition. Pregnancy is defining as a factor contributing to right-sided NS by compression of large veins6. B Left-sided IVC, hemiazygos continuation and persistent left B- Doppler ultra sonography showed a grossly distended left renal vein. superior vena cava combination is another rare cause of right NCS8. All of the anatomic mechanisms involved in renal vein compression are resulting without flow obstruction leads to LRV hypertension with a measurable renocaval pressure gradient Clinical features of patients with NS are various. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. Some patients have severe and persistent symptoms. Symptoms are aggravated by physicalactivity9. Symptoms include hematuria, orthostatic proteinuria, flank pain, abdominal pain, varicocele, dyspareunia, dysmenorrhea, fatigue and orthostatic intolerance10. The symptoms of autonomic dysfunction suchas hypotension, syncope, and tachycardia could be seen but they are rare11.while some patients suffer from severe, persistent symptoms, it is possible for others, especially children, to remain asymptomatic.12 Doppler ultrasono graphy (DUS) is recommended as a first-line study. It allows real-time assessment of the flow and Figure 2 Magnetic Resonance Imaging (MRI) showed compression of the left peakvelocities within the lumen of the LRV. A ratio of peak renal vein between the superior mesenteric artery and the Aorta. systolic velocity of the aortomesenteric segment to the hilar Case Summary portion of>4.2 to 5.0 Is considered one of the diagnostic criteria of NS13.CT and MRI can also be obtained, and they can A 15 -year-old man with good past health presented with a left be used to demonstrate compression of the LRV, gonadal vein varicocele and intermittent left flank pain. He had no urinary distention, and pelvic congestion. Furthermore, findings such symptoms and no haematuria. The clinical examination showed as an LRV hilar diameter to aortomesenteric diameter ratio of a grade II varicocele. Doppler ultra sonography showed a >4.9, the “beak sign,” and an SMA branching angle of grossly distended left renal vein, and left varicocele secondary <35degreesfrom the aortic origin can be also useful for the to the nutcracker syndrome ( fig.1). The cross sectional view of 13 diagnosis of NS .Retrograde venography is the most magnetic resonance imaging (MRI) showed compression of the informative method although it is an invasive test. It is not left renal vein between the superior mesenteric artery and the commonly per formed in patients who have not severe aorta (Fig .2). This compression caused the marked dilatation 14 symptoms. of the distal left renal vein with associated venous hypertension (nutcracker phenomenon). The patient subsequently, under Spontaneous resolution by physical development during went laparoscopic varicocelectomy and conservative treatment childhood is possible15. Conservative approach with was adopted for the left renal vein compression, due to the observation during minimum 2 years without medication is the intermittent nature of the clinical symptoms and its moderate best option for patients younger than 18 years old. As in the intensity. The evolution is favorable. case of our patient, a laparoscopic varicocelectomy and conservative treatment was adopted for the left renal vein DISCUSSION compression. Nutcracker syndrome (NS), first described in 1937 by Surgical procedures are used for treatment in patients with Grant,1refers to patients presenting with symptoms and signs severe symptoms. Nephropexy, intravascular and extravascular associated with the anatomic compression of the left renal vein stent implantation, transposition of the LRV or SMA, gonado (LRV).Most commonly known is the anterior NS, which refers caval by pass, renal auto trans plantation and nephrectomy are to the compression of the LRV by the superior mesenteric surgical procedures. 3,4 artery (SMA) and the aorta .A second variant is the posterior Open surgical techniques for anterior NS include LRV NS, in which the LRV is compressed between the aorta and the transposition, LRV transposition with patch venoplasty, patch 3,5 vertebral body. The terms nutcracker phenomenon and venoplasty without LRV transposition, LRV transposition with nutcracker syndrome (NS) are sometimes used as synonym in saphenous vein cuff, gonadal vein transposition and saphenous the literature. Nutcracker phenomenon descript anatomic vein bypass16. 36837 | P a g e AbdelhalimMahmoudi, Khalid Khattala and Youssef Bouabdallah., Nutcracker Syndrome: A Rare Etiology of Varicocele Surgical placement of an externals tent to the LRV is another 7. Basile A, Tsetis D, Calcara G, Figuera M, Coppolino F, surgical approach to NS 17. Endovascular stenting is an PattiMT, Midiri M, Granata A. Nutcracker syndrome alternative treatment option. It can be preferred to surgery due to leftrenalvein compression by an aberrant right because of the long period of renal congestion, additional renalartery. Am J Kidney Dis 2007; 50: 326-329 . anastomoses and extensive dissection requirement of the 8. Yildiz AE, Cayci FS, Genc S, Cakar N, Fitoz S. Right surgery. nutcracker syndrome associated with left-sided inferior vena cava, hemiazygos continuation and persistant left CONCLUSION superiorvena cava: a rare combination. Clin Imaging NS is a