The Egyptian Journal of Radiology and Nuclear Medicine

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1 The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) Contents lists available at ScienceDirect The Egyptian Journal of Radiology and Nuclear Medicine journal homepage: Review Testicular vein syndrome: Review of the literature and recent case report Hidayatullah Hamidi Radiology Department, French Medical Institute for Mothers and Children (FMIC), 3rd District, Kabul, Afghanistan article info abstract Article history: Received 26 December 2016 Accepted 26 August 2017 Available online 28 December 2017 Keywords: Testicular vein syndrome Ureteral obstruction Aberrant abdominal vessels Obstructive uropathy Purpose: To perform a systematic review of cases published about the extremely rare entity of testicular vein compression on ureter with resultant hydroureteronephrosis and addition of a new case of left side testicular vein syndrome. Materials and method: A search of English-language literature through Google Scholar and PubMed was performed up to December 2016, besides; references of all identified case reports were reviewed. Results and conclusion: Based on this review of the literature only seven cases have been reported in English-language journals until December 2016 and the author present the 8th case. Four of the cases involved the right side and four the left side (Including the present case). In three cases, pathologically altered testicular vein resulted in obstructive uropathies, while in remaining five cases; the normal testicular vein was indenting over the ureter anteriorly at the crossing point. Ó 2017 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier. This is an open access article under the CC BY-NC-ND license ( 4.0/). Contents 1. Introduction Material and methods Results Case Case Case Case Case Case Case Case 8: (Current case) Discussion Number, course and size of testicular veins Clinical perspective Imaging work up Treatment and prognosis Conclusion Conflict of interest Acknowledgment References Peer review under responsibility of The Egyptian Society of Radiology and Nuclear Medicine. address: Hidayatullah.hamidi@fmic.org.af X/Ó 2017 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier. This is an open access article under the CC BY-NC-ND license (

2 1126 H. Hamidi / The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) Introduction Obstruction of ureter by an atypical compressing vessel is not an unusual entity and often results from the renal lower polar aberrant artery [1]. Other cause of vascular compression can be a retrovascular course of the ureter especially the retrocaval ureter [2]. It can also occur at the point of intersection of the ureter with retroperitoneal vessels like ovarian vein and very rarely, the testicular vein; Testicular Vein Syndrome (TVS) [2]. TVS was first described in 1975 reported by Hans-Eberhard et al. when they reported a case of an enlarged right spermatic vein with atypical course resulting in hydroureteronephrosis in a 28-year-old man in Thereafter only 6 cases of this rare entity have been reported in the literature. The author presents the 8th case of TVS with a review of previously reported cases. 2. Material and methods A search of the English-language literature through Google Scholar and PubMed was performed up to December 2016 with key phrases of; testicular vein syndrome, testicular vein compression on ureter, spermatic vein compression on ureter and hydronephrosis caused by testicular vein/spermatic vein. Besides references of all identified case reports were reviewed. Inclusion criteria were male patients with hydronephrosis caused by testicular vein compression. The exclusion criterion was proximal urinary tract obstructive entities caused by aberrant or abnormal vessels rather than the testicular vein. The literature was surveyed for clinical presentations, preference of side, involved site, pathological changes and size of the testicular vein, changes in affected ureter, grading of resultant proximal Table 1 Information about the reported cases, details about side and site of pathology, presenting symptoms and laboratory findings. N Author/Year Age (years) Location Side Level Symptoms Blood and urine exam 1 Mellin et al. (1975) 28 Wisconsin, USA Right L4 Right flank and right lower abdominal dull pain Normal for 4 months 2 Kretkowski et al. (1977) 20 Maryland, USA Left L2/3 disc Single episode of gross hematuria one month Normal ago 3 Lassnig et al. (1978) Young adult Left Not available 4 Meyer et al. (1991) 42 Chicago USA Right L3 Intermittent right flank pain and microscopic Normal hematuria, history of lithotripsy and pyelolithotomy for right renal stones 2 and 10 years ago. 5 Ugurel et al. (2005) 54 Ankara, Turkey Right L3 Multiple episodes of right flank pain for ten Normal years, lithotripsy for right renal stones 4 years ago 6 Gupta et al. (2011) 37 Bhopal, India Left L3 Left flank dull pain for 6 months Normal 7 Punit Tiwari (2011) 55 Kolkata, India Right L3 Recurrent right flank pain (Dull, aching and Normal occasionally colicky) for 2 years 8 Current case 27 Current case Left L2/3 disc Left flank dull pain for 6 months, single episode of gross hematuria 4 months ago Normal The cases are numbered according to the literature reviewed 1 8. L = Lumber spine). Table 2 Imaging work up. Intravenous urography (IVU) Retrograde ureterogram Ultrasound Cross sectional imaging: CT/MRI 1 Right hydroureteronephrosis to the No intrinsic ureteral obstruction Not performed Not performed level of L4 intervertebral disc suggesting retrocaval ureter 2 Moderate hydronephrosis possibly Suggested Ureteropelvic junction Not performed Not performed due to abnormal vessels or Ureteropelvic junction obstruction obstruction 3 Not available Not available Not available Not available 4 Partial obstruction of right ureter at L3 level with proximal dilatation. Upright view suggested extrinsic compression 5 Partial obstruction at Ureteropelvic junction 6 Left side mild hydronephrosis and proximal hydroureter 7 Findings suggestive of retrocaval ureter Not performed Not performed CT: Right testicular vein indenting over right ureter anteriorly resulting in proximal ureteral dilatation Not performed Dilation of pelvicalyceal system and upper ureter proximal to an abrupt narrowing at L3 vertebral level. Jet effect seen Showed the level of obstruction with no intrinsic ureteral obstruction Mild persistent right hydronephrosis Mild to moderate left hydronephrosis and dilatation of proximal ureter suggestive of upper ureteral obstruction Right hydronephrosis and dilated proximal ureter 8 Not performed Not performed Left side grade I hydronephrosis and proximal hydroureter CT: Right testicular vein crossing over right ureter anteriorly with proximal hydroureteronephrosis CT: Anterior compression of left ureter by left testicular vein crossing- anterior to left ureter resulting in left hydronephrosis and proximal hydroureter MRI: Dilatation of proximal ureter with a sharp angulation and cutoff just proximal to normal running right spermatic vein CT: Left testicular vein indenting over proximal ureter at the level of L2/3 intervertebral disc and resultant proximal ureteral and renal pelvis dilatation

3 H. Hamidi / The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) hydroureteronephrosis, diagnostic workup, rules of various imaging modalities, treatment approaches and lastly their prognosis. 3. Results The clinical presentation and demographic characteristics of the patients are presented in Table 1. Seven cases were reported in English literature about this very unusual entity till December 2016; the first case reported in 1975 and the last in Four of the cases involved the right side and four the left side (including the present case). In three cases, pathologically altered testicular vein resulted in obstructive uropathies. While in remaining five cases, a normal testicular vein was indenting over the ureter anteriorly at the crossing point Case 1 The first case of an enlarged right spermatic vein with atypical course resulting in hydroureteronephrosis was reported by Hans-Eberhard et al. in a 28-year-old man in Transection of the obstructing spermatic vein released the obstruction [1] Case 2 The first case of left side hydronephrosis due to compressed left upper ureter by thrombophlebolitic testicular vein was reported by Ronald Kretkowski and Navin Shah in a 20-year-old male in 1977 [3]. Imaging workup suggested left ureteropelvic junction blockage (Table 2). During surgery, a normal ureteropelvic junction was noted however thrombophlebitis of left testicular vein was observed resulting in pressure on and inflammatory adhesions over the ureter resulting in obstruction of the upper third of ureter [3] Case 3 The third case was reported by Lassing et al. in 1978 in a young adult where pathologically altered left testicular vein resulted in proximal hydroureteronephrosis. The compressing testicular vein was transected surgically Case 4 Meyer et al. described the conventional axial computed tomography (CT) findings of ureteral obstruction caused by compression of the right testicular vein for the first time in a 42-year-old man in 1992 [4,5]. Hydroureteronephrosis caused by intermittent obstruction of the ureter by testicular vein was believed to contribute to prior history of stone formation [5] Case 5 Multidetector computed tomography features of a case of right testicular vein compression over right ureter with resultant hydroureteronephrosis was first described by Ugurel et al. in a 54-year-old man in 2005 [6]. Intravenous contrast-enhanced CT images revealed compression of right testicular vein over right ureter with resultant moderate proximal hydroureteronephrosis [6] Case 6 Besides reviewing the previously published 5 cases, Arvind Nand Kishore et al. reported the sixth case of TVS and the first being successfully treated with the laparoscopic approach in a 37-year-old man in 2011 [7]. Fig. 1. (A F) Split-bolus contrast-enhanced axial CT sections through the course of ureters and testicular veins. (A) Going from the fifth lumber vertebrae (L5) ahead toward first lumber vertebrae (F), the course of bilateral ureters (White open arrows) and testicular veins (Red open arrows) can be followed. The right testicular vein drains into inferior vena cava at L2/3 intervertebral disc level (C). The left testicular vein compresses the left ureter at crossing point at L2/3 intervertebral disc level (C). Left ureter proximal to this point is dilated with dilatation of renal pelvis (D F). The left testicular vein drains to left renal vein (LRV) after confluence of renal hilar veins (F) Case 7 Punit Tiwari et al. reported right side TVS depicted with magnetic resonance urography (MRU) and treated with the laparoscopic approach in a 55-year-old man in 2011 [8].

4 1128 H. Hamidi / The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) Fig. 2. MPR sagittal oblique image at the level of compression of left testicular vein (white arrow) over the left ureter (Red arrow) Case 8: (Current case) Authors present the 8th case of testicular vein syndrome in a 27-year-old male with dull and sometimes sharp left flank pain for last 6 months. The patient had a single episode of gross hematuria 4 months ago. Frequent ultrasound examinations reported left side mild to moderate hydronephrosis and proximal hydroureter without any sonographically detectable cause. Plain abdominal radiograph could not reveal any radiopaque stone at the course of the left ureter. Laboratory examinations were unremarkable. The patient underwent CT with 128 slice Siemens scanner. CT images revealed the left side grade I hydronephrosis with proximal hydroureter (Fig. 1) to the level of the L2/3 intervertebral disc where extrinsic compression by normal-sized left testicular vein was observed. The left testicular vein had aberrant course anterior to the left ureter and draining on the superior aspect of left renal vein (Figs. 2 and 3). No other apparent abnormality was noted in the left testicular vein. No abnormal dilatation or thrombosis of left testicular vein was noted. As the case was less severe, the patient was treated conservatively and until 6 months of follow-up. No increase was noted neither in the grade of hydronephrosis nor in clinical symptoms. 4. Discussion Ureteral obstructions at the point of intersection of the ureter with retroperitoneal vessels commonly a dilated or aberrant ovarian vein (ovarian vein syndrome) and very rarely, a testicular vein (testicular vein syndrome) is a known entity [2]. The obstructive uropathy caused by the ovarian vein: (ovarian vein syndrome: first described in 1964) [7] is right sided in about 95% of cases and is believed to have resulted from compression of a dilated, aberrant or thrombosed ovarian vein over the right ureter at the pelvic brim [2]. When Kretkowski et al. [3] reported the second case of obstructed left ureter by dilated thrombophlebitic left testicular vein. They suggested the testicular vein Fig. 3. Virtual Rendered Technique (VRT) reconstructed image of left kidney and ureter. The narrowed portion of the ureter down from the ureteropelvic junction is seen. syndrome as the male counterpart of the ovarian vein syndrome [3,7] however, these are not similar entities. The ovarian vein syndrome is usually right sided, often occurs in pregnancy and is associated with pathologic changes in the ovarian vein (usually dilated and thrombosed vein) [6] whereas, the reported cases of TVS in the English literature, show no preference for the site of pathology (Four cases (50%) are right-sided and four cases (50%) -including present case- are left sided). Pathological changes of the testicular vein are also not obligatory features as were previously assumed [4] Number, course and size of testicular veins Testicular vein or spermatic veins are paired veins that originate at the level of the inguinal canal from a pampiniform plexus and drain the testes in males [9,10]. They ascend along

5 H. Hamidi / The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) the iliopsoas muscles anterior to the ureters on either side [10]. The right testicular vein runs obliquely while its left counterpart has a more vertical course [6,7]. Both vessels cross anterior to ipsilateral ureters at the level of the third lumbar vertebra [6,7]. In a study on the anatomy of testicular veins in 100 adult male cadavers Luciano A. Favorito et al. found out in 85% of cases there was single right testicular vein while in 15% of cases double right testicular veins were detected. 99% of single right testicular veins were draining into Inferior Vena Cava (IVC) and 1% to right renal vein. Left side single testicular vein was observed in 82% of the cases and multiple (2, 3, and 4) in 18%. Drainage of all left testicular veins was to the left renal vein in all cases, independently of the number of testicular veins [11]. In another study on evaluation of testicular vein anatomy in 101 male patients, Taylan Kara et al. could visualize all the testicular veins with multidetector computed tomography. The single right testicular vein was present in 99 (98%) patients and double veins in 2 (2%) patients. The Right testicular vein drained into IVC in 88 (87.1%) patients and right renal vein in 13 (12.9%) patients. On the left side, the single testicular vein was noted in 88 (87.1%) patients and duplicated in 13 (12.9%) patients all of which were draining into the left renal vein. [9] They reported the mean diameter of right testicular vein as 3.5 mm, 3.1 mm and 2.9 mm at proximal, middle and distal parts, respectively. The mean diameter of left testicular vein was 3.6 mm, 3.2 mm and 3.0 mm, at abovementioned parts, respectively [9] Clinical perspective The signs and symptoms are related to the severity of ureteral obstruction. Flank pain is the main complaint and can present as dull, aching, or occasionally colicky [1,5 8]. Hematuria (whether gross or microscopic) is also reported as presenting symptom [3,5,8]. Two patients had a history of nephrolithiasis probably due to stasis [5,6]. No varicocele was reported in any of the cases Imaging work up Intra-Venous Urography (IVU) as the classic imaging investigation of upper urinary tract abnormalities is unable to diagnose this entity, however, can give indirect clues and suggest further imaging workup (Table 2). Depending on the level of ureteral obstruction it can resemble ureteropelvic junction obstruction [3,6], retrocaval ureter [1,8] or suspect extrinsic compression [5]. Ultrasound is also inconclusive and depicts similar findings [6 8]. Retrograde ureterogram can exclude intrinsic obstruction [7,8], determine the level of obstruction [7,8] or suspect ureteropelvic junction obstruction [3] however, it is not part of routine investigations nowadays. CT is the modality of choice that not only excludes intrinsic obstruction but also evaluates extrinsic compressions and any abnormality in the vascular structures. Multi planner reconstructions (MPR) and three-dimensional Volume Rendering Techniques (VRT) can well depict different aspects of these entities [2]. As per clinical setting, CT urography can be performed with unenhanced, nephrographic and excretory phase scans whereas Ugurel et al. additionally emphasized the importance of arterial and venous phases images (especially venous phase) in the detection of vascular causes of obstructive uropathy in suspected cases [6,7]. These scans can be performed singularly or combined, but obviously, they are important in the diagnosis and treatment strategy of vascular compressions causing extrinsic ureteral obstructions [7,12]. In our department, we usually do single post-contrast scan (split-bolus technique; 30 ml and 50 Ml) 5 min after and s after dual contrast injection and usually, the vascular structures can still be visualized. The advantage of the split-bolus technique is the reduction of the radiation exposure and avoiding scan repetition. Magnetic resonance features of TVS are also described [8]. Findings are similar to CT imaging with the advantage of its radiation free nature Treatment and prognosis The therapeutic approach can be selected according to the severity of the obstruction. For low-grade obstruction or in asymptomatic cases, no or conservative treatment may be chosen [5,6]. Surgical intervention (laparotomy or laparoscopy) is indicated in symptomatic cases or when moderate or severe hydronephrosis is present. Transection of testicular vein at crossing point over ureter [1,4,7], excision of involved segment of testicular vein with complete ureterolysis (in case of periureteric adhesions) [3] Table 3 Treatment approach and prognosis. N Type of treatment Surgical findings Treatment approach Prognosis/follow up 1 Surgery Ureter anterior to IVC, Ureteral obstruction at crossing point of the testicular vein which run lateral and cephalad again behind the renal pelvis, draining either into right renal vein or IVC 2 Surgery Impingement of upper ureter by large, dilated, tortuous, thrombosed left testicular vein just distal to ureteropelvic junction. Fine adhesions between the vein and ureter Transection of vein at the crossing point Excision of involved segment of testicular vein and complete ureterolysis of proximal ureter IVU after 4 months: Diminished hydroureteronephrosis. Patient improved clinically but still mild discomfort of same nature as preoperatively persisted IVU after 5 months: Marked decline of hydronephrosis and normal appearance of ureteropelvic junction 3 Surgery Transection of obstructing testicular vein 4 No treatment Spontaneous relief or symptoms. Follow up with scintigraphy 5 Conservative Followed up with ultrasound and scintigraphy 6 Laparoscopy Although the compressed segment of the ureter appeared free of intrinsic obstruction, it appeared atretic 7 Laparoscopy Markedly dilated ureter anterior to IVC. Ureteral obstruction at the crossing point behind testicular vein 8 No treatment Ligation of testicular vein Ureteroureterostomy and excision of atretic segment of ureter IVU after 3 months showed just mild fullness of the left pelvicalyceal system At 4 months of follow-up, a radionuclide renal scan showed adequate function and excretion of the tracer Resection of testicular vein Completely relief of symptoms but IVU after 3 months showed residual hydroureteronephrosis No increase in the grade of hydronephrosis nor in clinical symptoms until 6 months follow up

6 1130 H. Hamidi / The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) ligation of testicular vein and ureteroureterostomy with excision of atretic segment of ureter [7] are the therapeutic approaches performed in reported cases according to the pathologic processes (Table 3). Treatment outcomes were positive in reported cases (Table 3). The hydroureteronephrosis relieved in the majority of cases [1,3,7] but in one case, the patient still complained of mild discomfort of the same nature as was present preoperatively [1]. Another patient with laparoscopic resection had complete relief of symptoms but 3 months later, follow-up IVU illustrated residual hydroureteronephrosis [8]. 5. Conclusion Compression of the ureter by testicular vein is an extremely rare cause of obstructive uropathy in male patients with only seven cases reported in English literature and the 8th being presented in the current article. Testicular vein syndrome should be regarded as the diagnosis of exclusion and may be suspected in male patients with hydronephrosis and proximal hydroureter [6,7]. Ultrasound and IVU findings are inconclusive, while CT and MRI can well depict this entity and guide therapeutic workup. Conflict of interest The authors declared that there is no conflict of interest. Acknowledgment To Abdul Basir Shahin CT radiographer of FMIC radiology department for providing the CT images. References [1] Mellin H-E, Madsen PO. Obstruction of ureter by abnormal right spermatic vein. Urology 1975;6(4): [2] Lamba R, Tanner DT, Sekhon S, McGahan JP, Corwin MT, Lall CG. Multidetector CT of vascular compression syndromes in the abdomen and pelvis. Radiographics 2014;34(1): [3] Kretkowski R, Shah N. Testicular vein syndrome: unusual cause of hydronephrosis. Urology 1977;10(3): [4] Lassnig H, Frick J. Left spermatic vein syndrome. Eur Urol 1978;4(2):141. [5] Meyer JI, Wilbur AC, Lichtenberg R. Ureteric obstruction by the right testicular vein: CT diagnosis. Urol Radiol 1991;13(1): [6] Ugurel MS, Ilica AT, Kantarci F, Kocaoglu M, Somuncu I. Obstructive uropathy caused by testicular vein compression: multidetector-row computed tomography findings. J Comput Assist Tomogr 2005;29(5): [7] Arvind NK, Singh O, Gupta SS. Testicular vein syndrome and its treatment with a laparoscopic approach. JSLS: J Soc Laparoendosc Surg 2011;15(4):580. [8] Tiwari P, Tiwari A, Kumar S, Bera M. An unusual cause of ureteral obstruction role of magnetic resonance imaging. Urology 2011;78(4): [9] Kara T, Younes M, Erol B, Karcaaltincaba M. Evaluation of testicular vein anatomy with multidetector computed tomography. Surg Radiol Anat 2012;34 (4): [10] Karcaaltincaba M. Demonstration of normal and dilated testicular veins by multidetector computed tomography. Jpn J Radiol 2011;29(3): [11] Favorito LA, Costa WS, Sampaio FJ. Applied anatomic study of testicular veins in adult cadavers and in human fetuses. Int Braz J 2007;33(2): [12] Huwyler M, Willmann JK, John H. Functional ureteral obstruction due to complex pelvic venous anomaly. Urol Int 2007;79(3):284 6.