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Diagnosis and F이low-Up of a Case of Nutcracker Syndrome with MR Angiography

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Diagnosis and F이low-Up of a Case of Nutcracker Syndrome with MR Angiography 대 한 방 사 선 의 학 회 지 1993 ; 29 (3) : 426~429 Journ al of Korea n Radiological Society, Ma y, 1993 Diagnosis and F이low-up of a Case of Nutcracker Syndrome with MR Angiography Gwy Suk Seo, M.D., Hyo Keun Lim, M.D., Sang Hoon Bae, M.D., Kyung Hwan Lee, M.D., Dong Wan Chae, M.D.*, Hong Rae Cho, M.D.**, Ku Sub Yun, M.D. Departmeη t 01 Diagnostic R adiology, Hallym Uηzveγsi ty College 01 Mediciηe - Abstract - A case of nutcracker syndrome which was initially diagnosed by magnetic resonance angiography (MRA) is reported. On preoperative MRA in an 18-year-old male patient with gross hematuria, left renal vein was oblit­ erated at the level of superior mesenteric arteη and there was no connection with inferior vena cava. The fol­ low-up MRA after surgical coπection with external prosthesis demonstrated entire course of left renal vein without evidence of obstruction which might suggest a possible usage of MRA for a non-invasive diagnosis of nutcracker syndrome. Index Words: Magnetic resonance angiography 966.1299 Renal vein, stenosis 966.78 So-called the nutcracker syndrome, which We described a patient who had a clinical shows variable degree of hematuria mairùy be­ finding of nutcracker syndrome and the m ‘ jor cause of compression of the left renal vein be­ role of MRA in the diagnosis and the postoper­ tween abdorninal aorta and superior mesenteric ative follow-up. artery, is quite a nuisant disease entity in terms of imaging diagnosis (1 -3). Although left renal CASE REPORT venogram with pressure measurement was re­ garded as a g이d standard of the diagnosis, A 18-year-old male patient who had a re­ arteriogram or ultrasonogram is now regarded current massive gross hematuria for six months as a helpful, but no longer a valuable diagnostic was adrnitted. He was very thin and pale-look­ modality in the disease (1 -7). With the recent ing. His hematocrit level was fluctuating. Cysto­ development of Magnetic resonance angiogra­ scopic exarnination revealed the blood jet from phy (MRA), renal artery has become a good ob­ the orifice of left ureterovesical junction. ject for non-invasive imaging and in addition, Under the light rnicroscopic exarnination of the renal vein has been challenged for non-invasive urine red blood cell (RBC), the fraction of evaluation (6 ,8-10). dysmorphic RBC was less than 10% * 한림대학교 의과 대 학 내 과 학교실 * Depaγtmeη t 01 Internal Medicine, Hα， Uym Universψ College 01 Me diciη e ** 한럼대학교 의과대학 외 과학교실 ** Departmeη t 01 General SU1ge1Y, Hallym University College 01 Mediciηe 이 논문은 1 993 년 1 월 5 일 접수하여 19 93 년 2 월 1 8 일에 채택되었음 . Received January 5, Accepted February 18, 1993 - 426 - Gwy Suk Seo, et al : Diagnosis and Fo ll ow-up of a Case of Nutcracker Syndrome with MR Angiography The MRA was performed by way of two-di­ During the procedure of conventional ve­ mensional time- of-f1ight method using F1SP nography the advance of the catheter into the (fast image steady precession) with partial satu­ distal portion of left renal vein was very diffi­ ration of aortic flow at the level of the dia­ cult and even with a guidewire. On venogram, phragm on a 1.5T superconductive magnet almost no backflow to the distal renal vein was (Siemens, Erlangen, Germany). The scan para­ noted even though pressure injection of con­ menters are as follows. TRπE was 34j8msec trast media was done. The pressure gradient and flip angle was 40 degree and field of view across the narrowed portion was 3mmHg. On was 350mm. On oblique view of MRA, the angle between the long axis of superior mesen­ teric artery and abdominal aorta was quite acute. The portion of left renal vein at the level of abdominal aorta and superior mesenteric arteπ was not visualized (Fig. 1). 1n contrast, right renal vein was visualized through the en­ tire length. Fig. 2. Conventional arteriogram on venous phase shows extrinsic compression of left renal vein (arrow). Fig. 1. Superior mesenterÏc arteη draws an acute Fig. 3. Axial image of post-operative spin echo MR angle with aorta, and focal non-visualization of left (Tl weighted) shows that external stent (arrowheads) renal vein (arrow) is noted at compression site on pre was precisely located. -operative 2D MRA. 낌 Journal of Korean Radi 이 ogical Society 1993; 29 (3) : 426~429 venous phase of arteriogram, smooth indenta­ tion and poor filling of left rena1 vein were DISCUSSION noted (Fig. 2). The patient continued to bleed. And sur멍­ Since the initia1 report by De Schepper in cal intervention was tried. On operative field, 1972, nutcracker syndrome has now got a wide left renal vein was entrapped between abdomi­ acceptance as a cause of unexplained hematuria nal aorta and superior mesenteric artery. A (1 1). It is suspected when a non-obese person ready-made stent (with Goretex, 14mm in di­ has a recurrent gross hematuria and blood jet ameter, 5cm in length) was inserted in the com­ is noted from the left uretera1 orifice. Usu떠 ly pressed vein extern떠 ly. Follow-up MR (Fig. 3) the microscopic morphology of urine red blood and MRA (Fig. 4) showed decreased proximal cell is not of glomerular origin as our case, that dilatation with a good visua1ization through the is, the portion of dysmorphic red blood cell entire length of left renal vein. And the follow should be less than 20%. Measurement of pres­ up conventional angiogram a1so showed paten­ sure gradient across the obstruction site is the cy of left renal vein. The patient has been only method for the confirmation of overa11 doing well for the postoperative six months. agreement, because the imaging diagnosis is not sufficient. Noninvasive modalities such as sonography or CT were tried for this purpose, but there were linútations, and recently MR was performed and it direct1y showed tortuous col­ latera1 vessels through the noninvasive way (6 , 7). Rapid progress in MRA in recent severa1 years has foreseen possible selective rena1 veno­ gram (7-10). On an멍ogram ， left rena1 vein is not visu떠­ ized due to obstruction by extrinsic cause and instead retroperitonea1 collatera1 veins are illed, and sonogram or CT shows non-specific finding such as ca1iber discrepancy of pro잉ma1 and dista1 part of left rena1 vein or acute angu­ lation of superior mesenteric arteη and aorta (6,7,12-14). The MRA of our case which showed obliteration of the left rena1 vein and postoperative improvement on fl이 low-up study can be a positive indicator of this uncommon disease entity. For the management of the se­ vere cases, various surgica1 technques such as nephrectomy, reimplantation of left rena1 vein or externa1 stenting of left rena1 vein has been tried as a new method (3 ,11). At first we con­ sidered insertion of intravenous stent for the Fig. 4. Post-operative follow-up MRA shows im­ relief of obstruction but the risk of venous provement of the renal venous drainage (arrow) into thrombosis and technica1 problem discouraged inferior vena cava us. So we tried an externa1 stent application to - 428 Gwy Suk Seo, et al : Diagnosis and Follow-up of a Case of Nutcracker Syndrome with MR Angiography the collapsed renal vein instead of more aggres­ nomenon. 페R 1990; 154:305-306 sive surgeIγ and have got a good result. A맹in ， 7. Kim SH, Kang ]W, Kim WS , Yi ]G, Park ]H, MRA played an important role in postoperative Kim CW. Anterior-posterior diarneter of left evaluation which was well correlated with con­ renal vein in normal and nutcracker sy띠rome. ] ventional angiogram, If more experiences accu­ Korean Society of Med Ultrasound 1990; 9 (2): mulate, the role and characteristics of MRA in 127-130 nutcracker syndrome will be more darified. 8. Chien D, Edelman RR. Basic principles and clini­ cal applications of magnetic resonance angiogra­ phy. Seminars in Roentgen 1992; 27:53-62 REFERENCES 9. Kent KC , Edelman RR, Kim D, Steinman T1 , Porter DH, Ski1man]J. Magnetic resonance im­ 1. Weiner SN, Bemstein RG, Morehouse H , Gold­ aging: A reliable test for the evaluation of proxi­ en RA. Hematuria secondary to left peripelvic mal atherosclerotic renal arterial stenoss. ] Vasc and gonadal vein varices. Urology 1983; 22 (1): Surg 1991; 13:311-318 81-84 10. Debatin ]F, Spritzer CE, Grist TM, et al. Imag­ 2. Hohenfellner M, Steinbach F. Schultz-Larnpel ing of the renal arteries: value of MR an밍O망 a­ D. et al. The nutcracker syndrome: new aspects phy. A]R 1991; 157:981-990 of pathophysiology, diagnosis and treatment. ] 11. Wendel RG, Crawford ED, Hehman KN. The Urology 191; 146:685-688 “ nutcracker" phenomenon: an unusual cause for 3. Bames RW, Fleisher 111 H, Redman ]F. Smith renal varicosities with hematuria. ] Urology ]W, Harshfield DL, Ferris 페. Mesoaortic com­ 1980; 123:761-763 pression of the left renal vein: repair by a new 12. Cope C, Isard HJ. Left renal vein entrapment. a stenting procedure. ] Vasc Surg 1988; 8:415- new diagnostic finding in retroperitoneal disease. 421 Radiology 1969; 92:867-872 4. Park YS , Lee CY, ]in DK, Cheong HI, Choi Y. , 13. Beckmann CF, Abrams HL. Circumaortic venous Ko KW. Nutcracker syndrome: Report of a case. ring: Incidence and significance. A]R 1979; 132: Korean] of Nephrology 1989; 8 (2):136-140 561-565 5. 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