Iliac vein compression cause of varicocele. syndrome: An unusual

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1 Iliac vein compression cause of varicocele syndrome: An unusual M. David Bomalaski, MD, Joseph L. Mills, MD, Luis R. Argueso, MD, Roy M. Fujitani, MD, Alvin L. Sago, MD, and Allen E. Joseph, MD, Lackland AFB, San Antonio, Texas Iliac vein compression syndrome is the phenomenon of nonthrombotic iliac vein obstruction caused by compression of left iliac vein between the right iliac artery and fifth lumbar vertebra. Affected patients usually present with unilateral leg edema. The condition is most often seen in women, in whom it may also be a cause of vulvar varieosities. Presented here is a case of idiopathic iliac vein obstruction associated with an ipsilateral varicocele in a young man. This varicocele was caused by multiple collateral venous channels and was resistant to surgical high ligation. This case illustrates an unusual cause of varicocele formation and an anatomic reason for failure of standard surgical therapy. (J VAsc SURG 1993;18: ) Compression of the left common iliac vein between the right common iliac artery and fifth lumbar vertebra is a recognized cause of nonthromboric venous obstruction of the left leg. The condition has been given the eponyms May-Thurner syndrome and Crockett syndrome, and the descriptive terms venous spur and venous compression syndrome. It usually presents with venous insufficiency and left lower extremity edema and may mimic deep vein thrombosis. It can cause chronic leg pain and edema and has been implicated in chronic pelvic pain in women? Some autopsy series have reported that up to 32% of the population may have bands or adhesions noted at the level of the left iliac vein where it is crossed by the right iliac artery? This results in clinically significant obstruction in only a small number of patients. Review of the literature shows that clinically significant obstruction is five rimes more common in women, 3 in whom it may be associated with vulvar varicosities. 1 Although the overall incidence ofvaricocele in the male population is 15%, the incidence of infertility in the male population is only approximately 5%. 4 Therefore many men with varicocele remain fertile. However, in the infertile male population, ligation of From the Departments of Urology, Vascular Surgery, and Radiology, USAF Medical Center Wilford Hall, San Antonio. The views expressed herein are those of the authors and do not reflect the official policy of the Department of Defense or other depamnents of the U.S. Government. Reprint requests: David Bomalaski, MD, Departtnent of Urology, USAF Medical Center Wilford HalI/SGHSU, Lacldand AFB, TX /4/ an associated varicocele will restore fertility in up to 50% of patients, and it remains the number one surgically correctable cause of infertility in men. s Because the adverse effects ofa varicocele are thought to be cumulative, if there is a discrepancy in testicular size or if the varicocele is symptomatic with pain or discomfort, ligation of the varicocele in the adolescent is indicated. Presented here is a case of left-sided iliac vein obstruction that presented as a symptomatic varicocele in a 12-year-old male patient. CASE REPORT A 12-year-old boy presented with the complaint of a tender, heavy feeling of gradual onset in his left hemiscrotum. History showed only the usual childhood diseases with no other medical or physical problems. Physical examination results were entirely normal with the exception of a large left varicocele. Testicular size and parenchyma were normal on examination. Urine analysis and culture results were unremarkable. Examination of the patient showed no leg edema or varicose veins. The patient underwent spermatic vein ligation via an inguinal approach. After operation the patient was noted to have a persistent, symptomatic left varicocele unchanged by physical examination. There continued to be no other physical complaints or findings. One year later, at the age of 13, the patient underwent a repeat spermatic vein ligation via a high retroperitoneal approach. After operation the patient again had no change in the physical appearance of his varicocele. He continued to have episodic scrotal discomfort and a heavy sensation, but he was in no way limited in his physical activities. Three years later, at the age of 16, the patient was again admitted for correction. He had initially presented with a large grade III varicocele. Over the course of several years

2 JOURNAL OF VASCULAR SURGERY Bomalaski et al Volume 18, Number 6 he was monitored by different providers, all of whom documented the same results. The patient stated that he had noted no change in appearance or symptoms since his first surgery. Evaluation showed the varicocele as his only abnormal physical finding. Semen analysis showed 33 million sperm/ml, with 48% motility and 54% normal forms. Serum testosterone, follicle stimulating hormone, and luteinizing hormone levels were all within normal limits. After discussion with the patient and his family, it was elected to attempt percutaneous embolization of what was presumed to be persistent spermatic veins. A left renal venogram showed retrograde filling of the proximal portion of the spermatic vein that ended abruptly, which should be found after successful high ligation of the spermatic vein. No patent collateral vessels were evident at this level. Fig. 1 is a venogram of the left common iliac vein obtained via a right transfemoral approach. Obstruction of the vein was noted at the point where it is crossed by the right lilac artery. The angiographer was able to pass the catheter past the point of obstruction and obtain views of the iliac vein distal to the obstruction. Multiple collateral channels were observed, some of which communicate with the scrotal varicosities. The venogram has been labeled to show both the site of obstruction (open arrowhead) and the dilated scrotal collateral channels. Many of these collateral channels are unnamed. A large dilated external spermatic vein draining from the external iliac is labeled (closed arrowhead). The diagnosis of ihac vein compression syndrome or May-Thurner syndrome was made and the procedure was terminated at this point with no attempt at embolization. Currently the patient is being carefully monitored with no change in his physical condition. DISCUSSION Iliac vein compression has been recognized since the turn of the century, and various authors have described syndromes of associated venous insufficiency. 2 The exact prevalence and degree of disability associated with such compression is unknown. Autopsy studies have confirmed the presence in the iliac vein of asymptomatic intraluminal webs and adhesions as a normal anatomic variant. 6 It is believed that significant morbidity occurs in only a minority of these patients and that most patients remain symptom free because of adequate collateral circulation. Those that have symptoms have them in adult life when they present with signs of venous insufficiency and leg edema. Once venous insufficiency begins to appear most authors recommend surgical correction with either saphenofemoral vein crossover bypass, 2 or patch angioplast3p with or without transposition of the vein anterior to the artery. 7 Blood flow from the testicle has classically been Fig. 1. Venogram of left common iliac vein. Point of obstruction is seen at level where iliac vein is crossed by right iliac artery (open arrowhead). Multiple collateral venous channels are seen including large external spermatic vein (closed arrowhead). divided into testicular, cremasteric, and vasal. 4 After internal spermatic vein tigation varicocele persistence is often due to collateral channels from one of these systems. Venographic studies in men with varicoceles have shown a number of collateral channels to include anterior and posterior scrotal veins draining into the femoral system, the external spermatic vein that drains into the external iliac vein, the ductus deferens that drains into the internal iliac vein via vesical collateral channels, and prepubic and transcrotal collateral channels from the left to the right side: 8 These collateral networks became significant in our patient as both a reason for failed varicocele ligation and as a potential route of venous drainage of the left leg. The most common cause of a varicocele is believed to be incompetence of the internal spermarie vein, which inserts on the left side at a

3 1066 Bomalaski et al. IOURNAL OF VASCULAR SURGERY December degree angle into the renal vein and on the right inserts at an oblique angle into the inferior vena cava. This difference in insertion is believed to be the reason that varicoceles occur much more frequently on the left. Another cause is the nutcracker effect. This refers to the compression of the left renal vein between the aorta posteriorly and the superior mesenteric artery anteriorly, resulting in elevated venous pressures in the left renal vein and internal spermatic vein. Coolsaet 8 discovered in 1980 the so-called distal nutcracker effect with compression of the left common iliac vein by the left common illac artery as it crosses the vein. This induces collateral pelvic circulation from the left iliac system to the right, some of which involves the scrotal veins. We believe our patient had a varicocele on the basis of such an effect. Other pathologic causes of a varicocele have been identified. It may represent tumor or fibrosis in the retroperitoneum with obstruction of the inferior vena cava or spermatic veins. 9 Such varicoceles are often sudden in onset, involve the right side, and do not decompress in the supine position. Varicoceles are graded clinically from I to III. A grade I varicocele is small in size and palpable but not visible. A grade II varicocele is moderate in size, palpable, and visible. A grade III varicocele is large and readily visible. In addition to this, duplex studies can detect nonpalpable subclinical varicoceles, the significance of which has yet to be determined. There are two commonly performed approaches to spermatic vein ligation, s The retroperitoneal approach involves division of the internal spermatic vein via a muscle-splitting incision made medial to the anterior superior iliac spine. It is argued that the testicular artery may be safely divided at this level because of cremasteric and vasal arterial collateral channels. Its disadvantage is that distal venous collateral channels contributing to the varicocele may be missed. The other commonly used approach is inguinal. With this method an incision is made over the inguinal canal with isolation and ligation of the internal spermatic vein and collateral channels at this level. This approach allows individual ligation of the external spermatic, pampiniform veins, and ductus deferens veins. Its disadvantage is that the testicular artery and lymphatics are more susceptible to injury at this level. Percutaneous catheterization with embolization of the internal spermatic vein has also been advocated because it serves as both a diagnostic and therapeutic method. Technical expertise necessary to perform this procedure is not available at all hospitals. There is also the risk of embolus migration and inadvertent pulmonary embolization. A new technique being developed is laparoscopic ligation of the internal spermatic vein just proximal to the internal inguinal ring. This is especially useful in decreasing postoperative morbidity in the case of bilateral varicoceles. Its exact role in unilateral varicosities has yet to be determined. Routine preoperative venography has been advocated by some authors to better define the cause of a particular patient's varicocele. 8 Others have argued that this is neither cost-effective nor necessary, especially when the inguinal approach to ligation is used. l Intraoperative venography is practiced by some, but persistent varicocele can still be encountered even after negative intraoperative venography results. 11 A more reasonable approach, as demonstrated by this case, would be venographic evaluation of any patient who has had a failed previous internal spermatic vein ligation. This patient is remarkable for several reasons. He presented as an adolescent male without signs ofiliac venous obstruction except for his varicocele. Symptomatic iliac vein obstruction is extremely rare in the pediatric population, with only two reported cases in the literature of presumed congenital webs occurring in two adolescent girls with leg edema. 12 Iliac vein obstruction has been demonstrated to cause vulvar varicosities in women via the external pudendal vein, especially in association with pregnancy) It now seems that it may also be a cause of varicoceles in males for the same reason. As was seen in our patient and represented in Fig. 2, there are collateral venous channels between both lower extremities and both sides of the scrotum. The collateral venous return of the scrotum is shown in Fig. 2. Interestingly the natural history of this entity is unknown. Currently the patient has only minimal symptoms, an acceptable semen analysis result, and a normal testicular examination. Both lower extremities have been carefully examined and compared by several different physicians as part of the decision to merely observe the patient at this time. The thigh measurements are equal bilaterally, and the patient has no demonstrable leg edema or varicosities. He is able to fully participate in sports and all levels of physical activity. The fact that autopsy studies have shown a high incidence of intraluminal iliac vein webs in symptom-free individuals further argues against surgical correction

4 JOURNAL OF VASCULAR SURGERY Bomalaski et al Volume 18, Number 6 ISV I D SV PP Fig. 2. Diagram of pelvic venous anatomy shows left iliac vein being crossed by right iliac artery. Collateral channels ofvaricocele include multiple internal spermatic veins (ISV), anterior, posterior and transcrotal veins (SV), external spermatic vein (ESD, ductus deferens (DD), and prepubical veins (PP). SV with the associated morbidity and uncertain durability of a major venous reconstruction. The options have been fully discussed with the patient and his family, and they agree that the patient should continue to undergo observation. Future treatment of this patient will depend on the development of symptoms. If fertility is an issue and he has a stress pattern semen analysis of oligospermia and decreased motility, division of scrotal collateral veins based on venography could be offered. This may place the patient at higher risk of having symptomatic venous insufficiency in his leg. If venous insufficiency of his left leg develops, then the patient will need either saphenofemoral vein crossover bypass or patch angioplasty with or without transposition of the common iliac vein anterior to the artery. This would also decompress the varicocele but at a higher operative risk than individual collateral vein ligation. The natural history of this patient's disease process is unknown. He may not have either problem and therefore may not need any treatment. He will be closely monitored to detect problems at the earliest possible point. In conclusion iliac vein compression syndrome can be associated with cfinically significant venous outflow obstruction. Its true incidence, natural history, and degree of morbidity in the pediatric population are unknown, because most cases present later in life with lower extremity venous insufficiency. It seems that it may also be a cause of varicocele, which is resistant to standard procedures ofspermatic vein ligation. When confronted with a persistent varicocele despite surgical intervention, the diagnosis of iliac vein compression syndrome should be considered. The optimal treatment of such patients remains to be established. REFERENCES 1. LePage PA, Villavicencio JL, Gomez ER, Sheridan MN. The valvaflar anatomy of the iliac venous system and its clinical implications. J VAsc SURG 1991;14: Erich WE, Krumbharr EB. A frequent obstructive anomaly of the mouth of the left common iliac vein. Am Heart J 1943;26: Taheri SA, Williams J, PoweU S, et al. Iliocaval compression syndrome. Am J Surg 1987;154: Turner Tr. Varicocele: still an enigma. J Urol 1983;129:

5 JOURNAL OF VASCULAR SURGERY 1068 Bomalaski et al. December Stecker 5F. Varicocele. AUA Update Series, 1985;4:1-8, Lesson Akers DL, Markowitz IM, Kerstein MD. Iliac vein bypass with autogenous saphenous vein for iliac vein compression syndrome. Am Surg 1987;53: Lalka SG, Malone JM. Surgical management of chronic obstructive venous disease of the lower extremity. In: Rutherford RB, ed. Vascular Surgery. 3rd ed. Philadelphia: WB Saunders, 1989: Coolsaet BLRA. The varicocele syndrome: venography determining the optimal level for surgical management. J Urol 1980; 124: Belker AM. The varicocele and male infertility. Urol Clin North Am 1981;8: Lipshultz LI. Editorial comment. I Urol 1980;124: Kass EL Evaluation and management of the adolescent with a varicocele. AUA Update Series 1990;9:89-96, Lesson Zahrani HA, Cuschieri RJ. Congenital intraluminal iliocaval webs. Scott Med J 1989;34:469. Submitted Sept. 21, 1992; accepted Jan. 8, 1993.