Pelvic insufficiency: a deeper look at female and male gonadal vein incompetence

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1 REVIEW ARTICLE Pelvic insufficiency: a deeper look at female and male gonadal vein incompetence Tamara Allcorn Cooloola Radiology, Gympie, Queensland, Australia Keywords pelvic, insufficiency, vein, ultrasound, incompetence, gonadal. Correspondence Tamara Allcorn, Cooloola Radiology, Gympie, Queensland, Australia. t.allcorn@bigpond.com Received: 7 March 2014; revised 30 April 2014; accepted 7 May 2014 doi: /sono Abstract Insufficiency within the pelvic veins although a proven clinical entity, is not commonly assessed and is often overlooked and underdiagnosed in women. Insufficiency of the pelvic veins, otherwise known as pelvic congestion syndrome in women, and varicocele in men, can be the cause of increased pain and discomfort for the patient. Insufficiency within the gonadal veins can, when prolonged, lead to an increase in varicose veins within the lower extremities, or in the instance of a patient who has already undergone surgery, these veins may be a strong contributor to recurrence rates. By comparing the similarities between male and female insufficiency, a standardised and more common practice of diagnosing female insufficiency should be implemented. The overall treatment and outcome for the patient, not to mention the long term benefits, especially if leg varicosities are involved as well, will be significantly improved if this condition is diagnosed correctly. Introduction Insufficiency within the pelvic veins, although a proven clinical entity, is not commonly assessed and is underdiagnosed in most instances in women. The male version of pelvic insufficiency, varicocele, in contrast is well documented and diagnosed. 1 Insufficiency within the gonadal veins can, when prolonged, lead to an increase in varicose veins within the lower extremities, or in the instance of a patient who has already undergone surgery, these veins may be a strong contributor to recurrence rates. 1 Insufficiency of the pelvic veins, otherwise known as pelvic congestion syndrome (PCS) in women, and varicocele in men, can be the cause of increased pain and discomfort for the patient. 1 The outcome of the patients treatments is reflected in the accuracy of diagnosis prior to surgery. Anatomy Knowledge of anatomy of the left and right gonadal veins is essential to understanding and diagnosing pelvic insufficiency in both men and women. Within the upper abdomen, the inferior vena cava is located posterior to the duodenum, the head and neck of the pancreas, the lesser Funding: None Conflict of interest: None sac and the liver. 2 The tributary veins that arise off the inferior vena cava (IVC) are the paired lumbar and renal veins and the hepatic veins, Importantly to note, on the right side alone, the right gonadal, suprarenal and inferior phrenic veins drain into the IVC. 2 In comparison with that of the left side where the left gonadal and suprarenal veins join the left renal vein 2 (Figure 1), and the left inferior phrenic veins drain into the left suprarenal vein. 2 Female ovarian vein insufficiency Clinical indications Patients with pelvic insufficiency typically have varied symptoms that are nonspecific that makes diagnosis difficult. 1 Symptoms generally relate to abdominal pains within the left iliac fossa, flanks and hypochondrium, positional pains (occasionally worse when lying down on affected side), cyclical discomfort (often worst shortly before menstruation), during and post-coital pains and urinary symptoms such as infections, hydronephrosis, pyelonephritis, renal colic and frank haematuria. 1 Noticeable varicose veins in the pelvic region are common, particularly in pregnant women, however, commonly go unrecognized. 1 Vulval and peri-vulval veins will often become dilated and distended during pregnancy; 12

2 T. Allcorn Figure 1 Anatomy of the left ovarian vein. Note the ovarian vein joining the left renal vein, compared with the right joining the IVC. however, these will generally return to normal after delivery. In women who have subsequent pregnancies, these veins undergo continued stress and as a result will become more prominent. 3 Obvious peri-vulval varicose veins can be seen to extend over the buttock and down the back of the thigh, whilst vulval dilated veins can be clearly seen medially in the upper thigh. 3 Pathophysiology The first investigation into PCS was conducted by Taylor in 1949 in multiparous women who were describing left-sided pains demonstrating positional variances and post-coital discomforts. 1 The pathophysiological mechanism is still widely discussed and varied. Abnormal positioning of the ovarian vein has been described in the literature as a likely cause of reflux. The left ovarian vein generally crosses the ureter at a level of approximately L3/L4; however, if the vein is positioned more caudally, crossing the ureter at the pelvic brim, then it is likely that compression of the ureter will occur. 1 The ovarian vein in this situation has been generally described as being larger in size because of the increased pressure required to pass over the ureter, thus more likely to result in reflux. 1 Similarly, another congenital abnormality is the number of valves actually present within the ovarian veins. 1 Postmortem investigations have revealed that the left renal vein is twice as likely as the right to be without valves. 1 The most accepted physiology is related to women who have had multiple pregnancies. 3 The pressures placed on the uterus during pregnancy and child birth are extreme. Large increases in the amount of blood required by the uterus results in dilatation of ovarian veins. 3 Persistent, significantly elevated venous pressures are needed in the gravid uterus, and visible vulvar veins can occasionally be seen. 4 Postpartum, the ovarian vein has been noted to collapse following birth; however, during subsequent pregnancies, the already stretched veins begin to become more prominent causing increased pooling of blood in the pelvis. 3 The dilated veins, if under enough pressure, can expand to mildly compress the ureter, resulting in a sense of urgency of micturition as well as heaviness in the perineum, particularly so during menstruation. 3 Another pathophysiological theory is related to hormone levels. The high levels of a combination of hormones during pregnancy particularly oestrogen and progesterone can alter the muscular layers within the veins, resulting in extended relaxation. 1 This relaxation associated with the high demands of blood required to the pelvis could be enough to result in reflux within the vessel. 1 Nutcracker syndrome is an underdiagnosed occurrence that can also result in ovarian vein insufficiency. The left renal vein can be compressed between the superior mesenteric artery and the aorta, resulting in a dilatation of the left renal vein. 4 There is a resulting large increase in pressure within the left renal vein that can cause buildup of pressure within the left ovarian vein, ultimately weakening and distending the vein walls. 3 If there is substantial venous pressure, the vein will dilate and reflux will occur (Figure 2). Measuring and comparing the anteroposterior diameter of the vein as well as the peak velocity flow in both the hilar aspect of the left renal vein and the left renal Figure 2 A 22-year old with evidence of compression of the left renal vein in-between the SMA and aorta suggestive of nutcracker syndrome. 13

3 T. Allcorn vein, which lies between the aorta and superior mesenteric artery, can aid in confirming diagnosis. 5 A ratio greater than 5.0 is generally accepted for both anteroposterior diameter and peak velocity to confirm nutcracker syndrome. 5 It is important to note here that there is a difference between nutcracker syndrome and nutcracker phenomenon. It is possible to see impeded outflow from the left renal vein and to not have any clinical symptoms. It is possible that this variation can be seen in relation to a normal variant or be related to other conditions. 6 The term nutcracker syndrome should be reserved for patients who are demonstrating the associated symptoms such as haematuria and pain. 6 Care must be taken to not apply too much pressure to the abdomen when examining the vessels, as this can result in artefacts and false positives. 6 For this reason, it is ideal, if nutcracker syndrome is suspected, to image the patient in supine and upright positions. 6 Diagnostic testing Ultrasound The majority of initial investigations for pelvic insufficiency begin with pelvic ultrasound. Unfortunately, most PCS is found incidentally. Patients are often referred for an ultrasound for investigation of pelvic pains, coital discomforts and positional pains that because of their general nonspecific symptoms are investigated for pelvic inflammatory disease, endometriosis, inflammatory bowel disease and urinary tract infections. 7 For a normal pelvic organ investigation, a full bladder is recommended; however, although the organs will be seen clearly, the weight of the bladder and the patient in a supine position will force the veins to collapse. 7 Therefore, it is essential that postvoid transabdominal scanning or preferably transvaginal ultrasound be performed to further assess in the setting of clinical suspicion. 7 On transvaginal ultrasound, there are a number of features specific to diagnosing PCS. Within the broad ligament, excessive pelvic varicose veins can be seen. 8 Measurements greater than 7 mm are features suggestive of reflux as well as evidence of reverse flow whilst lying, sitting or standing without augmentation. 7,8 In an ideal setting, it is preferable to assess for PCS by locating and examining the left and right ovarian veins cranially to caudal. 7 Ideally, if the patient is fasted, bowel motility and bowel gas are reduced, enabling a clearer image. It is preferable to start by using transverse sections in the upper aspect of the abdomen and identify the aorta and superior mesenteric artery (SMA). The ideal window is through the left lobe of the liver and pancreas. 7 Here, the left renal vein should be seen coursing in-between the SMA and aorta. Following the left renal vein to approximately half way along its length to where the left ovarian vein should join. 8 If incompetent, the vein is generally easily seen 8 (Figure 3). Consider the transverse aorta as a clock face; in this plane, the ovarian vein should lay at the three o clock position, and by turning the probe longitudinally, the left ovarian vein is able to be followed inferiorly. 7 It is essential to ensure that the left ovarian vein joins the left renal vein. 9 Care must be taken to not mistake any accessory renal veins or the inferior mesenteric vein for the ovarian vein. 9 Retro-aortic left renal veins, duplication of the renal vein or large ureteric veins should be documented. 9 The vein diameter at its confluence should be measured and assessed in colour and pulsed Doppler for flow direction. 9 Less common occurrence of reflux is seen within the right ovarian vein; however, cases have been documented. 9 The right ovarian vein will normally join the IVC approximately 2 to 5 cm distal to the right renal vein. 7 In a patient with a normal, nonrefluxing right ovarian vein, the vein is essentially nonidentifiable. 7 The ideal window is through the liver or gallbladder by following the IVC upward to identify the junction of the right renal vein and IVC. 7 Occasionally, patients lying in a decubitus position, in preference to supine, may assist with visualisation. Distal to this junction, the right ovarian vein enters anterolaterally at a very acute angle. 7 The right ovarian vein, if incompetent, can be seen coursing parallel to the IVC 9 (Figure 4). The diameter of the vein at the confluence should be measured and assessed for reflux with colour and spectral Doppler. The internal iliac vein may also contribute to the reflux thus it is essential in the setting of ovarian insufficiency that this should be assessed for reflux also. The internal iliac veins bilaterally can be found by placing the transducer in longitudinal midway between the umbilicus and the groin on an oblique angle. 9 The level at which the Figure 3 Clearly incompetent large left ovarian vein seen joining the left renal vein. 14

4 T. Allcorn the majority of clinical settings, venography is used when pelvic coiling or ovarian vein embolisation are being considered or just prior to embolisation to confirm ultrasound findings.7 Other methods of investigation Figure 4 An incompetent right ovarian vein joining the IVC. common iliac vein bifurcates is widely variable therefore it may be easier to either follow the common iliac vein distally or the external iliac vein proximally to locate.9 The internal iliac vein drains directly towards the probe and should be easily assessed with colour Doppler.9 Normal internal iliac flow will demonstrate respiratory variations, although, the Valsalva manoeuvre may be useful.9 Incompetent flow will generally be continuous and away from the probe.7 Pitfalls are common with all sonographic examinations, and the ovarian vein scan is not excluded from this. The most common pitfalls are as follows: operator dependent a comprehensive understanding of pelvic anatomy is essential, obtaining a suitable Doppler angle, abdominal gas, patient body habitus, anatomic variations and compression of the veins due to distended bladder.7 Venography Venography is generally accepted as the gold standard in detecting ovarian vein reflux. This method is particularly useful for clear identification of all varicose veins as it demonstrates retrograde flow.4 A venogram from the mid portion of the left renal vein is generally performed during Valsalva manoeuvre that should illustrate renal vein patency and show gonadal vein incompetence.4 Further to this, if the catheter is advanced into the gonadal vein, this second injection can confirm the presence of any collateral vessels. Occasionally, a third injection is needed to assess the mid vein and distal segments.4 Generally, ovarian veins measuring over 10 mm in diameter are accepted as incompetent using this method.1 In Contrast enhanced computed tomography as well as magnetic resonance imaging can both be used to detect enlarged and tortuous vessels. In both settings, it is considered that an ovarian vein over 8 mm is incompetent.1 Exploratory laparoscopy is not generally performed as an investigation for incompetent veins, although dilated vessels may be noted incidentally. It has been suggested that during surgery, if the patient s head is tilted up, the ovarian and broad ligament veins will be seen to rapidly distend if reflux is present.7 Internal spermatic vein (or testicular vein) insufficiency Clinical indications Abnormal distention of the pampiniform venous plexus can cause not only discomfort for a male but also infertility. The venous drainage system from the testes starts within the veins of the mediastinum testis and extends to create a network of vessels, known as the pampiniform plexus.4 Blood from the pampiniform plexus flows into the testicular vein, which then divides into medial and lateral segments.4 Similarly to the female pelvis, the vein on the left joins the left renal vein, and the right connects with the IVC. There has recently been a correlation made that reflects an inverse relationship between the occurrence of varicoceles and body mass index.10 With intensive physical activity of greater than 2 to 4 h daily for 4 to 5 days a week over several years, men are not only more prone to varicoceles but symptoms as well as semen quality appear to worsen.10 There is also a strong, however not proven yet, correlation that suggests that it may be an inherited condition.10 Pathophysiology Abnormalities (such as congenital lack of valves on the left) or vein compression by adjacent structures (such as renal tumours, adenopathy or situs inversus) can result in reflux within the vessel, thus creating the dilatation and distended appearance.4 The pathophysiology related to infertility in the occurrence of varicoceles is still unclear11; however, a likely cause relates to the complex network of blood vessels 15

5 T. Allcorn that supports the inguinal canal that will normally cool the arterial blood from the abdomen to testicular temperature via counter current heat exchange. 4 If the veins are not draining blood away as they should, there may be an increase in temperature, although small, that will be enough to heat the testes enough to cause damaging effects. 4 Varicoceles are the most common treatable cause of infertility. Diagnostic testing Clinical evaluation is generally the first step. A warm environment is preferred with the patient in a standing position and the scrotum is inspected for visible dilations around the spermatic cord. 4 Both relaxed position and Valsalva manoeuvre may be used. Varicoceles have been described as feeling like a bag of worms or in the earlier stages of the condition, more like a thickened cord. 4 There are a number of grading systems, but the most common used are as follows 4 : Grade 1: varicocele palpable during Valsalva. Grade 2: varicocele palpable in the standing position. Grade 3: varicocele detectable by visual scrutiny alone. Clinical examination of the patient is limited and subjective, demonstrating a sensitivity of 50 71% and specificity of 70%. 4,11 For this reason, it is essential that any clinical suspicion is further investigated by means of ultrasound, venography or magnetic resonance/computed tomography. Ultrasound The criteria for diagnosing varicoceles in men are relatively straight forward. A linear probe is ideal, and patient should be examined in both supine and upright positions. 12 The appearance of prominent tortuous veins in the pampiniform plexus, measuring greater than 2 mm in diameter are the most common finding (Figure 5). 12 The veins can be seen to increase in size with the Valsalva manoeuvre, and positive reflux that is residual for longer than 2 s is diagnostic. 4 It is noted that a high number of false positives are referred onto tertiary referral or specialists based solely on a measurement of the diameter of the vessels; therefore, it is essential to always assess for flow reflux. 4 Most commonly, the pampiniform plexus dilates and demonstrates reflux on the left side. Although varicoceles are generally left sided, it is essential to scan both left and right to rule out any bilateral conditions. There is however the possibility of a false varicocele. A true varicocele on the right should demonstrate reflux flow through the right testicular vein. On ultrasound, the right testicular vein can be found joining the IVC, demonstrating reflux on Doppler if incompetent. Reflux of a true varicocele should also be clearly seen through the inguinal canal. 12 The absence of reflux within the inguinal canal is suggestive that the dilatation of vessels on the right is rather a result of collateral vessels from the refluxing left pampiniform plexus feeding the right pampiniform plexus. 12 In this case of a false varicocele, there is no right reflux demonstrated and surgical treatment of the right is not required. 12 In pronounced varicoceles, the left testicular vein should be attempted to be visualized joining the left renal vein, assessing for reflux. Nutcracker syndrome is commonly seen in association with varicoceles (Figure 6). Venography Similarly to the female condition, venography for men is considered the gold standard and is performed in an almost identical way. Again, venography is only used prior to therapeutic intervention, generally following an initial clinical examination and ultrasound. 4 Treatments for both male and female varicoceles For the patient who suffers from severe dilation and reflux in the gonadal veins, there are a number of treatments currently available ranging from medical therapies to surgery. There is debate however on to which treatment is the best for which situation and patient. 13 Medroxyprogesterone acetate, more commonly recognized as progesterone, can be used in the treatment of ovarian vein reflux. Progesterone causes a certain degree of venoconstriction within the ovarian vessels that can relieve some of the symptoms short term. 13 The progesterone implant Implanon (N.V. Organon, Oss, The Netherlands, a subsidiary of Merck & Co., Inc., Whitehouse Station, USA) Figure 5 Left-sided varicocele in a male. Note the tortuous collection of veins. 16

6 T. Allcorn Figure 6 The same patient also had nutcracker syndrome. Note the narrowing of the left renal vein between the SMA and aorta. The left testicular vein was visualized with reflux. has also recently been investigated, appearing effective in short term relief of symptoms. 13 Endovascular treatments are generally the most common method performed for both men and women. Vein embolisation techniques with the use of coils as well as sclerosants are also proven to be a successful and provide long term benefits. 14 A guide wire is passed through the right femoral vein, or jugular vein, and advanced into the affected vein. 7 Sclerosant is then injected slowly with the patient holding their breath and performing a Valsalva manoeuvre. 7 The most common sclerosant used is sodium tetradecyl sulfate and polidocanol, although there are a large number of sclerosants available. 4 The combination of external compression may also be used during the sclerosant injection, particularly in men to avoid the sclerosant passing into the scrotum. 13 Complications are rare and will generally be related to thrombophlebitis or in the event of the wrong sized coil, pulmonary embolisation. 13 The risk of perforation has in recent times been diminished with the use of hydrophilic wires and microcatheters. 4 Embolisation with coils has a number of advantages. The coils are generally easily handled and are manoeuvreable during surgery. 13 Minimal patient discomfort is reported, and the coils can be altered in size to suit the patient s requirements. 4 Most commonly in. coils that range from 5 to 8 mm are used. 4 Surgical treatments are rarely performed now because of the efficacy of endovascular procedures; in addition, the likely two days hospitalisation and two weeks discomfort post-surgery is limiting, particularly for this demographic of generally young mothers; however, on rare occasions, surgery is still necessary. 7 Complete removal of the uterus and ovaries, hysterectomy and oophrectomy, has been documented to show symptomatic improvements in two-thirds of patients; however, there is high associated morbidity. 15 Ovarian vein ligation previously was the treatment of choice and had a reported cure rate of 73%. 7 The recurrence rate however was significant because of missed collaterals and reflux contributing from near-lying vessels. 7 There is also a high risk of damaging the nervous plexus that can ultimately lead to recurrent symptoms, as well as significant risk of haemorrhage during surgery if the vein is not handled gently. 7 In the case of adolescent varicocele, a prophylactic repair is recommended generally only when a reduction in testicular size is greater than 2 cm 3 or deviates from the normal testicular volume for that age group. 4 Complications if the pelvic veins are not treated If incompetent veins in the pelvis are not treated, further complications, besides ongoing pains, can arise. It is common to see incompetent pelvic veins in both men and women that communicate with the veins in the legs. 9 Communicating varicosities from the pelvis to the legs can, if not identified, dramatically affect the outcome of treating incompetent leg veins. 9 There is a high recurrence rate of varicose veins in the legs in patients with pelvic insufficiency. There are three patterns that have been suggested of pelvic incompetence communications that can be easily identified during a leg venous insufficiency scan 9 : Anterior: The superficial external pudendal or superficial inferior epigastric veins demonstrating reflux into the greater saphenous vein or thigh varicosities near the saphenofemoral junctions. 9 On ultrasound, this can be identified by large, tortuous vessels in the groin. Medial: The posteromedial tributary that joins the greater saphenous vein approximately 10 cm from the saphenofemoral junction. 9 Generally, a large vessel of similar size to the great saphenous venous however outside of the saphenous sheath, with tributaries able to be traced to the vulva. Posterior: On the posterior aspect of the thigh, varicosities generally communicate with the vein of Giacomini and can easily be traced up to the buttock or peri-rectal region. 9 Conclusion Although pelvic insufficiency in men is very well documented, pelvic insufficiency in women needs closer attention. This common condition affects the health of many women and may be overlooked and underdiagnosed. By comparing the similarities between 17

7 T. Allcorn male and female insufficiency, a standardised and more common practice of diagnosing female insufficiency may be implemented. The overall treatment and outcome for the patient, not to mention the long term benefits, especially if leg varicosities are involved as well, will be significantly improved if this condition is diagnosed correctly. References 1 Bhutta HY, Walsh SR, Tang TY, Walsh CA, Clarke JM. Ovarian vein syndrome: a review. Int J Surg 2009; 7: Mozes G, Gloviczki P. Venous embryology and anatomy. In: Bergan JJ, ed. The Vein Book. San Diego: Elevier Academic Press; 2007; Hobbs JT. Varicose veins arising from the pelvis due to ovarian vein incompetence. Int J Clin Pract. 2005; 59: Bittles MA, Hoffer EK. Gonadal vein embolization: treatment of varicocele and pelvic congestion syndrome. Semin Intervent Radiol. 2008; 25(3): Kim SH, Cho SW, Kim HD, Chung JW, Park JH, Han MC. Nutcracker syndrome: diagnosis with Doppler US. Radiology 1996; 198: Kurklinsky AK, Rooke TW. Nutcracker phenomenon and nutcracker syndrome. Mayo Clin Proc 2010; 85: Richardson G. Pelvic congestion syndrome: diagnosis and treatment. In: Bergan JJ, ed. The Vein Book. San Diego: Elsevier Academic Press; 2007; Richardson GD, Driver B. Ovarian vein ablation: coils or surgery? Phlebology 2006; 21: Paige J. Duplex scanning of the pelvis veins for venous reflux. Sound Effects 2001; (4): Es teven SC. Varicocele. In: Parekattil SJ, Agarwal A, eds. Male Infertility. New York: Springer; 2012; Pilatz A, Altinkilie B, Kohler E, Marconi M, Weidner W. Colour Doppler ultrasound imaging in varicoceles: is the venous diameter sufficient for predicting clinical and subclinical varicocele? Worl J Urol 2001; 29: Liguori G, Trombetta C, Garaffa G, Bucci S, Gattuccio I, Salame L, Belgrano E. Colour Doppler ultrasound investigation of varicocele. World J Urol 2004; 22: Rane N, Leyon JJ. Littlehales T. Ganeshan A, Crowe P, Uberoi R. Pelvic congestion syndrome. Curr Probl Diagn Radiol 2013; 42: Ascuitto G, Mumme A, Asciutto KC. Geier B. Pelvic vein incompetence influences pain levels in patients with lower limb varicosity. Phlebology 2010; 25: Umeoka S, Koyama T, Togashi K, Kobayashi H, Akuta K. Vascular dilatation in the pelvis: identification with CT and MR imaging. RadioGraphics 2004; 24: