Fig. 18.1
Anatomy and reflux of the pelvic veins (arrows show pathologic reflux). (A) Anatomy. (B) Anatomic variations of the ovarian veins. (C) Internal iliac veins and their communications with the utero-ovarian plexus and thigh superficial veins: 1, ovarian vein; 2, internal iliac vein; 3, uterine vein; 4, obturator vein; 5, external pudendal vein; 6, great saphenous vein; 7, varicosity of the anteromedial aspect of the thigh; 8, varicosity of the posterolateral aspect of the thigh; 9, sciatic vein; 10, vulvar varicosity; 11, internal pudendal vein; 12, cystic and vaginal veins; 13, buttock veins
These veins are, in the classic anatomic description, forming two collectors, the anterior (obturator, inferior gluteal, medial pudendal, middle hemorrhoidal, uterine, umbilical, vesical, and vaginal veins) and the posterior (superior gluteal, ascending lumbar, and lateral sacral veins).
Anatomic variations of the IIV are very frequent and three main types can be found: confluence of the two collectors in one IIV (50%), separate ostia for each trunk in the iliac axis (36%), and plexiform type (14%) [10]. One frequent variation is the presence of a communication with the contralateral common iliac vein.
There are connecting networks between the branches of the internal iliac veins and the lower limbs veins that can explain the high frequency of lower limb varicose veins due to pelvic venous insufficiency through the obturator and inferior gluteal veins mainly but also by the pudendal veins.
Pathophysiology
According to Greiner [11], pelvic varicose veins can be due to three different mechanisms:
Type 1: reflux secondary to pelvic vein incompetence represents the most frequent etiology. Hormonal factors contribute to varicose veins and their concentration is higher in the pelvis. Estradiol inhibits the reflex vasoconstriction of vessels and causes uterine enlargement with dilatation of the ovarian and uterine veins preponderantly during pregnancy [12]. Moreover a 35% decrease of pelvic veins diameter and decrease of pelvic blood flow and symptoms was found after intravenous injection of dihydroergotamine in women with pelvic congestion and pain [13]. Moreover, ovarian cyst is frequently found in patients with pelvic varicose veins [13, 14].
Type 2: secondary to an obstruction of the outflow as May-Thurner syndrome [15, 16], nutcracker syndrome [16, 17] and left renal vein thrombosis, post-thrombotic disease involving the common iliac veins or the IVC (or both), and Budd-Chiari syndrome. These obstructions must always be eliminated before embolization, especially in nulliparous women.
Type 3: secondary to a local extra venous phenomenon. The main cause is endometriosis, but it can also be due to tumors (benign or malignant), post-traumatic lesions.
Clinical Findings
PCI occurs mainly in young multiparous women and usually disappears after menopause [18].
The pelvic congestion syndrome (PCS) always includes chronic (up to 6 months) pelvic pain (heaviness), diffuse or localized in the iliac fossae (predominantly on the left side). It increases during the day, mostly if the patient stays sitting or standing and when lifting, and can be relieved by lying down and before menses. It can be associated with dyspareunia, dysmenorrhea, and urinary symptoms (dysuria, pollakiuria, bladder urgency). Patients should be questioned about perineal (mainly vulvar) varicose veins, past (mainly during pregnancy) and present.
On clinical examination , the combination of tenderness on abdominal palpation over the ovarian point and a history of pain after sexual activity was 94% sensitive and 77% specific for discriminating PCS from other causes of pelvic pain [18].
Perineal and lower limbs varicose veins should be searched as well as history of previous treatment. On the lower limbs, they can be located at the groin (mainly after GSV surgery), but atypical varicose veins can also be found (buttock, posterior and lateral aspect of the thigh, etc.) (Fig. 18.2).
Fig. 18.2
Atypical varicose veins at the posterior aspect of the right thigh
Diagnosis
Duplex Scanning
Pelvic and abdominal ultrasonography with color duplex scan should be performed with transparietal 5 MHz and transvaginal probes ideally after 3 days of a no-residue diet and with an empty stomach [15]. Pelvic varicose veins are defined as multiple dilated tubular veins around and into the pelvis with a venous blood Doppler signal and a diameter larger than 5 mm [15]. Internal iliac veins and the gonadal veins should be imaged looking for dilatation and reflux (reversed caudal flow). It can be improved by using the Valsalva maneuver. The positive predictive value of a 6 mm diameter ovarian vein for the diagnosis of PCS caused by the ovarian vein is 83.3% [19]. The obturator, sciatic, and internal pudendal veins must also be imaged. Collateral pathways can be found. In addition, duplex scanning should evaluate the common iliac veins, IVC, and renal veins to search for venous obstruction or anatomic variations.
It can also be used to explore pelvic content looking for other causes of pelvic pain.
Lower limb duplex scan must be performed to search for varicose veins, which can be secondary to pelvic varicose veins. Superior and inferior gluteal points are highly specific of incontinence of the corresponding veins [20].
Computed Tomographic Venography (CTV)
CT should be timed with a portal phase for evaluation of the genital and renal veins, and a separate imaging should be performed at a venous phase to evaluate the pelvic and iliocaval veins. According to Rozenblit, an incompetent ovarian vein is defined as a completely opacified vein during the portal phase of CT angiography and dilated if it measures 7 mm or greater at its larger diameter (Fig. 18.3a) [21]. Pelvic varices are visualized as dilated, tortuous, enhanced tubular structures around the pelvic organs (Fig. 18.3b, c). CT does not have very good visualization of the IIV network.
Fig. 18.3
Preoperative CTV. (A) Incompetent left ovarian vein (arrow). (B) Pelvic varicose veins (arrow head). (C) Three-dimensional reconstruction showing the incompetent left ovarian vein and the pelvic varicose veins
Magnetic Resonance Venography (MRV)
Pelvic MRI is essential in the exploration of pelvic pathology. On T1-weighted MRI, pelvic varicose veins have no signal intensity because of the flow void artifact; on gradient-echo MRI, varicose veins have high signal intensity. On T2-weighted MRI, they usually appear as an area of low signal intensity, although hyperintensity or mixed signal intensity may also be noted, possibly because of the relatively slow flow through the vessels. Two- and three-dimensional, T1-weighted gradient-echo sequences performed after the intravenous administration of gadolinium are the best sequences for demonstrating pelvic varicose veins [22–24]. In order to explore completely patients in case of PVI, MRV must also analyze veins in the abdomen and assess the renal veins.
Phlebography
Abdominopelvic retrograde phlebography is the “gold standard” for diagnosis [25]. It can be performed under local anesthesia via the common femoral vein (5F hydrophilic Cobra 2 catheter and Simmons catheter for the right ovarian vein) or right brachial or jugular vein (5F multipurpose catheter) approach [25, 26]. The main advantage of the jugular/brachial approach is a higher rate of right ovarian vein catheterization (18% failure for brachial approach [25] versus 58% failure through femoral approach [26]). Regardless of the approach, ultrasound-guided access should be used. Through a femoral approach the internal iliac vein can be catheterized with a Cobra 2 or a UF catheter. In case of previous left ovarian vein embolization, the left internal iliac vein can be found near the coils of the left ovarian vein.
The operator should image the four veins responsible for venous return from the pelvis: both internal iliac and gonadal veins with Valsalva manoeuver. Kim advocates the use of balloon occlusion venography to image the internal iliac veins [27]. Phlebography provides identification of the pathologic veins, their diameter, and their length. Moreover, it must explore the iliac veins, the IVC, and the LRV for obstructive disease. In the case of suspected nutcracker syndrome, the reno-caval pullback gradient must be measured (considered as significant if >3 mmHg).
Chung and Huh [28] reported criteria used for the phlebographic diagnosis of PCS caused by the ovarian vein : ovarian vein larger than 5 mm in diameter, retention of contrast medium for longer than 20 s, existence of congestion in the pelvic venous plexus or opacification of the internal iliac vein, or filling of vulvovaginal and thigh varicosities (Figs. 18.4a, b, 18.5a, b, 18.6a, b and 18.7a). Each variable is assigned a value between 1 and 3, depending on the degree of abnormality, and a venogram score of 5 or higher indicates the presence of PCS.
Fig. 18.4
Embolization of an incompetent left ovarian vein. (A) Catheterization with a Cobra (C2) catheter and angiography in the left renal vein showing absence of left renal vein compression and the presence of an incompetent left ovarian vein, (B) Left ovarian vein phlebography showing an incompetent vein and pelvic varicose veins. (C) Result after embolization using foam and coils according to the sandwich technique (multiples coils were used in the trunk of the vein due to the presence of multiple branches)
Fig. 18.5
Right ovarian vein (ROV) embolization through arm approach in a patient who previously had left ovarian vein and right inferior gluteal vein embolization. (A) Selective catheterization and phlebography of the ROV using a multipurpose catheter. (B) Phlebography of the caudal part of the ROV showing incompetence and presence of pelvic varicose veins. (C) Result after embolization using foam and coils according to the sandwich technique: incomplete result with persistent reflux down the ovarian vein. (D) Completion phlebography after deployment of another coil
Fig. 18.6
Embolization of branches of the left internal iliac vein (LIIV) after embolization of the left ovarian vein (LOV). (A) Catheterization and angiography of the LIIV with a Cobra 2 catheter: the LIIV is found close to the LOV coils. (B) Superselective phlebography showing an incompetent vesicular vein. (C) Results after embolization
Fig. 18.7
Incompetence of the right internal iliac vein. (A) Selective phlebography through contralateral approach using a Cobra 2 catheter. (B) Result after embolization
Differential Diagnosis
The differential diagnosis is a major concern for PCS as there are multiples causes of chronic pelvic pain (endometriosis, uterine fibroma, pelvic cancer, pudendal nerve compression, etc.). These must be eliminated before treatment because the presence of pelvic varicose veins does not necessarily mean that the cause of the pain is PCS. The diagnosis relies on analysis of the symptoms and on a thorough work-up. Laparoscopy was used till the development of MRI but is quite invasive: it can show pelvic varicose veins if performed in a feet-down position while limiting the pressure of peritoneal insufflation, and it can identify sometimes other pelvic pathologies. Pelvic ultrasound and MRI represent nowadays the imaging modalities of choice for the evaluation of pelvic pain, but sometimes a more extensive evaluation is needed.
Treatment
Medical Treatment
Surgery
Different surgical techniques have been reported for the treatment of PCS, including ovarian or internal iliac vein ligation (or both), ovarian and uterine artery and vein ligation, oophorectomy, and even total hysterectomy with bilateral salpingo-oophorectomy. Ovarian vein ligation or resection can be performed laparoscopically, but they remain invasive techniques. Despite the fact that bilateral oophorectomy combined with hysterectomy and hormone replacement therapy has been shown to be effective in patients who failed to respond to medical therapy [14], this is an invasive option that is not acceptable for women who want to become pregnant.
Embolization
The procedure can be performed under local anesthesia either together with diagnostic phlebography or as a separate procedure. After selective catheterization and contrast-enhanced study of the refluxing vein or veins, embolization is performed. In case of extensive lesions, the patient should be preoperatively counseled that multiple (two or even three) procedures may be needed.
It is mainly performed using coils (0.035 in. for 4 or 5 Fr catheter and 0.018 in. for microcatheters, pushable or detachable, fibred or not) (Figs. 18.4c, 18.5c, d, 18.6b, c and 18.7b). In case of very large veins, vascular plugs (Amplatzer®) can be used. These devices can be used in conjunction with foam in order to reduce the number of coils needed and the rate of recurrences. Foam is prepared from sodium tetradecyl sulfate (Thrombovar or Sotradecol) or polidocanol (Aetoxisclerol) according to the Tessari method and can be injected either before coiling or by using the sandwich technique (see Figs. 18.2e, 18.3b, 18.4b and 18.5b). It can use air or a 50%/50% mixture of CO2 and O2. Some teams are using glue, but their philosophy of the treatment is different: the goal is more to occlude all varicose veins rather than suppressing reflux as coils and foam do. Moreover, the use of glue is far more time consuming, painful, and expensive than the sandwich technique. The use of sodium morrhuate together with Gelfoam has also been reported [27].
Some rules must be followed: embolization should begin the more distally possible, the main trunk of the IIV must not be embolized, and regarding the gonadal veins, embolization must be performed proximal to the last collateral in order to prevent recurrences. Regarding the right ovarian vein (ROV) , even if dilated, embolization is not always necessary when the left ovarian vein (LOV) is incompetent. The incompetent LOV and branches of the IIV must be treated first and decision regarding ROV treatment will rely on its evolution. When treating branches of the IIV, Kim recommend to use balloon occlusion [27]. Moreover, the presence of significant communications between the tributaries of the IIV and the CFV and EIV must be emphasized. If not identified, it may pause a risk of coil embolization and dislodgement. Patients should be informed that in complex cases with multiple trunks incompetence embolization may need to be repeated to treat all the lesions.
Complications are rare and include hematoma at the access site, extravasation of contrast material (that excludes the use of foam ), coil or glue embolization (Fig. 18.8), deep venous thrombosis and pulmonary embolism, and transient cardiac arrhythmia. In case of inadvertent coil dislodgement, it must be retrieved using a snare.
Fig. 18.8
Migration of a coil in the left renal vein during left ovarian vein embolization. It was subsequently retrieved with a snare
A review of the literature is provided in Table 18.1 [16, 24–28, 31–51]. Chung compared ovarian vein embolization, hysterectomy with bilateral oophorectomy and hormone replacement therapy, and hysterectomy with unilateral oophorectomy through a prospective randomized study of 164 women with PCS [28]: embolization was significantly more effective than the other two techniques. Asciutto showed that using embolization untreated patients had no improvement while treated patients were improved [24]. Monedero reporting on 1186 cases of embolization for recurrent lower limb varicose veins caused by PVI had better results with coils and foam using the sandwich technique than with coils alone (95.6% rate of improvement versus 76% at 6 months), thus also reducing the cost of the procedure [25]. According to the literature, better results were obtained in series reporting embolization of all incompetent veins rather than those treating ovarian veins only (Table 18.1). A review of the literature published in 2016 found 20 studies with a total of 1081 patients: technical success rate was 99%, and long-term results were good with 86.6% of improvement [52].In case of lower limb varicose veins linked with PVI, we wait at least 3 months to take them in charge after embolization as this can reduce their volume and change the type of treatment to be used (16).
Table 18.1
Results of embolization for pelvic congestion syndrome
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