Pelvic congestion syndrome (PCS) is one of the common causes of chronic pelvic pain that predominantly affect the multiparous women, and their presentation usually occurs during their childbearing period mainly in the premenopausal age [1,2,3]. This was confirmed in this study, where, the patients’ ages ranged from 19 to 47 years with a mean (± SD) (35.1 ± 7.31 years), and most of them were multiparous apart from one patient who was nulliparous. A correlation between the PCS and the decline in the ovarian activity in premenopausal women had been suggested [5].
Many diagnostic modalities were used in the diagnosis of PCS including invasive and noninvasive tests [4]. In the present study, we tried to give a simple order for the examination steps in an easy-to-perform method for assessment of the PCS that can be feasible for all expert and non-expert radiologists.
CT venography (CTV) may be of great value in delineation of the precise anatomical details of the pelvic, renal, and the gonadal vein abnormalities (Fig. 10), but on the other hand, it may be of less value on the hemodynamic changes [6]; the need for contrast administration and the exposure to radiation may be considered as limitations for the use of the CTV in this issue.
Magnetic resonance imaging (MRI) is currently considered as an investigation of choice for PCS, as it provides the information about both the anatomical and flow abnormalities without exposure to ionizing radiation, unlike the CTV [7]; however, it is considered expensive when compared with the trans-abdominal venous duplex examination; moreover, MRI may not be suitable for certain patients like claustrophobic and patients with cardiac pacemakers.
Park et al. and Whiteley et al. had considered the trans-vaginal ultrasound as the gold standard for diagnosis of PCS [2, 8]; they explained this by its ability for better visualization of the parametrial veins and for detection of any other gynecological problem [8]; however, the whole course of the gonadal veins could not be assessed by the trans-vaginal route as well as the renal and the iliac veins compression; moreover, the trans-vaginal examination cannot be done in certain circumstances like in virgin women (we had one case). Therefore, the trans-abdominal and the trans-perineal venous duplex scanning appeared to be a more beneficial examination, and this was greatly matching Labropoulos et al. study [1].
Being available, noninvasive, cheap, and avoiding the exposure to radiation or the use of contrast material, the trans-abdominal, and the trans-perineal venous duplex scanning had gained a great diagnostic value through providing anatomical and hemodynamic details about the examined veins in cases of PCS; nevertheless, the patients can change their positions from supine to standing and vice versa, and the examiner can do the abdominal pressure when the patients cannot perform controlled Valsalva [1].
By trans-abdominal venous duplex scanning, the left gonadal vein elicited reflux by raising the abdominal pressure either by gentle sub-xiphoid compression or by controlled Valsalva. The left gonadal vein dilatation was also demonstrable with a mean diameter (± SD) = 7.9 ± 1.1 mm (Table 2); this greatly matched the literature [1,2,3]. There was a debate about the vein diameter or the presence of reflux as a primary criterion for diagnosis of PCS; in this study, we greatly agreed with Labropoulos et al. [1] where the reflux was considered as the primary criterion; this is because all the left gonadal veins were refluxing in this study (100%) (Table 1), but regarding the vein diameter, some authors consider gonadal vein diameter cutoffs starting above 4 mm as diseased veins [2]; however, the right gonadal vein was competent in 46 cases (92%) in this study with mean diameter (± SD) = 4.0 ± 0.8 mm (Tables 1 and 2); whereas, the refluxing right gonadal vein was detected in 4 cases (8%) with a mean diameter (± SD) 5.9 ± 0.4 mm (Tables 1 and 2); this could be explained by the fact that the vein could be dilated to compensate for the volume overload from the contra-lateral vein reflux; in other words, a smaller but refluxing vein should be considered as a pathological vein rather than a larger but competent vein. In our experience, a venous reflux coexistent with a large vein diameter should be considered as the criteria for pelvic venous insufficiency in addition to the presence of parametrial varices.
Szaflarski et al. had recently conducted a study on a large number of patients (1042) using the CT abdomen and pelvis for evaluating the severity of the ovarian vein dilatation that was present only in 143 patients; they selected the diameter parameter as a criterion for detection of the PCS with the mean diameter for the dilated left gonadal vein = 7.5 mm while for the right gonadal vein = 7.2 mm; they also found—based on quartile analysis—that the ovarian vein dilatation grading was mild (< 6 mm) moderate (6–8 mm) and severe (> 8 mm); again, in our study, we relied on the diameter and on the presence of the venous reflux (lasting for > 0.5 s), and this could explain the difference between the two studies [9].
Refluxing proximal segments of the internal iliac veins were detected in 3 patients (6%) one of them (2%) on the left side and two (4%) were on the right side (Table 1); this greatly matched with Liddle and Davies, where, they consider the presence of the internal iliac vein reflux in PCS, but actually, the majority of cases with pelvic varices had ovarian vein reflux [4]. Similarly, the venous reflux was the considerable criterion rather than the vein diameter when we went for assessment of the internal iliac vein, as we noticed that the internal iliac veins may dilate secondary to refluxing ovarian vein, but their competence was still preserved.
In agreement with Zerhouni et al. and Unlu et al., in the current study, the left renal vein nutcracker phenomenon was detected in the majority of cases (82%) being of the anterior type (pre-aortic) in (76%) of cases and being posterior type (retro-aortic) in (6%) of cases (Table 3); this eventually considered the left renal vein entrapment as the primary cause of left gonadal vein reflux [10, 11]. For nutcracker phenomenon diagnosis, the aorto-mesenteric window (distance), the aorto-mesenteric angle, the velocity ratio in the left renal vein segments were measured as well as the diameter of the left renal vein in the aorto-mesenteric space and compared with the preceding (hilar) dilated segment [1, 12].
Szaflarski et al. had described the presence of nutcracker only in 14.4% of their patients, but this could be explained by the different sample sizes where they included 143 patients in their study [9].
The left common iliac vein compression by the crossing right iliac artery was detected in 3 of our cases (6%) (Table 3) by using the trans-abdominal ultrasound examination [1]; however, the internal iliac veins proximal segments in these cases were competent; this may emphasize that the pelvic venous insufficiency is attributed mainly to the gonadal vein reflux rather than the iliac vein reflux; this was concordant with Rastogi et al. study, where they considered the two conditions being unrelated [13].
Craig and Hobbs and Hobbs had declared a strong association between PCS and the varicosities that were detected in the vulva as well as their extensions in the thighs and buttocks especially, if the varicosities had atypical distribution in the lower limbs, if there was a recurrence with no obvious cause or in those with primarily failed surgical treatment [14, 15].
Jung et al.’s study considered the vulvoperineal and the round ligament varicosities as parts from the PCS; they also found that about 85–90% of the unusual causes of the lower limb varicosities were arising primarily from the vulvoperineal varicosities then from the round ligament varicosities [16]. In our study, we searched for the vulvoperineal and the round ligament varicosities in all of our cases, and we found that vulvoperineal varicosities were present in all cases of PCS (100% of cases, n = 50), and to lesser extent, round ligament varicosities were present (6% of cases, n = 3), and the thigh extension of the vulvoperineal varicosities was found in (74% of cases) and in all cases (3%) with round ligament varicosities (Table 4); this high proportion of thigh extension of the vulvoperineal varicosities was rationale as all patients in this study were referred mainly from the vascular surgery department with atypical limb varicosities, and the patients’ clinical diagnosis was PCS.
Recently, a correlation between the ovarian varices and infertility had been found in some patients, and the effect of the gonadal vein embolization on the subsequent conception was studied by Liu et al., and they reported the safety and the efficacy of the technique for the women trying to have conception [17].
Unlike Niclot et al. and Iupatov et al. studies, we excluded pregnant women from our study and those who had an ilio-femoral DVT (post thrombotic PCS) as we were focused to establish the technique in the patients who had primary venous insufficiency related to the gonadal or the iliac veins [18, 19].
In agreement with the literature, PCS is not uncommon entity that often misdiagnosed or undiagnosed clinically and radiologically, thus we recommended that one should consider the diagnosis PCS in the diagnostic list when evaluating the female pelvis for chronic pelvic pain and in cases of atypical lower limb varicosities, especially, in limbs where the varicosities assemble to the proximal inner thighs and to the perineum.
Several limitations were met in this work; the first one was regarding the technique of examination, being an operator dependent technique that needed experience and repeated practice for reducing the duration of the examination; the second one, was that the trans-abdominal studies depends greatly on the good bowel preparation and the body habitus; however, this was overcome by an overnight fasting, changing the patient position during the examination, and cleansing enemas when needed; the third one was the lack of control (healthy) group for detection of the cutoffs for normal gonadal veins diameters; however, this was described by other studies; the last one was the absence of a gold standard comparative imaging study (like selective gonadal venography, CTV or MRV); this was attributable to that many patients did not go for further imaging work up especially if they had no intention of doing interventional procedures.