© Springer India 2015
Subramoniam Vaidyanathan, Riju Ramachandran Menon, Pradeep Jacob and Binni JohnChronic Venous Disorders of the Lower Limbs10.1007/978-81-322-1991-0_2121. Pelvic Venous Syndromes
(1)
General Surgery, Amrita Institute of Medical Sciences, Amrita Lane, Kochi, Kerala, 682041, India
Introduction
Pelvic venous syndromes include a group of poorly understood disorders of the pelvic and gonadal circulation in the female, characterized by chronic pelvic pain. Broadly, two groups of conditions can be identified – pelvic congestion syndrome (PCS) and vulvovaginal varices. Lower limb varicose veins can be an associated feature in patients with PCS. The pelvic origin of these varices is not often suspected by the treating physician. Attempts to ablate the leg veins without controlling the reflux from the pelvis are an important cause of recurrence.
This chapter reviews the following aspects:
Definition and etiopathogenesis of PCS
Clinical features and classification
Imaging in PCS
Management principles
Overview of vulvovaginal varices
Pelvic Congestion Syndrome
Pelvic congestion syndrome is characterized by chronic recurring pelvic pain and discomfort of at least 6 months duration, for which no obvious local pathology could be identified. It is experienced typically in the premenstrual phase. The pain is aggravated by prolonged standing, postural changes, or activities that increase intra-abdominal pressure. A classical feature is aggravation of pain after sexual intercourse – postcoital ache. It may be associated with dysmenorrhea, deep dyspareunia, and urinary urgency [1–4]. The association of pelvic pain and pelvic varices was first documented by Taylor in 1949 [2]. Apart from PCS, several other terms have been used to describe this entity [2]: pelvic pain syndrome, pelvic venous incompetence, etc. [2]. PCS is the most widely accepted terminology in current clinical practice.
PCS is observed usually in premenopausal multiparous women aged between 20 and 45 years [4]. It is rare in nulliparous women. No case has been reported in postmenopausal women [1]. Prevalence of PCS is around 30 % in patients presenting with chronic pelvic pain in whom no other obvious pathology could be found [5]. The reported incidence of this problem in the USA is one in seven women between the ages of 18 and 50 [2].
Etiopathogenesis
The etiology of PCS is not very clear. Most of the workers attribute the condition to a pelvic venous pathology. This was based on the observations of dilatation, incompetence, and reflux of the ovarian and internal iliac veins resulting in periovarian varicosities. Tubo-ovarian varicoceles have been termed the female equivalent of testicular varicocele [1]. Administration of venoconstrictors or ovarian vein ligation/embolization produced symptom relief in several patients. This is yet another finding supporting pelvic venous pathology as the possible etiological factor for PCS [1]. Several causes are suggested for the pelvic venous pathology in these patients:
Incompetence/absence of valves in the ovarian and pelvic veins is a common finding and could result from congenital or acquired causes. This results in pooling and distention of the ovarian and pelvic venous system [2]. Since the valves are absent more commonly on the left ovarian veins, the pathology is more common on the left side. The incidence on the left side is around 15 % and on the right side 6 % [1]. But incompetence of the ovarian veins is not uncommon in asymptomatic multiparous women [1].
Pregnancy exacerbates PCS. Gravid uterus increases venous return. It can also produce obstruction of the draining veins. Mass effect from enlarged veins in the pelvis can lead to bulk symptoms and irritation [2].
Estrogen has a major role in the pathogenesis of PCS. Estrogens are known to produce venous dilatation. Absence of PCS in the postmenopausal women may be related to the decline in estrogens. Induction of hypoestrogenic state has been attempted as a pharmacological approach in the treatment of PCS [6].
Obstruction to venous outflow can develop at the pelvic or suprapelvic locations. Greiner and group have identified a subset of patients with PCS developing secondary to stenosis or obstruction in a draining vein [3]. The left renal vein could be compressed between the abdominal aorta and the superior mesenteric artery – the nutcracker syndrome [3]. At the pelvic level, iliocaval obstructions, thrombotic or nonthrombotic (May-Thurner syndrome), are reported causes of PCS [7].
Factors that determine the intensity of the symptoms include response of the vein wall, presence of venous drainage routes to the lower limbs (pelvic leakage points), and location of the venous anomaly (ovarian or internal iliac tributaries) [3]. One can identify several subtypes of PCS depending on the above factors [3]:
Ovarian vein syndrome (the classical type) is characterized by chronic pelvic discomfort and pain, dysmenorrhea, dyspareunia, and postcoital discomfort.
Neurological syndromes are characterized by pudendal, obturator, sacral, and piriformis muscle neuralgia.
Visceral syndromes are not very common.
It has been emphasized that pelvic symptoms may be absent or minimal when the pathological veins are effectively drained through escape routes. In this setting, the pelvic transmural pressure is not elevated. Such patients may present with congestive symptoms in the lower limbs [3].
Classification
Greiner has identified three distinct pathological processes responsible for the development of PCS [3]. Recognition of such pathophysiological subtypes would greatly help in the fine-tuning of therapy for this difficult condition.
Type I: Reflux pathology. This results from venous anomalies in pelvis without obstruction to venous flow. The common causes include:
Congenital/acquired valvular incompetence
Absence/destruction of valves
Dilatation of pelvic veins (congenital or acquired)
Venous malformations
Type II: Obstructive pathology. This results from stenosis or obstruction in a draining vein. The obstruction can be suprapelvic or pelvic. Suprapelvic obstruction can involve left renal vein (nutcracker syndrome) or in the IVC (congenital, extrinsic compression, and thrombosis). Primary causes of obstruction at pelvic level include May-Thurner syndrome and congenital anomalies of the internal iliac vein. Secondary causes include extrinsic compression and thrombosis.
Type III: Local extrinsic cause. Several local pathologies in the pelvis are to be considered and ruled out. These include:
Endometriosis
Tumors
Benign mass
Secondary uterine retroversion (postpartum)
Post surgery/infection/adhesions
Loss of support (Allen-Masters syndrome)
Non-obstetric trauma
Clinical Features
PCS is common in women in the age group of 20–45 years. The condition has been diagnosed even in girls of 13 years of age [2]. There is no genetic or ethnic predisposition, but it is certainly more common in multiparous women [2]. The symptoms of PCS include:
Pelvic pain is the most common symptom. The duration of pain is more than 6 months [1, 5]. It manifests during or after first pregnancy and is aggravated by subsequent pregnancies [2]. The pain varies in intensity but is usually described as a noncyclical dull ache or heaviness that is worse in the premenstrual phase. Postural changes, prolonged standing, walking, or activities that increase intra-abdominal pressure, all can aggravate the pain. It can be unilateral, more on the left side. It can also be bilateral or shift from one side to the other. The pain is typically aggravated after sexual intercourse – postcoital ache [1–3].
Other symptoms include dysmenorrhea, deep dyspareunia, and urgency and frequency of micturition [1, 4, 5]. Pudendal, obturator, sacral, and piriformis muscle neuralgia are uncommon symptoms in some women [3]. Rectal discomfort is not an uncommon symptom of PCS [4].
Vulvar and lower limb varices. PCS can be associated with vulvar varices. They can be located over the gluteal region, vulva, and posterior aspect of thigh [1–3]. Lower limb varicose veins are common findings in a large number of patients with PCS when there is a pelvic escape route [1, 3, 5]. In a retrospective analysis of 48 women with PCS, Malgor and group have observed that all but three had lower limb varicose veins [8]. Pelvic venous reflux is reported to be a major contributory factor for recurrence of varicose veins in women [9].
Nutcracker syndrome. In this condition apart from the pelvic pain, the patient can experience flank pain along with hematuria, often microscopic than overt [2].
Palpation of the abdomen demonstrates tenderness over the ovarian point (junction of upper 1/3 and lower 2/3 of the spino-umbilical line) [1, 2]. Bimanual pelvic examination demonstrates marked ovarian tenderness, tenderness on moving cervix, and uterine tenderness. A positive history of postcoital ache along with ovarian point tenderness on abdominal palpation is considered to be diagnostic of PCS (94 % sensitive and 77 % specific) [1, 2, 10].
Imaging in Pelvic Congestion Syndrome
The road to the diagnosis of PCS is often long and arduous; it is often established only after excluding other causes of pelvic pain [4]. The objective of imaging in PCS is to document the presence of the typical pelvic venous changes. The most widely reported finding is incompetence and dilatation of ovarian veins. Presence of dilated ovarian veins is necessary, but not sufficient for diagnosis of PCS [1]. Imaging is also important in ruling out other causes of chronic pelvic pain.
Ultrasound Scan
This is the basic imaging modality in PCS. This is useful in excluding other pelvic pathologies for chronic pelvic pain. Transabdominal and transvaginal scans are carried out. A lower limb duplex scan is also combined along with this. To enhance the sensitivity, the test is conducted in the upright position with the patient performing Valsalva maneuver. If PCS is suspected, the ovarian veins should be evaluated. Dilatation of the left ovarian vein with reversal of flow on USG is diagnostic of PCS [2]. The mean diameter of left ovarian vein in patients with PCS was found to be 7.9 mm; in control subjects, it was only 4.9 mm [11]. However, incompetent and dilated ovarian veins on helical CT scan were observed in 63 % of asymptomatic parous women and in 10 % of nonparous women [12]. Currently, an ovarian vein diameter of 6 mm or more on ultrasound is considered a significant finding supporting the diagnosis of PCS [2, 8]. This is reported to have a 96 % positive predictive value [13].
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