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International Journal of Surgery 7 (2009) 516–520

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International Journal of Surgery

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Review Ovarian syndrome: A review

Hina Y Bhutta a,*, Stewart R Walsh a, Tjun Y Tang a, Colin A Walsh b, James M Clarke a

a Department of Surgery, Norfolk & Norwich University Hospital NHS Trust, Colney Lane, Norwich, NR4 7UY, UK b Department of Obstetrics and Gynaecology, St George Hospital, Kogarah, New South Wales, Australia

article info abstract

Article history: The Syndrome was first reported in 1964, yet its existence as a true pathophysiological Received 28 June 2009 entity remains controversial. It may present as an acute or chronic disease, typically affecting young, Received in revised form multiparous women. This review discusses the literature to date on this poorly recognised cause of 23 September 2009 ureteric obstruction and pelvic pain, including developments in the diagnosis and management of this Accepted 25 September 2009 eminently treatable condition. Available online 8 October 2009 Ó 2009 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.

Keywords: Ovarian Vein Syndrome Chronic Pelvic Pain Congestion Ureteric Obstruction

1. Review criteria chronic variants occur most commonly in multiparous women. Several pathophysiological mechanisms have been suggested. Clark Using Embase and Medline, a literature search was undertaken. postulated that an aberrant ovarian vein, which might arise from English and foreign language articles on the ovarian vein syndrome persistent embryological posterior subcardinal branches, exerts were appraised. Chronic pelvic pain, pelvic congestion syndrome occlusive pressure on the ipsilateral .1 By crossing the ureter and ureteric obstruction were key terms, which were also searched. at the level of the , where its position is relatively fixed, In 1964, Clark published a series of 129 cases of right-sided ovarian rather than at the usual more cephalad level of L3/L4, Clark felt the vein syndrome in which he purported that an aberrant ovarian vein aberrant vessel is more likely to cause ureteric compression. He also was the cause of an obstructive uropathy, occurring at the level of the described aberrant ovarian as being much larger than normal, pelvic brim.1 Since then, several others have published case reports of more likely to branch into a number of distal tributaries, and more the ovarian vein syndrome (OVS),2–7 but the largest contemporary likely to drain into the right , all of which he felt could series includes only eight cases.7 That the syndrome truly exists as explain the phenomenon of right-sided OVS (Fig. 1). a distinct pathophysiological entity remains controversial. It is often Dykhuizen described a sheath of at the pelvic encompassed within a larger collection of disorders, which share the brim, which appeared to be a retroperitoneal continuation of the unifying feature of chronic pelvic pain. suspensory ovarian ligament, encasing both ovarian vessels and ureter. This, he surmised, contributed to ureteric fixity secondary to 5 2. Pathophysiology periureteral fibrosis. Radiographically he observed that, in sus- pected right-sided OVS, the ovarian vein fixed the right ureter as OVS may present as an acute affliction during or soon after they crossed, whilst the right moved with respiration. , or as a chronic, recurring disease. Both acute and Others believe that the ureter becomes trapped within a fibrovas- cular mesh of tortuous dilated veins.3 Alternatively, pressure from the gravid may cause ovarian * Corresponding author. Tel.: þ44 07773 420 717. vein dilatation and valvular incompetence. The dilated ovarian E-mail address: [email protected] (H.Y. Bhutta). veins then compress the ureter against the external iliac or

1743-9191/$ – see front matter Ó 2009 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.ijsu.2009.09.008 H.Y. Bhutta et al. / International Journal of Surgery 7 (2009) 516–520 517

Fig. 1. Normal of ovarian (1), veins (2) and (3) demonstrated on left. Right- image demonstrates an aberrant, enlarged right ovarian vein (4), compressing the right ureter (5) between itself and the external iliac vessels (6). psoas muscle. However, persistent, significantly elevated venous observation that it primarily affects pre and peri-menopausal pressures are needed for the ovarian veins to feasibly occlude women.1,6,7 However, that oophorectomy does not seem to be a thick-walled muscular ureter. Such pressures are not achieved, a successful treatment for OVS weakens the hormonal hypothesis.1 even during pregnancy.8 Dure-Smith argued that whilst the pres- More rarely, an ovarian may develop as a result of sure of the gravid uterus itself may suffice to occlude the ureters, it back-pressure from the inferior vena cava or left renal vein. An seems unlikely that ovarian vein pressure could achieve this. example of this is the ‘’, in which the left renal Quoting studies demonstrating a 60-fold increase in flow vein is compressed between the and the superior mesenteric through the ovarian veins during pregnancy, and a 3-fold increase artery.15 in venous diameter, but a comparatively small rise in pressure in Historically, the right ureter is involved in OVS more commonly these capacitance vessels,9 he argued that ovarian veins could only than the left.1,4 This might be explained by its proximity to the iliac reach pressures high enough to occlude the ureters during labour, vessels, and the course of the right ovarian vein. The right ovarian or with transient activities such as the valsalva manoeuvre. He vein usually forms a direct anastamosis with the inferior vena contested that post-partum, the ovarian veins collapse, so post- cava.8 Aberrant veins draining into the right renal vein might be pregnancy ureteric dilatation caused by the ovarian veins seems responsible for the development of OVS.1,7 However, post-mortem implausible. He also felt that the presence of a connective tissue examinations show that the left ovarian vein, which usually drains sheath encasing vessels and ureter was dubious, and not described into the left renal vein, is twice as likely as the right to be valve- by anyone other than Dykhuizen, although subsequently, this less,16 and the left vein usually expands to a greater degree than the observation has been corroborated.10 Canine experiments repro- right both during and after pregnancy.17 These observations suggest ducing ovarian vein dilatation by ligating other main veins have that the left ovarian vein is more susceptible to becoming varicose. failed to reproduce ureteric obstruction.11 In Dykhuizen’s series, Incidental ovarian varices are present in 10–47% of females.18,19 extirpated ovarian veins demonstrated medial wall thickening, although others have found the vessels to be histologically normal.3 Hormonal changes associated with pregnancy may also explain 3. Clinical features OVS. Altered levels of circulating oestrogen and progesterone could affect the muscular ureteric wall, causing a decrease in tone that OVS is uncommon in nulliparous women.1,7 Symptoms are facilitates its compression.12,13 In Dykhuizen’s series, oral proges- variable and non-specific, including , particularly in togen appeared to exacerbate symptoms, presumed to be due to the iliac fossae, flanks and hypochondrium. The pain tends to be increased ovarian vein blood flow.5 Oestrogen may account for the positional, and is worse lying down on the affected side. It is often development of pelvic varicosities in the pelvic congestion cyclical, peaking shortly before menstruation.1,7 Urinary symptoms syndrome, via nitric-oxide mediated vascular from ureteric obstruction include recurrent urinary tract infections, relaxation.14 Similarly, oestrogen/progesterone imbalances during hydronephrosis, , and frank haematuria. pregnancy are a putative biological mechanism leading to ovarian Whilst antibiotic therapy effectively treats infection, OVS pain vein dilatation and OVS. The hormonal hypothesis is strengthened is refractory, requiring more definitive treatment.5 Despite by the trend for symptoms of OVS to be cyclical, and the ureteric obstruction, there are few data regarding effects on renal 518 H.Y. Bhutta et al. / International Journal of Surgery 7 (2009) 516–520 function, unless there is a pre-existing anatomical renal tract historically been regarded as the gold standard investigation in abnormality.20,21 OVS, clearly identifying and demonstrating retro- Given the non-specific features of OVS, the differential diagnosis grade blood flow.It is especially useful if considering ovarian vein is broad. Gynaecological conditions such as endometriosis, pelvic embolization. Ovarian veins>10 mm in diameter are accepted as inflammatory disease and salpingitis must be considered. Ovarian being varicose on .27 vein , a condition which typically presents in the Ovarian varicosities may also be apparent on contrast-enhanced first few days post-partum with iliac fossa pain, fevers, and often computed tomography (CT) or magnetic resonance imaging (MRI). a pelvic or abdominal mass should be excluded if such features are MRI is preferable as it avoids the large radiation dose associated present. with CT, which should be avoided where possible in young women. Additionally, contrast-enhanced MRI permits images to be obtained 3.1. Ovarian vein syndrome vs. pelvic congestion syndrome in arterial and venous phases, though this is being superseded by magnetic resonance venography,28 which may actually identify the OVS should be distinguished from Pelvic Congestion Syndrome cause of ovarian . For PCS, an ovarian vein (PCS). Whilst sharing a similar pathology (dilated ovarian veins), diameter 8 mm on CT or MRI is considered diagnostic.29 This affecting a similar demographic (women of child-bearing age), and criterion might equally be applied to identify ovarian varicosities in responding to similar surgical treatment (venous embolization/ OVS. Up to 47% of women may have ovarian vein dilation observed ligation), the clinical manifestation is different. OVS is attributed to incidentally on scans performed for other reasons.19 Use of MRI for dilated ovarian veins only, whilst PCS is thought to be a manifes- OVS follow-up post-embolization with coils is limited by artefact. tation of dilatation of the entire anastomotic network of pelvic Laparoscopy may also have a diagnostic and therapeutic role, veins (ovarian, uterine, iliac), often with distal extension to involve particularly in excluding other aetiologies, but is not typically a first lower limb veins22 . line investigation. Pelvic pain is the commonest indication for PCS is characterised by cyclical pelvic pain, often preceding the diagnostic laparoscopy, but no diagnosis is made in the majority of onset of menstruation. It typically, but not exclusively, begins cases.30 Studies to compare the sensitivity and specificities of these during pregnancy in multiparous women, when the gravid uterus various investigations in either the ovarian vein syndrome, or the causes pressure on, with subsequent dilatation of, the ovarian more well known pelvic congestion syndrome, have not been veins.23 A dull ache and a sensation of perineal heaviness persist published. Whilst ultrasonography is clearly user-dependent, CT beyond pregnancy, and vascular congestion spreads distal to the and MRI may both underestimate the presence of varicose veins as ovarian veins giving rise to vulval and lower limb varicosities, with they collapse whilst the patient is supine during CT and MR congestion of the pelvic organs. Pain is often postural, being worse imaging. on standing and eased on lying down, and patients complain of dyspareunia and post-coital pain.24 Urinary symptoms are infre- 5. Treatment quent, with urinary frequency and urgency being the most preva- lent features. On clinical examination, there is commonly point 5.1. Medical therapy tenderness over the region of the and cervical excitation. By contrast, OVS is typified by a constellation of urinary symptoms. Medical therapies with medroxyprogesterone acetate (MPA) as Extrinsic compression of the ureters such as tumour compres- a method of causing venous contraction, and with goserelin acetate sion or retroperitoneal fibrosis, renal colic, and infections e.g. (gonadotrophin releasing hormone analogue), have been used in tuberculosis, are all possible differentials, as are general surgical the treatment of chronic pelvic pain although long-term relief emergencies such as appendicitis. Neurological and musculoskel- seems limited.31 In a randomised trial comparing MPA directly with etal disorders should also be considered. Consequently, many feel goserelin acetate in the treatment of women with PCS, 12 months that OVS is ultimately a diagnosis of exclusion.20 after the completion of treatment, goserelin acetate was found to be more effective than MPA, both subjectively, reducing 4. Diagnostic testing pelvic symptoms and anxiety, and radiologically, reducing pelvic congestion on venography.32 Recently, the sub-dermal eto- First line investigations include both abdominal and trans- norgestrol implant (ImplanonÒ) has shown promise in treating vaginal ultrasonography to assess the genitourinary tract, and PCS-related pelvic pain, with follow-up at 12 months showing principally to exclude mass lesions, which may be accountable significant reduction in pain scores and improved venographic for the presenting symptoms. An ovarian vein diameter 6mm appearances compared with no treatment.33 on scan indicates an ovarian varicosity,25 while duplex scanning may demonstrate reversed or reduced flow. These findings form part of the imaging criteria for a diagnosis of 5.2. Radiological therapy PCS, but they may equally be applied to OVS. However, Park et al. found that the right ovarian vein was not identified with duplex Ovarian vein embolization has been used for many years as scanning in 90% of subjects.26 Intravenous urography typically a treatment for OVS resistant to medical therapy. Coil embolization, demonstrates a hydroureter, with a clearly demarcated trans- first described in 1993,34 and now percutaneous chemical scle- verse defect at the L3/4 level. The renal and calyces may be rotherapy with Gelfoam35 or sodium tetradecylsulphate35,36 can be spared,4 and the middle third of the ureter may be laterally offered on an out-patient basis as less invasive options than displaced, with medial displacement of a normal calibre pelvic surgery. Short-term success from embolization therapy is esti- ureter.5 Marked improvement in hydroureter may be seen on mated at 80–98%.37–39 Longer-term efficacy, as observed in PCS, post-operative intravenous urogram, in keeping with a clinical also appears promising.40 Technical success rates from embolo- improvement in many case reports.3,4,10 therapy for the treatment of varices in PCS have been measured at Secondary investigations include retrograde pyelography, which 98–100%, and follow-up at 12 months has shown a mean reduction demonstrates a similar ureteric appearance as seen on intravenous in pain scores of 65%.41 Side- effects of embolization include urography, with an often normal pelvic ureteric segment, thrombophlebitis, recurrent disease, and embolic material and delayed drainage.4 Percutaneous ovarian venography has occluding non-targeted veins. H.Y. Bhutta et al. / International Journal of Surgery 7 (2009) 516–520 519

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