View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector International Journal of Surgery 7 (2009) 516–520 Contents lists available at ScienceDirect International Journal of Surgery journal homepage: www.theijs.com Review Ovarian vein 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 Ovarian Vein 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 ureter.1 By crossing the ureter and ureteric obstruction were key terms, which were also searched. at the level of the pelvic brim, 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 veins 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 renal vein, 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 connective tissue 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 kidney moved with respiration. pregnancy, 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 uterus 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 artery 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 anatomy of ovarian arteries (1), veins (2) and ureters (3) demonstrated on left. Right-hand 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 varicocele 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 ‘nutcracker syndrome’, in which the left renal Quoting studies demonstrating a 60-fold increase in blood flow vein is compressed between the aorta 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 abdominal pain, 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 smooth muscle from ureteric obstruction include recurrent urinary tract infections, relaxation.14 Similarly, oestrogen/progesterone imbalances during hydronephrosis, pyelonephritis, renal colic 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 varicose veins 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 venography.27 vein thrombophlebitis, 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