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Table31.1 THE VENOUS CLINICAL SEVERITYSCORE
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CHARACTERISTIC ABSENT=0 MILD=1 MODERATE=2 SEVERE=3
Pain None Occasional/nonanalgesia
Varicose Veins > 4mm None Few Multiple GSV Extensive GSV and SSV
Venous Edema None Evening/Ankle A ernoon/Above knee Morning/requiring elevation
Skin Pigmentation None Limited/Brown Di use lower 1/3/purple Wide/purple
In ammation None Mild cellulitis in
Induration None Focal <5cm Medial or lateral less
Number of active ulcers None 1 2 3
Active ulcer duration <3months >3months <12months > 12months
Active ulcer diameter None <2cm 2–6cm >6cm
Compression None or
noncompliant
restricting
marginal area
Intermittent use Compression stockings
With moderate activity/
analgesia use
Moderate involving
most ofgaiter area
than lower 1/3
worn most days
Daily pain, limitations
to activities or regular analgesia
Severe cellulitis or signi cant
eczema
1/3 of leg or more
Compression stockings
worndaily
Reliability is the degree to which measurements on the same individual are similar under di erent conditions. Test-retest comparisons are the most appropriate method for assessing reliability if the instrument is intended as an evaluative tool. Reliability can also be assessed using inter­nal consistency; this checks the extent to which similar questions give consistent replies.
Responsiveness considers whether the tool is sensitive to assess measurable change. If meaningful comparisons are to be made then a standardized measure of responsiveness is required.  e standardized response mean represents the mean change in score over two points in time divided by the standard deviation of the score di erences and allows such a
14
comparison.
SYSTEMSPECIFICTOOLS
CLINICAL SCORING SYSTEMS
Consistent and accurate diagnosis and classi cation of clini­cal signs and symptoms are required before posttreatment responses can be analyzed.  e CEAP (for clinical, etiologi­cal, anatomical, pathophysiological) classi cation, originally developed in 1994, was included in the international recom­mendations for the reporting standards in venous disease
15
published in 1995.
It was further modi ed in 2004 16 and is available in several major languages and has now been adopted worldwide by the vascular community.  e CEAP classi cation is purely descriptive and is not a quanti able scoring system, nor is it sensitive to changes following inter­vention, and for this reason the Venous Clinical Severity Score (VCSS), Venous Segmental Disease Score (VSDS), and Venous Disability Score (VDS) were designed, based
17
on the CEAP classi cation.
 e VCSS is a simple clinical
scoring system is based around ten clinical domains with a potential maximum score of 30 (see Table31.1). It has been used in the assessment of treatment outcomes and in clinical
18,19
trials.
It is currently under revision, in order to modify the language with the aim of further evaluating the criteria in certain categories to make it more easily applicable to patients without a ecting the sensitivity, and therefore mak­ing it an easier tool for clinicians to use as part of their routine
20
practice as well as in clinical reserach.
 e VSDS assesses the anatomical and pathophysiological components of the CEAP.  e scores are allocated based on re ux or obstruc­tion observed in eleven venous segments on venous imag­ing—usually color duplex.  e VDS is a score ranging from 0 to 3 based on the degree of impairment to daily activities and reliance on compression. At present the VSDS and VDS are infrequently used in clinical practice and clinical trials.
In order to fully evaluate and compare di erent treat­ments or results from di erent publications, there is the need for standardization of the severity of venous disease, both from a clinical and function perspective.
Currently there are several system- or disease-speci c instruments for measuring health-related quality of life in patients with varicose veins or chronic venous disease of the lowerlimb.
SYSTEMSPECIFIC
QUESTIONNAIRES
A B E R D E E N V A R I C O S E V E I N
QUESTIONNAIREAVVQ
 e Aberdeen Varicose Vein uestionnaire was originally
21
designed by Garratt etal. as a postal questionnaire.
It con­sists of thirteen questions relating to varicose veins includ­ing observable signs, symptoms experienced, the use of
248 • PRIMARY SUPERFICIAL VENOUS INSUFFICIENCY
compression hosiery, the e ect of varicose veins on daily
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activities, and concerns regarding cosmesis, scores range from 0 (no disease) to 100 (severe disease).  e original paper surveyed 373 patients with varicose veins selected from a hospital and general practice setting. Acomparison was made with 900 members of the general population, selected randomly from the electoral register in Aberdeen, who were sent a similar questionnaire without the condition-speci c tool. Ahigh correlation with the SF36 generic health pro le con rmed the validity of the questionnaire and illustrated that the perceived health of patients with varicose veins was
21
signi cantly lower than that of the general population.
In 1999 the AVVQ was shown to be responsive to changes fol­lowing surgery in a cohort of 137 consecutive patients with
22
primary varicose veins.
Based on data collected from the AVVQ, SF36, and twenty- ve questions that focused on symptoms and concerns, it was concluded that the AVVQ was a valid measure of quality of life for patients pre- and postsurgery. It also con rmed that patients had a signi cant improvement in quality of life following surgery. Since its introduction, the AVVQ has become a well-established and valid tool for measuring quality of life and has been used in numerous clinical trials and cohort studies to assess improvements following surgery and endovenous thermal
18,23–26
ablation procedures in patients with varicose veins.
At
present it is validated in Englishonly.
CHRONIC VENOUS INSUFFICIENCY
QUESTIONNAIRE CIVIQ
 e original version of the CIVIQ was developed from a cross-sectional observational study in over 2,000 patients, of whom over 50% had a diagnosis of venous insu ­ciency based on clinical signs and reported symptoms.  e CIVIQ2 was devised following a second analysis using a questionnaire of 20 equally weighted questions on the 1,001 patients with venous disease based on 4 criteria including physical, psychological, and social concerns as well as pain.  e CIVIQ2 questionnaire has been shown to be appropri­ate, speci c, and reliable for the assessment of chronic lower
27
limb venous insu ciency.
And since its original publica­tion in 1996 it has been shown to be reliable and responsive in the assessment of patients with chronic venous insu ­ciency, following venous out ow stenting in a study to 870
28
patients
and has also been successfully used to evaluate improvement following endovenous thermal ablation pro­cedures for varicose veins in a number of clinical trials.
29,30
In recent years its use has becoming increasingly popular.
THE CHARING CROSS VENOUS
ULCERATION QUESTIONNAIRE
 C X V U Q 
 e CXVUQ was designed to assess quality of life speci ­cally in patients with venous leg ulceration. An ulcer-speci c
questionnaire was designed with questions relating to physi­cal discomfort, the e ects on daily activities and social activ­ities, emotional consequences, and perspectives regarding dressings and mobility. It was validated with the SF36 in a
31
group of ninety-eight patients,
it has found to be reliable and responsive to treatment in this patient group, however is not designed for use in patients without venous ulceration.
VENOUS INSUFFICIENCY
EPIDEMIOLOGY AND ECONOMIC
STUDY  V EINS
 e Veins uestionnaire was developed over 10years from an international prospective cohort study of 5,688 outpa­tients with chronic venous disease, that evaluated epidemi­ological factors and outcomes.  e questionnaire consists of two separate categories:  e VEINES quality-of-life questionnaire (VEINES QoL) consists of twenty- ve items relating to the impact of chromic venous disease on quality of life.  e VEINES symptom questionnaire (VEINS-SYM) consists of ten questions evaluating symp­toms. Together they form a questionnaire of thirty- ve di erent items with two summary scores. It was originally validated in English, French, French Canadian, and Italian and has been shown to be acceptable, reliable, valid, and responsive in patients with deep venous thrombosis. has also been shown to correlate with the SF36 and the
33
CEAP
classi cation as further evidence of its validity, and in a study of 1,313 patients the VEINES QoL was found to be more sensitive to quality-of-life changes associated with varicose veins in combination with venous disorders than
34
the SF36.
It was also suggested that in patients with vari­cose veins alone, cosmetic concerns and quality of life/relief of symptoms should be considered separately. In addition to the assessment of deep venous thrombosis the VEINES questionnaire has been used to evaluate a wide spectrum of venous disorders from telangiectasia, to varicose veins, edema and skin changes, and leg ulceration in patients from the original VEINES study population and responsiveness in an additional 1516 patients following treatment, where treatment outcomes were correlated with quality of life.
SPECIFIC QUALITY OF LIFE AND
OUTCOME RESPONSEVENOUS
SQORV
 e SQOR-V questionnaire was developed by a French-American collaboration and published in 2007.  e aim was to develop a patient-reported outcome mea­sure that would fully evaluate symptoms, impairment to activities, cosmetic concerns, and the psychological impact of the disease including concerns regarding risk to health.  e authors proposed that existing disease-speci c tools were insu ciently sensitive to fully evaluate venous symp­toms experienced by patients with supposedly mild disease
32
It
35
36
USE OF SYSTEMSPECIFIC QUESTIONNAIRES • 249
of clinical CEAP classC0–C3, and designed the question-
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naire in order to evaluate this particular group of patients. Forty-six questions were carefully composed to evalu­ate symptoms, with a rating scale of 1 to 5 instead of yes/ no answers in order to improve accuracy and sensitivity. uestions are divided into 5 domains, each with a possible total score of 20, giving a possible range of scores from 20 (no disease) to 100 (severe disease). It was originally devel­oped in English and then translated into French, in which it was initially validated, along with the SF12 and a Center for Epidemiologic Studies-Depression scale (CES-D) in a group of 202 patients. It has been shown to have internal consistency, reducibility, structural validity, convergent
36
validity, and clinical validity
and has now been validated in several other languages including English and Spanish. To date there have been few published studies of the SQOR-V, however early data collected in an English-speaking popula­tion suggest that it is responsive to change following endo­venous thermal ablation treatments in patients of C2–C4 disease, and correlates with the AVVQ and VCSS scores.
37
Larger studies are awaited to support it’s widespread use and superior sensitivity in this target patientgroup.
DIFFICULTIES WITH
DISEASESPECIFIC QUALITY
OFLIFE
following their intervention.  e data is collected centrally and will be used in the clinical evaluation and assessment of the performance of treatment centers and research into the
40
clinical and cost-e ectiveness of treatments.
C O N C L U S I O N
 e AVVQ was one of the  rst disease-speci c tools designed for the evaluation of varicose veins and remains the most popular to date, however, a number of newer question­naires are gaining in popularity. At present there is no single tool that can be used in isolation, and consideration should be given to the population under investigation. With the introduction of rationing for venous interventions, accurate and appropriate measurement of outcomes to support the cost-e ectiveness of treatments are of paramount impor­tance.  e cost-e ectiveness of treatments is calculated in quality-adjusted life years (QALYs), a measure of disease burden and at present is calculated based on generic quality of life tools. In the future the assessment of the disease bur­den attributable to chronic venous disease is likely to become increasingly important in order to justify the allocation of resources, and therefore the need arises for the development of a disease speci c tool in order to assess disease burden.
Because the symptoms of venous insu ciency are notori-
38,39
ously di cult to determine and assess,
 nding a single outcome measure that has su cient scope to encompass the wide spectrum of symptoms experienced at the extremes of venous disease while remaining sensitive enough to evaluate improvements following intervention in those with milder disease is challenging, and at present no single solution exists. At present the majority of published clinical studies rely on a number of di erent disease-speci c, generic, and clinical-outcome measures, making comparison among dif­ferent techniques and patient groups di cult. In recent years there has been a move away from the use of surrogate out­come measures of anatomical and hemodynamic function to evaluate treatments and toward functional and in particular disease-speci c, quality-of-life outcomes, which are though to be more truly representative of the patients’ experience.  e routine use of patient-reported outcome measures has several drawbacks, including being time-consuming and open to bias from patients and physicians, however they are likely to gain increasing popularity in the future. Already in 2009 Patient Reported Outcome Measures (PROMs) for hip and knee replacements and in hernia and varicose vein surgery have been introduced in many NHS trusts in the UK. Patients are requested to complete a preoperative dis­ease speci c and generic quality-of-life questionnaire. For varicose veins this is based on the EQ-5D and the AVVQ and they are then sent a postal questionnaire at 3months
R E F E R E N C E S
1. http://www.hscic.gov.uk/hes . Main Procedures and Interventions. 2008–2009.
2. Database updates from the International Venous Registry (IVR). March 2009 ; Inpress .
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4. Lindsey B , Campbell WB . Rationing of the treatment of varicose veins and the use of new treatment methods:Asurvey of practice in the United Kingdom , Eur J Vasc Endovasc Surg. 2006 . 32 ( 4 ): 472 .
5. Shepherd AC , Gohel MS , Lim CS , Hamish M , Davies AH . Endovenous ablation for varicose veins:Overtaking or overrated?, Phlebology. 2010 . 25 : 38–43 .
6. Winterborn RJ , Corbett CR . Treatment of varicose veins: e pres­ent and the future:Aquestionnaire survey , Ann R Coll Surg Engl. 2008 . 90 ( 7 ): 561–564 .
7. Evans CJ , Allan PL , Lee AJ , Bradbury AW , Ruckley CV , Fowkes FG . Prevalence of venous re ux in the general population on duplex scan­ning: e Edinburgh vein study , J Vasc Surg. 1998 . 28 ( 5 ): 767–776 .
8. Lim CS , Gohel MS , Shepherd AC , Davies AH . Secondary care treatment of patients with varicose veins in National Health Service England:At least how it appeared on a National Health Service web­site , Phlebology. 2010 . 25 ( 4 ): 184–189 .
9. Michaels JA , Campbell WB , Brazier JE , etal. Randomised clinical trial, observational study, and assessment of cost-e ectiveness of the treatment of varicose veins (REACTIV trial) , Health Technol Assess. 2006 . 10 ( 13 ): 1–196 , iii–iv .
10. Gohel MS , Barwell JR , Earnshaw JJ , etal. Randomized clinical trial of compression plus surgery versus compression alone in chronic venous ulceration (ESCHAR study):Haemodynamic and anatomi­cal changes , Br J Surg. 2005 . 92 ( 3 ): 291–297 .
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11. Ratcli e J , B r a z ie r J E , Ca mp b e l l W B , P a l f r e y ma n S , M a c I nt y r e J B ,
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Michaels JA . Cost-e ectiveness analysis of surgery versus conser­vative treatment for uncomplicated varicose veins in a randomized clinical trial , Br J Surg. 2006 . 93 ( 2 ): 182–186 .
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13. Kundu S , Lurie F , Millward SF , et al. Recommended reporting standards for endovenous ablation for the treatment of venous insu ciency: Joint statement of the American Venous Forum and the Society of Interventional Radiology , J Vasc Surg. 2007 . 46 ( 3 ): 582–589 .
14. Katz JN , Larson MG , Phillips CB , Fossel AH , Liang MH . Comparative measurement sensitivity of short and longer health sta­tus instruments , Med Care. 1992 . 30 ( 10 ): 917–925 .
15. Porter JM , Moneta GL . Reporting standards in venous disease:An update: International Consensus Committee on Chronic Venous Disease , J Vasc Surg. 1995 . 21 ( 4 ): 635–645 .
16. Eklof B , Rutherford RB , Bergan JJ , etal. Revision of the CEAP clas­si cation for chronic venous disorders:Consensus statement , J Vasc Surg. 2004 . 40 ( 6 ): 1248–1252 .
17. Rutherford RB , Padberg FT Jr., Comerota AJ , Kistner RL , Meissner MH , Moneta GL . Venous severity scoring: An adjunct to venous outcome assessment , J Vasc Surg. 2000 . 31 ( 6 ): 1307–1312 .
18. Darwood RJ ,  eivacumar N , Dellagrammaticas D , Mavor AI , Gough MJ . Randomized clinical trial comparing endovenous laser ablation with surgery for the treatment of primary great saphenous varicose veins , Br J Surg. 2008 . 95 ( 3 ): 294–301 .
19. Vasquez MA , Wang J , Mahathanaruk M , Buczkowski G , Sprehe E , Dosluoglu HH .  e utility of the Venous Clinical Severity Score in 682 limbs treated by radiofrequency saphenous vein ablation , J Vasc Surg. 2007 . 45 ( 5 ): 1008–1014 ; discussion1015.
20. Vasquez MA , Munschauer CE . Venous Clinical Severity Score and quality-of-life assessment tools: Application to vein practice , Phlebology. 2008 . 23 ( 6 ): 259–275 .
21. Garratt AM , Macdonald LM , Ruta DA , Russell IT , Buckingham JK , Krukowski ZH . Towards measurement of outcome for patients with varicose veins , ual Health Care. 1993 . 2 ( 1 ): 5–10 .
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23. Mekako AI , Hat eld J , Bryce J , Lee D , McCollum PT , Chetter I . A nonrandomised controlled trial of endovenous laser therapy and surgery in the treatment of varicose veins , Ann Vasc Surg. 2006 . 20 ( 4 ): 451–457 .
24. MacKenzie RK , Paisley A , Allan PL , Lee AJ , Ruckley CV , Bradbury AW .  e e ect of long saphenous vein stripping on quality of life , J Vasc Surg. 2002 . 35
25. Rasmussen LH , Bjoern L , Lawaetz M , Blemings A , Lawaetz B , Eklof B . Randomized trial comparing endovenous laser ablation of the great saphenous vein with high ligation and stripping in patients with varicose veins:Short-term results , J Vasc Surg. 2007 . 46 ( 2 ): 308–315 .
( 6 ): 1197–1203 .
26. Rautio T , Ohinmaa A , Perala J , etal. Endovenous obliteration versus conventional stripping operation in the treatment of primary vari­cose veins:Arandomized controlled trial with comparison of the costs , J Vasc Surg. 2002 . 35 ( 5 ): 958–965 .
27. Launois R , Reboul-Marty J , Henry B . Construction and validation of a quality of life questionnaire in chronic lower limb venous insuf­ ciency (CIVIQ) , ual Life Res. 1996 . 5 ( 6 ): 539–554 .
28. Neglen P , Hollis KC , Olivier J , Raju S . Stenting of the venous out­ ow in chronic venous disease:Long-term stent-related outcome, clinical, and hemodynamic result , J Vasc Surg. 2007 . 46 ( 5 ): 979–990 .
29. Almeida JI , Kaufman J , Gockeritz O , etal. Radiofrequency endove­nous ClosureFAST versus laser ablation for the treatment of great saphenous re ux:Amulticenter, single-blinded, randomized study (RECOVERY study) , J Vasc Interv Radiol. 2009 . 20 ( 6 ): 752–759 .
30. Lurie F , Creton D , Eklof B , etal. Prospective randomized study of endovenous radiofrequency obliteration (closure procedure) versus ligation and stripping in a selected patient population (EVOLVeS Study) , J Vasc Surg. 2003 . 38 ( 2 ): 207–214 .
31. Smith JJ , Guest MG , Greenhalgh RM , Davies AH . Measuring the quality of life in patients with venous ulcers , J Vasc Surg. 2000 . 31 ( 4 ): 642–649 .
32. Kahn SR , Lamping DL , Ducruet T , et al. VEINES-QOL/Sym questionnaire was a reliable and valid disease-speci c quality of life measure for deep venous thrombosis , J Clin Epidemiol. 2006 . 59 ( 10 ): 1049–1056 .
33. Kahn SR , M’Lan CE , Lamping DL , Kurz X , Berard A , Abenhaim LA . Relationship between clinical classi cation of chronic venous disease and patient-reported quality of life:Results from an interna­tional cohort study , J Vasc Surg. 2004 . 39 ( 4 ): 823–828 .
34. Kurz X , Lamping DL , Kahn SR , etal. Do varicose veins a ect qual­ity of life? Results of an international population-based study , J Vasc Surg. 2001 . 34 ( 4 ): 641–648 .
35. Lamping DL , Schroter S , Kurz X , Kahn SR , Abenhaim L . Evaluation of outcomes in chronic venous disorders of the leg:Development of a scienti cally rigorous, patient-reported measure of symptoms and quality of life , J Vasc Surg. 2003 . 37 ( 2 ): 410–419 .
36. Guex JJ , Zimmet SE , Boussetta S , Nguyen C , Taieb C . Construction and validation of a patient-reported outcome dedicated to chronic venous disorders: SQOR-V (speci c quality of life and outcome response:venous) , J Mal Vasc. 2007 . 32 ( 3 ): 135–147 .
37. Shepherd AC , Gohel MS , Lim CS , Hamish M , Davies AH .  e use of disease-speci c quality-of-life tools in patients with varicose veins: Abstract published from American College of Phlebology 22nd Annual Congress, Marco Island, FL, November 6–9, 2008. Phlebology. 2009 . 24 : 89 .
38. Bradbury A , Evans C , Allan P , Lee A , Ruckley CV , Fowkes FG . What are the symptoms of varicose veins? Edinburgh vein study cross sec­tional population survey , Br Med J. 1999 . 318 ( 7180 ): 353–356 .
39. Campbell WB , Decaluwe H , Boecxstaens V , etal.  e symptoms of varicose veins:Di cult to determine and di cult to study , Eur J Vasc Endovasc Surg. 2007 . 34 ( 6 ): 741–744 .
40. http://www.hscic.gov.uk/proms . Patient Reported Outcome Measures (PROMs). 2009 .
USE OF SYSTEMSPECIFIC QUESTIONNAIRES • 251
32.
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PELVIC CONGESTION SYNDROME
DIAGNOSIS AND TREATMENT
Graeme D. Richardson
INTRODUCTION
Pelvic congestion syndrome (PCS) is still treated with skep­ticism by the medical community, and in most instances is called “the female varicocoele.”
PCS is a distinct clinical entity in relatively young multiparous women characterized by chronic pelvic pain in the setting of pelvic venous varicosities.  e syndrome,  rst described as a vascular condition by Taylor in 1949 was more recently shown by Hobbs venous engorgement of the pelvis due to gross dilatation and incompetence of one or both of the ovarian veins. In a series of   y symptomatic patients with either pelvic or vulval varicose veins assessed by our ultrasound techniques in Wagga Wagga, the cause was found to be ovarian vein re ux in 71% of cases, more o en the le than the right (24:9).  ese cases could well be described as the female varicocoele. Saphenofemoral tributaries were the only cause of vulval varicose veins in approximately 10% of cases, and the remainder were assumed to be caused by internal iliac re ux alone.  e latter probably accounts for at least 10% of the cases of PCS. In addition, it seems likely that segmental pelvic vein re ux accounts for a further 10% of cases. Many patients with recurrent leg varicose veins are found to have a signi cant component of their problem from the pelvis. Seeking symptoms of PCS, a history of vulval varicose veins of pregnancy, and looking for a contribution from the pelvis in all patients presenting with leg varicosities will result in a greater awareness of a common yet poorly understood clini­cal problem.
E T I O L O G Y
Although rarely seen in nulliparous teenagers and young women, when one may assume the cause is identical to male varicocoele, this condition largely follows pregnancy. Vulval varicose veins are said to occur in 2 to 7% of pregnancies.  ese become larger in subsequent pregnancies, although
2
to be the result of
1
3,4
they o en disappear in the postpartum period. Usually a er three pregnancies some varicose veins remain in the vulva, upper medial thigh, perianal, or gluteal regions. Probably the majority of cases are related to massive enlargement of the ovarian veins draining the pregnant uterus, perhaps associated with internal iliac vein compression. Perhaps a er pregnancy some ovarian veins do not return to normal size, and the limited one or two valves at the upper end of the ovarian veins may become incompetent. Maybe segmen­tal re ux occurs in tributaries of the internal iliac veins such as the uterine veins, and the round ligament veins, and can be responsible for persisting pelvic varicosities, even though we are unable to demonstrate ovarian vein or main trunk internal iliac vein re ux. We have o en demonstrated this segmental re ux in our pelvic ultrasound assessment.
Compression syndromes are a further cause of le ovar­ian vein re ux, particularly superior mesenteric artery com­pression of the le renal vein and retro aortic le renal vein with compression. Compression of the le common iliac vein by the right common iliac artery can produce internal iliac re ux.
While hormonal and psychiatric factors have at times been implicated in the symptomatology, exacerbation of symptoms with menstruation, sexual activity, and ovulation suggests increased arterial  ow to the pelvis at these times results in pooling of venous blood in the pelvic varicosities.  is results in pressure in the pelvis alone, or if there are pel­vic escape veins some or all of the pressure is transmitted to the vulva, buttock, or leg varicosities.
If large pelvic veins persist in the broad ligament, typi­cal pelvic symptoms occur. Associated with these varicosi­ties there may be pelvic escape through either the internal iliac tributaries, namely obturator or internal pudendal, or the round ligament into the vulva and upper medial thigh (Figure32.1A), or posteriorly into the buttock and posterior thigh, sometimes including varices of the vein of the sciatic nerve, producing sciatica.  ese veins usually feed into either the long or short saphenous system, and if these are not treated at the time of treatment of long or
252
A B
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Figure32.1 ( A) Residual vulval varices. (B)Vulval to posterior thigh to lateral calf varices.
invalidate assessment for re ux. For example, if a catheter is selectively placed adjacent to or inside the ori ce of the right or le ovarian vein, it may pass the only valve present, and injection will then demonstrate “re ux.” Varicography can demonstrate the anatomy of vulval and buttock varices and the relevant pelvic escape veins, but not the physiology of re ux (Figure32.2). More recently, magnetic resonance imaging (MRI) and multislice computed tomography (CT)
11
have been used to detect pelvic varices
in the assessment of chronic pelvic pain. Dynamic MRI techniques are currently being developed in Madrid (personal communication) that can show ovarian vein re ux but will need to be compared with ultrasound techniques for cost and reliability.
short saphenous varicose veins, then they cause recurrent varicose veins. Atypical pattern is posterior vulval veins coursing posteriorly into the short saphenous via the vein of Giacomini (Figure32.1B).
DIAGNOSIS
Clinical suspicion of PCS relies on typical symptoms, namely pelvic heaviness or deep pelvic pain, which is present before the period, on Day 1 and sometimes Day 2 of menstruation, midcycle, and post coitus.  e latter is particularly noticeable on standing up immediately a er having had morning inter­course.  is aching may persist for several hours through the day.  e pelvic heaviness is particularly severe a er long peri­ods of standing. Many patients complain of dyspareunia and many are aware of vulval and leg varicosities that are worse at the time of their pelvic symptoms. Commonly there are bladder symptoms related to perivesical varicosities causing frequency or a di culty in starting the  ow of urine. Many patients have symptoms of irritable bowel syndrome.
 e diagnosis of PCS is o en delayed until investiga­tions looking for endometriosis, in ammatory bowel dis­ease, urinary tract disease, or pelvic in ammatory disease have proved negative. It is common for patients to have su ered marital stress and dissatisfaction with their treating doctor’s lack of interest in their condition.
ULTRASOUND ASSESSMENT
PCS is con rmed on transvaginal ultrasound by  nding excessive pelvic varicose veins in the broad ligament, which we would grade as mild (<5mm), moderate (5–7mm), or marked (8–10mm), depending on the diameter, and whether these pelvic varicosities are found to distend when the patient is tilted head up by 60 degrees on a motorized ultrasound examination table. Our ultrasound assessment begins with the patient presenting a er 6 hours of fasting and with a full bladder. Fasting reduces gut motility, and the full bladder enables standard gynecological pelvic ultrasound. Afull blad­der however compresses pelvic varicosities, which may be visi­ble by transabdominal ultrasound a er voiding. Transvaginal ultrasound then follows, and having con rmed PCS, we examine the ovarian veins and the internal iliac veins, includ­ing anterior and posterior divisions.  e round ligament veins and saphenofemoral tributaries are also assessed.
I N V E S T I G A T I O N S
All patients with symptoms consistent with PCS are care­fully examined to exclude other causes of pelvic pathology, and then undergo standard pelvic ultrasound and duplex ultrasound assessment of the pelvic, ovarian, and when appropriate, groin and lower limb veins. Earlier reports have advocated venography to demonstrate pelvic varices, either by use of vulval varicography,
8
periosseous
venography, or selective ovarian venography.
 ese techniques are invasive and may, in some cases,
5
transuterine venography,
PELVIC CONGESTION SYNDROME:DIAGNOSIS AND TREATMENT • 253
9,10
6,7
Figure32.2 Varicogram of vulval varices with round ligament and obturator pelvic escapeveins.
In Wagga Wagga, windows were developed to assess
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ovarian vein incompetence using transabdominal duplex ultrasound and color  ow Doppler (3.5 or 5 MHz trans-
12
ducer). veins and assess re ux in 93% of cases, well with the 92% visualization shown by Lechter
We demonstrated the ability to locate ovarian
13
which compares
14
using venography.  e le ovarian vein is found by  rst locating the le renal vein as it passes under the superior mesenteric artery.  e ultrasound window is through the le lobe of the liver and the pancreas.  e ovarian vein is located by follow­ing the le renal vein laterally and rotating the transducer through 90 degrees (Figure32.3A). Aretroaortic le renal vein, duplicated renal vein, or large ureteric veins are noted if present. It is important not to confuse accessory renal veins or the inferior mesenteric vein for the ovarian vein.  e right ovarian vein is found using a window through the liver or gallbladder, by following the inferior vena cava upward to where the right ovarian vein enters it anterolater­ally at a very acute angle. Sampling by color and wave form is taken about 2cm below the termination of the ovarian veins (Figure32.3B).  e criterion for incompetence in the ovarian vein is reversed  ow when lying down, sitting, or standing without augmentation. Treatment either by sur­gery, or more recently endovascular methods, is based on the ultrasound  n d i n g s .
L A P A R O S C O P Y
Laparoscopy is sometimes required to exclude other pos­sible causes for pelvic pain, such as endometriosis or pelvic in ammatory disease in patients who have pelvic varices on ultrasound assessment. We do this with the patient’s gyne­cologist. Laparoscopy involves using an extra le iliac fossa port to retract the sigmoid colon.  e patient, who initially is head down for gynecological laparoscopy, is then tilted head up, and the ovarian and broad ligament veins are seen to distend rapidly if re ux is present.
V E N O G R A P H Y
Many centers rely on clinical  ndings, then proceed to selec­tive venography for con rmation, and then to endovascu­lar treatment. Ultrasound con rmation of excessive pelvic varicose veins by transvaginal ultrasound, even if ultrasound assessment for ovarian vein re ux is not possible, should prevent unnecessary invasive venography, and assist in pro­vision of informed consent, should a patient be referred to an interventional radiologist for venography with a view to coils with or without sclerotherapy. Le renal venography is followed by selective ovarian venography (Figures 32.4 and32.5). If indicated by ultrasound  ndings, we may do selective iliac venograms.
T R E A T M E N T
Various methods have been used to treat the symptoms of pelvic congestion, including psychotherapy, ovarian sup-
15
pression, oophorectomy with hysterectomy.
intravenous dihydroergotamine, 16 and bilateral
17
Ovarian vein ligation
has been performed to eliminate re ux since 1985, as either
14
a bilateral procedure (Lechter,
Hobbs 2 ), or unilateral based on ultrasound assessment (Richardson 1989).  e long-term results of such treatment have been poorly inves­tigated. It is important that any assessment of treatment of venous conditions have at least a 5-year follow-up. In recent years, however, endovascular ablative techniques have been popularized and similarly must be adequately assessed.
As many of these patients have associated leg varicosi­ties, a treatment plan is required.  e pelvic veins are only treated if there are pelvic symptoms, or if they signi cantly contribute to the leg varicosities. In these cases the pelvic veins are treated initially, and the response of symptoms is determined over a period of 2 to 3months before treating the vulval or leg varicosities. In a few instances, the veins can reduce in size such that sclerotherapy of the residual
A B
Figure32.3 (A) Ultrasound le ovarian vein (red) and le renal vein (blue). (B)Ultrasound le ovarian vein and le renal vein with waveform showing re ux.
254 • PRIMARY SUPERFICIAL VENOUS INSUFFICIENCY
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Figure32.4 L e  renal venogram with re ux into a large le ovarian vein. Note the narrow upper end, which helps prevent embolization ofcoils.
vulval or leg veins might be appropriate, rather than surgical treatment.
Ovarian Vein Incompetence
As most cases involve treatment of the le ovarian vein, the choice of treatment is between surgery and endovascular ablation techniques. Laparoscopic treatment has been inves­tigated, and although it is possible to clip the upper end of the ovarian veins, it is currently not possible to remove a seg­ment, nor would it be easy to deal with nearby tributaries.
Surgery
Ovarian vein ligation has been performed on 120 patients
since 1989 by the author. It involves a “sympathectomy”
Figure32.5 Selective le ovarian venogram  lling large le broad ligament varices, with crossover to the right broad ligament, and drainage via the right ovarian vein and both iliac veins, also showing presacral veins.
incision with a muscle-splitting extraperitoneal approach to the ureter and the adjacent ovarian vein, which is ligated carefully using nonabsorbable material at the level of the pelvic brim.  e ligature is then used for traction to enable further multiple ligations upward to  nish at approximately 2cm from the le renal vein. Anarrow Dever retractor is useful to expose this uppermost portion.  ere is signi cant risk of major hemorrhage if the ovarian vein is not handled gently.  is surgery requires approximately 2days’ hospi­talization and 2 weeks’ discomfort, which for a mother of young children is a considerable inconvenience compared with outpatient endovascular treatment. When choosing the most appropriate method of ovarian vein ablation, we accept that surgical ligation is complete, and provided all tributaries have been ligated, should produce long-term ablation of the ovarian vein. It can be performed by any general surgeon and requires no special equipment. It does however produce a scar and discomfort. Ishould reiterate that Iwould only ligate an ovarian vein that was shown to re ux on ultrasound assessment. Other surgeons have rou-
14,2
tinely ligated both ovarian veins.
It would seem unwise to ligate a draining vein that did not re ux. Some surgeons have advocated an even more extensive dissection to include
18
the ovarian pedicle.
 ere is no evidence to suggest a more limited operation such as the Wagga Wagga technique has inferior results.
S U R G I C A L R E S U L T S
Long-term results in a series of seventy-two patients treated until June 1995 in Wagga Wagga certainly encourage one to treat patients based on the ultrasound  ndings of ovar-
19
ian vein re ux.
 ese patients were sent questionnaires and were assessed independently by a surgical registrar for their quantitative response of symptoms to surgical treat-
20
ment using visual analogue scales.
Sixty-seven of the seventy-two patients responded with a mean follow-up of thirty-three months (range 4 to 71) with a mean age of thirty- ve years and mean pregnancies of 3.1. Pelvic heavi­ness was found to improve signi cantly (>50%) in 70% of patients, and in 56% of patients this was almost complete.  irteen percent reported little or no improvement, and when these were subsequently investigated, including fur­ther ultrasound and venography, no ovarian re ux could be found, and in all cases alternative diagnoses such as irritable bowel syndrome were present. Dyspareunia was present preoperatively in 82% of cases, and 84% of these improved; 50% of these patients reported complete recovery.
Postintercourse pelvic aching was present in 75% of patients and improved in 70% of cases, with 64% having complete recovery. Bladder symptoms of frequency and obstruction improved in 45% of patients, and some of the 20% of patients who preoperatively were aware of bowel spasm improved. Two patients had normal pregnancies subsequent to ovarian vein ligation with no development
PELVIC CONGESTION SYNDROME:DIAGNOSIS AND TREATMENT • 255
of vulval veins in the pregnancy and no recurrence of
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symptoms.
Ovarian Endovascular Ablation
 ere have been several reports of single or a few case
reports of successful treatment by ovarian vein emboliza-
21–24
tion.
 us far, there has been no standardization of the techniques used by several centers, but in all instances coils of various diameters and lengths have been used. In some centers sclerotherapy has been used, but in the
23
Dutch
experience, sclerotherapy was contraindicated because of a perceived risk of entering the portal system. Ateam in Vancouver, which has a very large experience of treatment of male varicocoele using similar techniques, has utilized a combination of coils and glue (personal communication).
Since January 1999 we have been using endovascular techniques. We prefer to use an inguinal approach, and when cannulation of the ovarian vein is di cult, would use a guiding catheter and still use the groin, rather than a jugular or brachial approach. Our technique uses stainless steel coils with attached synthetic  bers (Cook), choos­ing a diameter to oversize by 2 to 3mm the ovarian vein diameter. In addition, sclerosant has been used with 2 ml of 3% aethoxysklerol diluted with about 1 to 2 ml of con­trast so that the spread of sclerosant can be clearly seen on the screen to avoid spillover into the le renal vein. Air is added and the syringe shaken to produce coarse bubbles. Our hope is that the sclerosant will help obliterate pelvic and broad ligament varices. By causing spasm we may help prevent migration of the coils. In no instances have we seen any contrast pass beyond the ovarian vein or broad ligament veins. While preparing the sclerosant as a foam would seem desirable, the contrast is further diluted and less visible than with coarse bubbles, and we are less sure of its spread. In an attempt to reduce the cost to the patient, we have tried to use the minimum number of coils to achieve the following principles.  e  rst coil is deployed at the level of the pel­vic brim just above where it crosses the ureter. Depending on the anatomy of the ovarian vein, we aim to place a coil across junctions or selectively coil major tributaries. We try to have good cross-section coverage of the vein by vary­ing the deployment, and we aim to have the highest point above all incompetent tributaries, and within 2 to 3cm of the le renal vein. Usually two long (20-cm) coils su ce, with occasional shorter coils in tributaries or at the upper end of the vein. We are aiming for the highest and longest possible ablation.
Our approach has been via the right femoral vein and having con rmed ovarian vein re ux by a selective le renal venogram, a guide wire is passed down the ovarian vein to the pelvis, and a catheter advanced to the level of the pelvic brim. Approximately one-third of the 2 ml of sclerosant is injected slowly, with the patient holding her breath with Valsalva as long as possible. In male varicocoele
patients, this is combined with compression at the level of the external ring to avoid the sclerosant passing into the scrotum.
 ere are risks to endovascular techniques including embolization, migration and perforation of coils, irritation of nerves such as the genitofemoral, and the possibility of later recanalization.  ere have been reports of recanaliza­tion resulting in recurrent symptoms requiring later surgical treatment.
Internal IliacVeins
Where patients are shown to have signi cant internal iliac vein re ux as a cause for the pelvic congestion syndrome, surgical treatment to ligate the main branch or selectively the anterior division has been performed on a few patients
18
in our series and by others.
 ere are risks to the surround­ing structures, such as ureteric and iliac vessels.  ere are also signi cant risks to endovascular treatment of the internal iliac system, being a very large vein at its junction with the external iliac vein.  e shape of the vein encourages emboli­zation. In one case, we have deployed a coil into the anterior division together with sclerotherapy. When the patient has ovarian and internal iliac vein re ux on ultrasound assess­ment, we have only treated the ovarian vein.  us far we have not needed to treat the internal iliac vein because of a disappointing result.
Vulval Varicosities
Having treated the ovarian vein, these improve and can be treated by avulsion techniques by minor surgery, or at the time of dealing with the long or short saphenous vari­cosities. Large round ligament veins can be ligated as they emerge from the external inguinal ring. Sclerotherapy of residual minor vulval varicosities is possible, and the author has on many occasions used 2% aethoxysklerol. To apply adequate compression a er the sclerotherapy Iuse cotton balls covered by tape and the patient wears a  rm support, such as bicycle pants, in an attempt to provide as much compression as practical. Side e ects from the sclerotherapy have been surprisinglyfew.
Ureteric VeinRe ux
Inevitably unusual cases will appear associated with venous anomalies. We have treated several cases with large re ux­ing ureteric veins that are tortuous and feed into the ovar­ian vein usually in the lower third of the abdomen.  ey are o en di cult to cannulate for coil treatment. Sometimes the ovarian vein joins a lower renal vein branch, or a large lumbar vein rather than the renal vein, and sometimes the ovarian or the renal vein is duplicated. In all cases coming to endovascular treatment we have to be prepared for such anomalies and devise the best treatment strategy.
256 • PRIMARY SUPERFICIAL VENOUS INSUFFICIENCY
Other Causes of Pelvic Congestion
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Some patients present with congestion symptoms or minor vulval varices, yet we are unable to demonstrate ovarian or internal iliac incompetence. As with most venous disease, there is variability related to long periods of standing and the menstrual cycle. Repeat ultrasound studies show this, and we try to perform studies when symptoms are maximal.  ere remain patients in whom there clearly are signi cant pelvic varices but no source of re ux. Some of these are due to venous obstruction associated with collateral venous pathways. We have observed patients with both  xed and intermittent re ux of internal iliac veins associated with common iliac vein obstruction. In some cases this is pos­tural, when recumbent there is reversed  ow, and is associ­ated with 1- to 2-mm anteroposterior (AP) diameter where the right common iliac artery crosses the le common iliac vein. Perhaps some of these patients would bene t from a venous stent. Aretroaortic le renal vein has frequently been associated with PCS and rarely le renal vein obstruction following surgical ligation. Iavoid ablation of the ovarian vein in these patients with collateral drainage of thekidney.
Segmental Pelvic VeinRe ux
 ere remains a group of patients where we cannot dem­onstrate a de nite cause. It appears quite feasible that some very large pelvic veins in pregnancy don’t shrink and thus produce segmental re ux in uterine and broad ligament veins.  ese patients and others whose symptoms fail to resolve a er ovarian or iliac vein ablation, are best treated by hysterectomy.
COMPARISON OF
RESULTS OF COIL AND
SURGICAL TREATMENT OF
OVARIANR EFLUX
Patients treated by surgery from 1989 until 1998, and endo­vascular treatment from 1999 until June 2002 were studied using a questionnaire with visual analogue scales. Statistical analysis of pelvic heaviness and overall satisfaction showed no di erence between endovascular and surgical treat-
25
Both treatments resulted in statistically signi -
ment. cant improvement a er treatment. Adecision to treat in both groups was based on clinical  ndings and ultrasound assessment, and there was no statistical di erence in the presenting features of patients in either the surgical or the endovascular series.
Patients undergoing coil treatment were also subjected to follow-up ultrasound studies at 6 weeks and 6months as well as abdominal radiographs.  ere was no evidence of coil migration in thirty-four patients. Early ultrasounds showed two clots in broad ligament veins, no signi cant
reduction in diameter at 6 to 10 weeks, but some evidence of reduction by 6months.
Long-term results of endovascular treatment have not yet been reported. Recanalization remains possible but should be amenable to further endovascular treatment. While the great majority of patients tolerate coil treatment with little discomfort, with anxious patients it is more dif­ cult to cannulate the femoral vein, and spasm of the ovar­ian vein could lead to perforation. Patients have far less loin discomfort than a er surgery, but it seems excessive exer­cise should be restricted. Afew patients have severe pain, and this could be due to thrombosis of the ovarian vein or perforation.
Patient satisfaction justi es ablation of an ovarian vein shown by ultrasound to re ux.
Provided endovascular ovarian vein ablation can be delivered safely and at reasonable cost, then there are de ­nite advantages over surgical treatment. Complications can occur from either method.  e incidence of long-term recanalization is unknown.
 ere is no evidence that endovascular treatment pro­duces better results than surgery. Provided patients are pre­pared to accept the scar, pain, hospitalization, and other potential complications of an operation, at this point one cannot say surgical treatment has been superseded.
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