INTRODUCTION P CS is a distinct clinical entity in relatively young multiparous women characterised by chronic pelvic pain in the setting of pelvic venous varicosities. The syndrome, first described as a vascular condition by Taylor in 1949 1 was more recently shown by Hobbs 2 to be the result of venous engorgement of the pelvis due to gross dilatation and incompetence of one or both of the ovarian veins. In a series of fifty 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 reflux in 71% of cases, more often the left than the right (24:9). These cases could well be described as The Female Varicocoele. Sapheno-femoral 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 reflux alone. The latter probably accounts for at least 10% of the cases of pelvic congestion syndrome. In addition, it seems likely that segmental pelvic vein reflux accounts for a further 10% of cases. Vulval varicose veins are said to occur in 2 to 7% of pregnancies 3, 4 . These become larger in subsequent pregnancies, although they often disappear in the post partum period. Usually after 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 after 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. Perhaps segmental reflux occurs in tributaries of the internal iliac system 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 reflux. We have often demonstrated this segmental reflux within the broad ligament veins and round ligament veins in our pelvic ultrasound assessment. Compression syndromes are a further cause of left ovarian vein reflux in some patients, particularly superior mesenteric artery compression of the left renal vein and retro aortic left renal vein with compression. Compression of the left common iliac vein by the right common iliac artery can produce internal iliac reflux. 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, or posteriorly into the buttock and posterior thigh. These 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 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 Giacomini vein. Address correspondence and reprint requests to: Dr Graeme Richardson, 10 Docker Street Wagga Wagga NSW 2650 4ustralia. Review A R T I C L E PELVIC CONGESTION SYNDROME: Diagnosis & Treatment G D RICHARDSON, T C BECK, M MYKYTOWYCZ A F LENNOX Riverina Cardiovascular & Physiology Centre 51 A USTRALIAN & N EW Z EALAND J OURNAL OF P HLEBOLOGY V OLUME 3(2):November 1999 Pelvic Congestion Syndrome (PCS) is still treated with scepticism by the medical community, yet in most instances is “The Female Varicocoele”. Diagnosis requires knowledge of typical symptoms & signs. It is often the reason for recurrent varicose veins in the lower limbs due to failure to diagnose & treat pelvic escape veins through the vulva or buttock. Careful vascular ultrasound will reveal large varicosities in the pelvis which fill on elevation of the patient. Skilled ultrasound can confirm left or right ovarian or internal iliac vein incompetence. Occasionally, laparoscopy is needed to exclude endometriosis or other pelvic pathology and confirm the pelvic varices. Selective venography in many centres confirms the diagnosis of ovarian vein incompetence which can then be treated by using endovascular ablation. A ten year experience with surgical ovarian vein ablation has proved very successful as judged by over one hundred patients. It may still prove to be the treatment of choice and can be performed by any general surgeon aware of important technical aspects. Laparoscopic treatment has been investigated, but probably is less effective. Endovascular coil ablation and sclerotherapy, we believe, is now the treatment of choice for this condition and also for male varicocoele. ABSTRACT pp51 - 56
Transcript
I N T R O D U C T I O N
PCS is a distinct clinical entity in relatively youngmultiparous women characterised by chronic pelvic
pain in the setting of pelvic venous varicosities. Thesyndrome, first described as a vascular condition by Taylorin 19491 was more recently shown by Hobbs 2 to be the resultof venous engorgement of the pelvis due to gross dilatationand incompetence of one or both of the ovarian veins. In aseries of fifty symptomatic patients with either pelvic orvulval varicose veins assessed by our ultrasound techniquesin Wagga Wagga, the cause was found to be ovarian veinreflux in 71% of cases, more often the left than the right(24:9). These cases could well be described as The FemaleVaricocoele. Sapheno-femoral tributaries were the onlycause of vulval varicose veins in approximately 10% ofcases, and the remainder were assumed to be caused byinternal iliac reflux alone. The latter probably accounts forat least 10% of the cases of pelvic congestion syndrome. Inaddition, it seems likely that segmental pelvic vein refluxaccounts for a further 10% of cases.
Vulval varicose veins are said to occur in 2 to 7% ofpregnancies3, 4. These become larger in subsequentpregnancies, although they often disappear in the postpartum period. Usually after three pregnancies somevaricose veins remain in the vulva, upper medial thigh,perianal or gluteal regions. Probably the majority of casesare related to massive enlargement of the ovarian veinsdraining the pregnant uterus, perhaps associated withinternal iliac vein compression. Perhaps after pregnancysome ovarian veins do not return to normal size, and thelimited one or two valves at the upper end of the ovarianveins may become incompetent. Perhaps segmental refluxoccurs in tributaries of the internal iliac system such as theuterine veins, and the round ligament veins, and can beresponsible for persisting pelvic varicosities, even though weare unable to demonstrate ovarian vein or main trunkinternal iliac vein reflux. We have often demonstrated thissegmental reflux within the broad ligament veins and roundligament veins in our pelvic ultrasound assessment.
Compression syndromes are a further cause of leftovarian vein reflux in some patients, particularly superiormesenteric artery compression of the left renal vein andretro aortic left renal vein with compression. Compressionof the left common iliac vein by the right common iliacartery can produce internal iliac reflux.
If large pelvic veins persist in the broad ligament, typicalpelvic symptoms occur. Associated with these varicositiesthere may be pelvic escape through either the internal iliactributaries, namely obturator or internal pudendal, or theround ligament into the vulva and upper medial thigh, orposteriorly into the buttock and posterior thigh. Theseveins usually feed into either the long or short saphenoussystem, and if these are not treated at the time of treatmentof long or short saphenous varicose veins, then they causerecurrent varicose veins. A typical pattern is posteriorvulval veins coursing posteriorly into the short saphenousvia the Giacomini vein.
Address correspondence and reprint requests to: Dr GraemeRichardson, 10 Docker Street Wagga Wagga NSW 2650 4ustralia.
ReviewA R T I C L E
PELVIC CONGESTIONSYNDROME:Diagnosis & Treatment
G D RICHARDSON, T C BECK, M MYKYTOWYCZ
A F LENNOX
Riverina Cardiovascular & Physiology Centre
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Pelvic Congestion Syndrome (PCS) is stilltreated with scepticism by the medical community,yet in most instances is “The Female Varicocoele”.Diagnosis requires knowledge of typical symptoms& signs. It is often the reason for recurrent varicoseveins in the lower limbs due to failure to diagnose &treat pelvic escape veins through the vulva or buttock.Careful vascular ultrasound will reveal largevaricosities in the pelvis which fill on elevation ofthe patient. Skilled ultrasound can confirm left orright ovarian or internal iliac vein incompetence.Occasionally, laparoscopy is needed to excludeendometriosis or other pelvic pathology and confirmthe pelvic varices.Selective venography in many centres confirms thediagnosis of ovarian vein incompetence which canthen be treated by using endovascular ablation.A ten year experience with surgical ovarian veinablation has proved very successful as judged byover one hundred patients. It may still prove to bethe treatment of choice and can be performed byany general surgeon aware of important technicalaspects. Laparoscopic treatment has beeninvestigated, but probably is less effective.Endovascular coil ablation and sclerotherapy, webelieve, is now the treatment of choice for thiscondition and also for male varicocoele.
ABSTRACT
pp51 - 56
The diagnosis of PCS is often delayed until investigationslooking for endometriosis, inflammatory bowel disease,urinary tract disease or pelvic inflammatory disease haveproved negative, resulting in psychological stresses,marital disharmony and much doctor dissatisfaction.Various methods have been used to treat the symptoms ofpelvic congestion, including psychotherapy, ovariansuppression5, intravenous dihydroergotamine6, andbilateral oophorectomy with hysterectomy7. Ovarian veinligation has been performed to elimate reflux forapproximately fifteen years, as either a bilateral procedure(Lechter8, Hobbs2), or unilateral based on ultrasoundassessment (Richardson) . Long term results of suchtreatment has been poorly investigated. It is importantthat any assessment of treatment of venous conditionsneeds to have at least five year follow up. Of recent yearshowever, endovascular ablative techniques have beenpopularised in an uncontrolled fashion, and again need tobe adequately assessed.
D I A G N O S I S
Clinical suspicion of PCS relies on typicalsymptoms, namely pelvic heaviness or deeppelvic pain, which is present before the periodand on Day 1 and sometimes Day 2 ofmenstruation, mid cycle and post coital. Thelatter is particularly noticeable on standing upimmediately after having had morningintercourse. This aching may persist for severalhours through the day. Many patients complainof dyspareunia and many are aware of vulvaland leg varicosities which are worse at the timeof their pelvic symptoms. In addition, pelviccramps can occur, but are non specific.Commonly there are bladder symptoms relatedto peri vesical varicosities causing frequency ora difficulty in starting the flow of urine. Manypatients have symptoms of irritable bowelsyndrome. It is common for patients to havesuffered marital stress and dissatisfaction withtheir treating doctor’s lack of interest in theircondition.
I N V E S T I G AT I O N S
All patients with symptoms consistent with PCS arecarefully examined to exclude other causes of pelvicpathology, and then undergo standard pelvic ultrasoundand duplex ultrasound assessment of the ovarian, pelvicand, when appropriate, groin and lower limb veins.Earlier reports have advocated venography todemonstrate pelvic varices, either by use of vulvalvaricography9, trans uterine10, 11, per osseous12 venographyor selective ovarian venography13,14. These techniques areinvasive and may, in some cases, invalidate assessment forreflux. For example, if a catheter is selectively placedadjacent to or inside the orifice of the right or left ovarianvein, it may pass the only valve present, and injection will
then demonstrate “reflux”. More recently, MRI has beenused to detect pelvic varices 15 in the assessment ofchronic pelvic pain, but this method does not giveinformation about ovarian, iliac or groin vein reflux.
U l t r a s o u n d A s s e s s m e n t
The diagnosis of PCS is confirmed by finding excessivepelvic varicose veins in the broad ligament which we wouldgrade as mild, moderate or marked depending on thediameter of these broad ligament veins, and when thepatient is tilted head up by 600 on a motorised ultrasoundexamination table, these pelvic varicosities are found todistend. This assessment is best carried out by trans vaginalultrasound. Our ultrasound assessment begins with thepatient presenting after six hours fasting, but with a fullbladder. The fasting is to reduce gut motility, and the fullbladder enables the usual pelvic ultrasound assessment.The full bladder however compresses pelvic varicositiesand, after emptying the bladder, these may then be seen bytrans abdominal ultrasound. Trans vaginal ultrasound then
follows and, having confirmed the diagnosis, we turn ourattention to the ovarian veins and the internal iliac veins,including its anterior and posterior divisions. The roundligament veins and sapheno femoral tributaries are alsoassessed.
In Wagga Wagga, ultrasound windows were developed toassess ovarian vein incompetence using trans abdominaland trans vaginal duplex ultrasound scanning and colourflow doppler (3.5 or 5 MHz transducer) 16. A previous studyby us demonstrated the ability to locate ovarian veins andassess reflux in 93% of cases17, which compares well with the92% visualisation shown by Lechter using venography. Thecriterion for incompetence in the ovarian vein is reverseflow either lying, sitting or standing without augmentation.Venography has rarely been used by us to confirm thediagnosis when treatment is by surgery. However, we now
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Figure 1: Duplex Ultrasound left ovarian vein reflux
proceed to endovascular treatment with priorinformed consent at the time of venography, butour treatment is still based on the ultrasounddiagnosis.
L a p a r o s c o p y
Laparoscopy is sometimes required to excludeother possible causes for pelvic pain, such asendometriosis or pelvic inflammatory disease. Inour hands, this is in combination with thepatient’s gynaecologist, and may precede surgicaltreatment, having confirmed ovarian vein refluxby laparoscopy. This involves using an extra leftiliac fossa port to retract the sigmoid colon andthe patient, who initially is head down forgynaecological laparoscopy, is then tilted headup and the ovarian and broad ligament veins areseen to distend rapidly if reflux is present.
T R E AT M E N T
As many of these patients have associated legvaricosities, a plan of treatment is required. The pelvic veinsare only treated if there are pelvic symptoms, or if it isconsidered imperative to obtain a good long term result fortreatment of the leg varicosities if there is no other sourcefound for the leg varices. In these cases the pelvic varicoseveins are treated initially, and the response of symptoms isdetermined over a period of two to three months beforetreating the vulval or leg varicosities. In a few instances, the
veins can reduce in size such that sclerotherapy of theresidual vulval veins or leg veins might be appropriate,rather than surgical treatment.
1 ) O v a r i a n Ve i n I n c o m p e t e n c e
As most cases involve treatment of the left ovarian vein,the choice of treatment is between surgical operation andendovascular ablation techniques. Laparoscopic treatment
has been investigated, and although it is possible toclip the upper end of the ovarian veins, it is currentlynot possible to remove a segment, nor would it beeasy to deal with nearby tributaries. Thelaparoscopic technique has been developed by LesNathanson in Brisbane.
a ) O p e r a t i o n
Ovarian vein ligation has been carried out verysuccessfully for over ten years, and the primaryauthor has treated one hundred and twenty patientsby this method. It involves a retroperitonealapproach using a sympathectomy incision withmuscle splitting approach to the ureter and then theovarian vein, which is first ligated using nonabsorbable material at the level of the pelvic brim. Itinvolves gentle techniques of ligation and theligature is then used for traction to enable furthermultiple ligations upwards to finish at approximately2 cms from the left renal vein.A narrow Dever retractor is very useful to exposethis uppermost portion. There is significant risk ofmajor haemorrhage if the ovarian vein is not treatedcarefully. Operation requires approximately twodays in hospital and two weeks of discomfort, whichfor a young lady with several children proves aconsiderable inconvenience when compared with thepossibility of endovascular treatment as anoutpatient without any restriction of normalactivities. When considering the most appropriate
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Pelvic Congestion Syndrome
Figures 2: DSA Image of coils in left ovarian vein
Figures 3: Catheter in left ovarian vein for Sclerotherapy
method of ovarian vein ablation, one has to consider thefact that the surgical operation is complete and, providedthe tributaries have all been ligated, should produce a longterm ablation of the ovarian vein. It can be carried out byany general surgeon and requires no special equipment. Itdoes however produce a scar and the discomfort associatedwith operation.
S u r g i c a l R e s u l t s
Long term results in a series of seventy two patientstreated until June 1995 in Wagga Wagga, certainlyencourages one to treat patients based on the ultrasoundfindings of ovarian vein reflux. These patients were sentquestionnaires and were assessed independently by asurgical registrar for their response of symptoms to surgicaltreatment using a visual analogue scale
18to quantify their
symptoms. Sixty seven of the seventy two patientsresponded with a mean follow up of thirty three months(range 4 to 71) with a mean age of thirty five years andmean pregnancies of 3.1. Pelvic heaviness was found toimprove significantly (>50%) in 70% of patients and in 56%of patients, this was almost complete. 13% reported little orno improvement, and when these were subsequentlyinvestigated, including further ultrasound and venography,no ovarian reflux could be found, and in all casesalternative symptoms, such as irritable bowel syndromewere present. Dyspareunia was present pre operatively in82% of cases and 84% of these improved, 50% of thesereported complete recovery. Post intercourse pelvic achingwas present in 75% of patients and improved in 70% ofcases with 64% having complete recovery. Bladdersymptoms of frequency and obstruction improved in 45%of patients, and some of the 20% of patients who preoperatively were aware of bowel spasm had improvement.Two patients had normal pregnancies subsequent toovarian vein ligation with no development of vulval veins inthe pregnancy and no recurrence of symptoms.
b ) O v a r i a n E n d o v a s c u l a r A b l a t i o n
There have been several reports of single or a few casereports of successful treatment by ovarian veinembolisation, (Edwards 199319, Sichlau 199420, Boomsma199821, Cordts 199822.) Thus far, there has been nostandardisation of the techniques used by several centresbut, in all instances, coils of various diameters and lengthshave been used. In some centres, sclerotherapy has beenused and in the Dutch 21 experience, sclerotherapy wascontraindicated because of risk of entering the portalsystem. A team in Vancouver, which has a very largeexperience of treatment of male varicocoele using the sametechniques has utilised a combination of coils and glue(personal communication).
Since January 1999 in Wagga Wagga, we have been usingendovascular techniques whenever possible. In one patient,failure to catheterise the lower portion of the ovarian veinassociated with spasm led to her decision to have surgicaltreatment, rather than a further attempt via trans jugularapproach. Our learning curve has been steep. We nowwould use a guiding catheter and still use the groin, ratherthan a jugular approach. In all other cases, we have beensuccessful, in now over twenty cases, in treating the leftovarian vein in females and, in three cases, the testicularvein in males, one of whom had recurrent varicocoele. Thetechnique we have used in Wagga Wagga thus far involvesuse of stainless steel coils with attached synthetic fibres(Cook), choosing a diameter aiming to oversize slightly thesize of the ovarian vein. In addition, sclerosant has beenused with 3% Aethoxysclerol diluted with a small amountof contrast so that the spread of sclerosant can be clearlyseen on the screen to avoid spill over into the left renal vein.In no instances have we seen any contrast pass beyond theovarian vein or broad ligament veins.
Our approach has been via the right femoral vein in thesupine position. Having confirmed theultrasound diagnosis of ovarian vein reflux by aselective left renal venogram, a guidewire ispassed down the ovarian vein to the pelvis, and acatheter advanced to the level of the pelvic brim.Approximately one third of the 2 mls ofsclerosant is injected slowly, with the patientholding her breath as long as possible and addedValsalva manoeuvre, and, in male patients,combining it with compression at the level of theexternal ring to avoid the sclerosant passing intothe scrotum. The first coil is placed atapproximately the level of the iliac crest, a furtherinjection of sclerosant is used, then a further oneto three coils are placed, depending on theposition of any tributaries. The uppermostportion of the top coil is placed as close aspossible to the renal vein, which is usuallyapproximately 2 cms away. Where there have
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been large tributaries, these have been separatelycatheterised and smaller coils inserted together withsclerosant to avoid collateralisation . In Cordts’ series fromHawaii, between six and twenty four coils have been used.Each coil costs approximately $50.00, and our intention isto use as few coils as possible to satisfy the principles oftreatment being high ligation and ablation of as much ofthe length of the ovarian vein and the tributaries aspossible.
There are risks to endovascular techniques including
embolisation of coils, possible migration of coils and
penetration and irritation of nerves, such as genito femoral,
and the possibility of late recanalisation. There have
certainly been reports of recanalisation resulting in
recurrent symptoms requiring later surgical treatment.
Long term results of endovascular treatment have not been
assessed, and will need to be compared with our long term
surgical results. We are currently critically evaluating our
endovascular technique, including follow up ultrasound
assessment and venography.
2 ) I n t e r n a l I l i a c Ve i n s
Where patients are shown to have significant internal iliac
vein reflux 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
in our series and by others23. There are risks to the
surrounding structures, such as ureter and iliac vessels, and
it is not simple surgery. There are significant risks to
endovascular treatment of the internal iliac system, being a
very large vein at its junction with the external iliac vein.
The shape of the vein encourages embolisation. In one case,
we have employed a coil into the anterior division together
with sclerotherapy.
3 ) Vu l v a l Va r i c o s i t i e s
Having treated the ovarian vein, these are usually not
troublesome, and can be treated by either 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 the residual minor
vulval varicosities is possible, and the primary author has,
on many occasions, used 2 to 3% Aethoxysklerol. The
difficulty is in applying adequate compression after the
sclerotherapy. It is my practice to employ cotton balls
covered by tape and then ask the patient to wear a firm
support, such as step-ins or bicycle pants, in an attempt to
provide as much compression as practical. The side effects
from the sclerotherapy have been surprisingly few despite
the problem of inadequate compression.
R E F E R E N C E S
1. Taylor, HC; Wright, H; Vascular Congestion and Hyperaemia, Am J Obst
Gynecol 1949, 57: 211-230
2. Hobbs, JT; The Pelvic Congestion Syndrome, British Journal of Hospital
Medicine March 1990, Vol 43, pp 200-206
3. Dodd, H; Wright, AP; Vulval varicose veins in pregnancy, Br Med J 1959;
1:831-2
4. Dixon, JA; Mitchell, WA; Venographic and surgical observations in vulvar
varicose veins, Journal of Surgery, Gynaecology and Obstetrics September
