Pelvic Congestion Syndrome Sophia A. Virani, HMS IV Gillian Lieberman, MD Beth Israel Deaconess Medical Center Department of Radiology May 2009
Agenda
Introduce index patient
Discuss pelvic congestion syndrome Etiology Menu of radiologic tests Treatment options
Review pelvic vasculature
Companion Cases
Let’s meet our patient …
Ms. C: Initial presentation
Ms. C is a 57 year old G3P2 post-menopausal woman who presents with one year of left lower quadrant pain Nagging pain Worse at night Often presents late in the afternoon after prolonged periods of standing
Chronic Pelvic Pain: Overview
Pelvic pain lasting greater than 6 months Not cyclical or intercourse related Often refractory to analgesic therapy with narcotics
Estimated cause of 10-40% of outpatient gynecologist visits
Etiology often elusive Patients can have extensive workups including laparoscopy in search of cause
Differential diagnosis is broad and includes endometriosis, adhesions, fibroids and pelvic varicosities
As part of her workup for pelvic pain, Ms. C had a transvaginal ultrasound which showed dilated pelvic veins, compatible with pelvic congestion syndrome
Pelvic Congestion Syndrome (PCS): Overview
Overview
Pathophysiology
Dilated pelvic veins with retrograde flow Stretch of engorged veins causes pain Can cause mass effect with symptoms such as constipation and bladder incontinence Can be bilateral, but left more often affected than right
Etiology
Dilated gonadal and pelvic veins determined as cause of chronic pelvic vein Affects primarily multiparous women of childbearing age Symptoms include pelvic pain, dyspareunia, thigh and vulvar varices Controversial as a diagnostic entity Æ mostly a diagnosis of exclusion
Poorly understood Hormonal factors are a likely contributor given that pre-menopausal women are primarily affected Mechanical factors ► Absent or incompetent valves ► Left renal vein variants associated with a higher incidence of pelvic varices
Treatment
No established medical treatments Surgical treatments include pelvic vein embolization and laparoscopic ligation of ovarian veins
Pelvic Congestion Syndrome is both diagnosed and treated radiologically. Let’s take a look at the imaging options for PCS
Pelvic Congestion: Menu of Radiologic Tests Test
Key Findings
Ultrasound
Contrast CT / CTA
MRI / MRV
Direct Venography
Advantages
Dilated ovarian veins Pelvic varices > 5mm Reversed flow with Doppler
Dilated ovarian veins Tortuous pelvic varices
Dilated ovarian veins Pelvic varices often hyperintense on T2
Dilated veins Reflux
Disadvantages
Non invasive Readily available Can detect other causes of symptoms
Non operator dependent Can visualize vascular anatomy for possible etiology
Non invasive No contrast MRV becoming popular; can image pelvic venous system in 1 breath hold
Gold standard Can be done @ time of embolization
Operator dependent
Supine position can underestimate size of veins Contrast exposure Expense Patient comfort Supine position can underestimate size of veins Follow up limited for patients who have had embolization with metal coils Invasive Contrast
Ms. C: Dilated Pelvic Veins on TVUS * * * *
*
BIDMC PACS Sagittal Transvaginal Ultrasound images of left ovary with and without without Doppler
Markedly dilated pelvic veins adjacent to left ovary
BIDMC PACS
Pelvic Congestion: US Findings
Both transvaginal and transabdominal ultrasound can be used in evaluation of pelvic congestion syndrome
Non-invasive, often first-line imaging
Can identify other causes of pelvic pain
Findings include: Dilated ovarian vein > 5 mm Tortuous pelvic veins around ovary and uterus > 5 mm Dilated arcuate veins crossing uterine myometrium Slow or reversed (caudal) flow with Doppler Changes with Valsalva ► Increase
in size of varices
► Variable
duplex waveform
Ms. C: Dilation of Pelvic Veins on TVUS before Valsalva
BIDMC PACS
PrePre-Valsalva TVUS of left adnexa
Pre-Valsalva Dilated pelvic vein Diameter = 8.4 mm
Ms. C: Dilation of Pelvic Veins on TVUS with Valsalva
BIDMC PACS
PostPost-Valsalva TVUS of left adnexa
Post-Valsalva Dilated pelvic vein Diameter = 8.8 mm
Anatomy Review: Gonadal Veins
Gray Anatomy online; Accessed 5/19/2009. www.bartleby.com
Right ovarian vein drains into IVC Left ovarian vein drains into left renal vein Left renal vein courses anteriorly to aorta
Volume-rendered MDCT image of left and right ovarian veins. From Karaosmanoglu et. al. “MDCT of the Ovarian Vein: Normal Anatomy and Pathology.” AJR 192: January 2009
Ms. C: Dilated Ovarian Vein on MRI
dilated left ovarian vein measuring 9 mm prominent adnexal veins
BIDMC PACS
T2 Weighted MRI, Coronal MIP Reconstruction
Pelvic Congestion: MRI Findings
Multi-planar imaging allows good visualization of pelvic anatomy and vasculature
Appearance of varices: Flow voids on T1 Can be hypo-intense, iso-intense or hyper-intense on T2 depending on velocity of blood flow High signal intensity on gradient echo
Time of flight sequences can assess direction of blood flow Technique that optimizes signal from flowing blood and diminishes signal from stationary tissue
Ms. C: Retrograde Flow in Left Ovarian Vein on Time-of-Flight MRI BIDMC PACS
BIDMC PACS
C+ Limited axial MRI TOF images; arterial phase
aorta
left ovarian vein
IVC
Time-of-Flight demonstrates reversed flow in left ovarian vein left ovarian vein has flow in the same direction as aorta No flow seen in image selected for venous flow
C+ Limited axial MRI TOF images; venous phase
Ms. C: Clinical Course
Given the imaging findings, pelvic congestion syndrome was thought to be the etiology of Ms. C’s pelvic pain.
She elected to undergo pelvic vein embolization.
Pelvic Vein Embolization
Overview Transcatheter embolization using coils and/or sclerosing agents to occlude ovarian vein Venograms performed during procedure to verify anatomy and assess extent of collaterals ► Collaterals between ovarian and internal iliac venous plexus may cause recurrence of symptoms Can perform balloon embolization of internal iliac veins to visualize collaterals
Effectiveness Studies have demonstrated symptomatic relief in 70-80% of patients Long-term effects not well characterized ► Recent study by Kim et. al 127 patients with pelvic congestion syndrome who underwent embolization 83% reported clinical improvement at 4-year follow up
Risks Ovarian vein thrombophlebitis, recurrence of varices, radiation exposure, migration of embolic material
Areas of debate Unilateral vs. bilateral embolization Internal iliac embolization
Ms. C: Ovarian Venogram During Embolization Tip of catheter in left ovarian vein showing RETROGRADE flow toward ovary
Dilated and tortuous left ovarian vein and collaterals
Congestion of veins in pelvis
BIDMC PACS
Left ovarian venogram, venogram, digital subtraction image
Ms. C: Left Ovarian Vein Coiling
Amplatzer vascular occlusion device within left ovarian vein
BIDMC PACS
Fluoroscopy image of left ovarian vein embolization
www.amplatzer.com
Ms. C: Post-Embolization Venogram Pre-embolization left ovarian venogram
Post-embolization left ovarian venogram
Occluded left ovarian vein
BIDMC PACS
Left ovarian venogram, venogram, DSA
BIDMC PACS
PostPost-embolization ovarian venogram, venogram, DSA
Ms. C reported significant pain relief at her two-week follow up visit
Ms. C’s case demonstrated pelvic vein congestion without an identified anatomic etiology. Let’s move on to see features of pelvic congestion secondary to anatomic anomalies in two companion patients
Companion Patient #1: Dilated Ovarian Vein and Pelvic Varices on CT
42 year old G2P2 with six months of left lower quadrant pain and a normal ultrasound BIDMC PACS
BIDMC PACS
C+ axial CT of pelvis
pelvic varices C+ axial CT, coronal reconstruction
dilated left ovarian
Companion Patient #1: Left Renal Vein Compression on CT
SMA Left Renal Vein
Aorta
BIDMC PACS
C+ axial CT
Left renal vein compressed between aorta and SMA
Nutcracker Syndrome: Overview
Left renal vein (LRV) entrapment syndrome
Etiologies
LRV compressed between aorta and SMV ► “anterior” nutcracker phenomenon Retro-aortic LRV compressed between aorta and spinal cord ► “posterior” nutcracker phenomenon Circum-aortic LRV
Clinical consequences
Increased pressure between LRV and IVC ► Can cause rupture of small veins into collecting system Hematuria Flank pain Venous obstruction and symptoms of pelvic congestion syndrome
Treatment options
Surgery Stents ► Can cause fibromuscular dysplasia Embolization
Companion Patient #2: Pelvic Congestion due to Retro-aortic Left Renal Vein
retroaortic left renal vein, compressed
pelvic varices
Axial MIP CT images From: Koc et al. “Association of left renal vein variations and pelvic varices varices in abdominal MDCT.” Eur Radiology 2007: 17: 12671267-1274
Companion Patient #1 underwent ovarian vein embolization. Let’s take a look at the images from her procedure
Companion #1: Left Ovarian Venogram Pre- and Post-Embolization Pre-embolization ovarian venogram
Post-embolization ovarian venogram
dilated left ovarian vein
Coils Occluded left ovarian vein
BIDMC PACS Fluoroscopy prior to ovarian vein embolization
BIDMC PACS Fluoroscopy post to ovarian vein embolization
Companion Patient #1: Clinical Course
Companion Patient #1 continued to have pain after her embolization procedure. One possible explanation for her residual pain is the presence of large collateral pelvic varices.
She did not undergo internal iliac vein balloon occlusion during her procedure.
Let’s move on to see how internal iliac balloon occlusion can identify additional varices in a companion patient.
Anatomy Review: Pelvic Venous Plexus
When there is reflux in the ovarian vein, collateral vessels can form that drain into the iliac vein
Umeoka et. al “Vascular Dilation in the Pelvis: Identification with MR and CT Imaging.” Radiographics 2004: 24:193-208.
Companion Patient #3: Dilated Ovarian Varices on Venogram
38 year old multiparous woman with symptoms of pelvic congestion syndrome Bilateral ovarian venograms demonstrate dilated ovarian varices and some reflux across midline
Left ovarian venogram
Right ovarian venogram
Coiled left ovarian vein
Courtesy Dr. Tim Killoran Left ovarian venogram; venogram; DSA
Courtesy Dr. Tim Killoran Right ovarian venogram; venogram; DSA
Companion Patient #3: Bilateral Ovarian Vein Coiling
Left and right ovarian veins embolized with platinum coils
Courtesy Dr. Tim Killoran Fluoroscopy, bilateral ovarian veins
Companion Patient #3: Right Internal Iliac Vein Balloon Occlusion
Catheter in right internal iliac
Courtesy Dr. Tim Killoran Right internal iliac balloon occlusion venography; venography; DSA
Balloon occlusion of internal iliac vein prevents flow out of varices that drain into iliac vein
Allows visualization of additional collaterals that may cause recurrent symptoms
No significant varices identified on right internal iliac occlusion
Companion Patient #3: Left Internal Iliac Vein Balloon Occlusion
multiple pelvic varices
Courtesy Dr. Tim Killoran Left internal iliac balloon occlusion prepre-embolization venography, venography, DSA
Companion Patient #3: Clinical Course
Companion Patient #3’s left internal iliac balloon occlusion demonstrated significant pelvic collaterals.
She underwent left internal iliac embolization to treat these varices
Companion Patient #3: Venogram After Left Internal Iliac Embolization
Significant decrease in size of varices
Courtesy Dr. Tim Killoran Left internal iliac balloon occlusion postpost-embolization venography, venography, DSA
Summary Pelvic congestion syndrome is a potentially overlooked cause of chronic pelvic pain Radiologic diagnosis
Ultrasound, MRI/MRV and CT can demonstrate dilated ovarian veins and pelvic varices Venography is gold standard
Ovarian vein embolization is an effective and promising therapy for this condition
Acknowledgments
Jay Pahade, MD Tim Killoran, MD
Gillian Lieberman, MD Maria Levantakis
References 1.
2.
3. 4. 5.
6. 7. 8. 9. 10.
11.
12.
13.
Ahmed K, Sampath R, Khan MS. “Current Trends in the Diagnosis and Management of Renal Nutcracker Syndrome: A Review” European Journal of Vascular and Endovascular Surgery, Volume 31, Issue 4, April 2006, Pages 410-416 Asciutto G, Mumme A, Marpe B, Köster O, Asciutto KC, Geier B. "MR venography in the detection of pelvic venous congestion." Eur J Vasc Endovasc Surg. 2008 Oct;36(4):491-6. Epub 2008 Aug 20.Click here to read Belenky A, Bartal G, Atar E, Cohen M, Bachar GN. "Ovarian varices in healthy female kidney donors: incidence, morbidity, and clinical outcome" AJR Am J Roentgenol. 2002 Sep;179(3):625-7. Ganeshan A., Upponi S., et al. "Chronic Pelvic Pain due to Pelvic Congestion Syndrome: The Role of Diagnostic and Interventional Radiology." Cardiovasc Intervent Radiol (2007) 30:1105–1111 Hartung O, Grisoli D, Boufi M, Marani I, Hakam Z, Barthelemy P, Alimi YS. "Endovascular stenting in the treatment of pelvic vein congestion caused by nutcracker syndrome: lessons learned from the first five cases" J Vasc Surg. 2005 Aug;42(2):275-80. Karaosmanoglu et. al. “MDCT of the Ovarian Vein: Normal Anatomy and Pathology.” AJR 192: (1) 295. January 2009 Kaufman J and Lee M. “Pelvic Congestion Syndrome.” Chapter in Vascular and Interventional Radiology: The Requisites. Mosby, Philadelphia PA 2004. Kim HS, Malhotra AD, Rowe PC, et al. “Emblotheraphy for pelvic congestion syndrome: Long-term results.” J Vasc Interv Radiol: 17: 289-297 (2006) Koc Z, Ulusan S, Oguzkurt L. "Association of left renal vein variations and pelvic varices in abdominal MDCT." Eur Radiol. 2007 May;17(5):1267-74. Epub 2006 Oct 13. Koc Z, Ulusan S, Tokmak N, Oguzkurt L, Yildirim T. "Double retroaortic left renal veins as a possible cause of pelvic congestion syndrome: imaging findings in two patients." Br J Radiol. 2006 Oct;79(946):e152-5. Kwon SH, Oh JH, Ko KR, Park HC, Huh JY. "Transcatheter ovarian vein embolization using coils for the treatment of pelvic congestion syndrome." Cardiovasc Intervent Radiol. 2007 JulAug;30(4):655-61. Epub 2007 Apr 28. Park SJ, Lim JW, Ko YT, Lee DH, Yoon Y, Oh JH, Lee HK, Huh CY. “Diagnosis of pelvic congestion syndrome using transabdominal and transvaginal sonography”. AJR Am J Roentgenol. 2004 Mar;182(3):683-8. Umeoka S, Koyama T, Togashi K, Kobayashi H, Akuta K. "Vascular dilatation in the pelvis: identification with CT and MR imaging." Radiographics. 2004 Jan-Feb;24(1):193-208. Review.
