Artículo de Revisión
Intersocietary Argentine Pelvic Congestion Syndrome Consensus. Part 1
Miguel Amore, Hernán Bertoni, Pamela Causa Andrieu, Luis Catalina, Carolina Chacon, Carlos D´Alotto, Marcelo Dándolo, Guillermo Eisele, Santiago Gil, Néstor Giráldez, Sebastián Gogorza, Oscar Gural, Alberto Kenny, Esteban Mendaro, Noelia Napoli, Juan Nigro, Juan Paolini, Eugenio Piraino
Revista Argentina de Cardioangiología Intervencionista 2020;(4): 0162-0193 | Doi: 10.30567/RACI/20204/0162-0193
Los autores declaran no poseer conflictos de intereses.
Fuente de información Colegio Argentino de Cardioangiólogos Intervencionistas. Para solicitudes de reimpresión a Revista Argentina de Cardioangiología intervencionista hacer click aquí.
Recibido | Aceptado | Publicado 2020-12-31
Esta obra está bajo una Licencia Creative Commons Atribución-NoComercial-SinDerivar 4.0 Internacional.
• Miguel Amore. firstname.lastname@example.org.
2do Jefe de Servicio de Flebología y Linfología, Dto. de Cirugía Cardiovascular. Hospital Militar Central.
Staff del Servicio de Flebología y Linfología. Fundación Favaloro.
• Hernán Bertoni. email@example.com.
Radiólogo Intervencionista. Jefe de Servicio Oncointervencionismo. Instituto Roffo.
Médico de Staff del Servicio de Cardioangiología Intervencionista. Instituto Fleni.
• Pamela Causa Andrieu. firstname.lastname@example.org.
Médica Asociada. Servicio de Diagnóstico por Imágenes. Hospital Italiano de Buenos Aires, Argentina.
Clinical Fellow. Department of Radiology. Memorial Sloan Kettering Cancer Center, Nueva York, EE.UU.
• Luis Catalina. email@example.com.
Médico especialista en Diagnóstico por Imágenes. Universidad de Buenos Aires.
Staff del Servicio de Ecografía Vascular. Fundación Favaloro y Diagnóstico Maipú.
Director Médico. Centro Diagnóstico Doppler San Miguel.
Encargado de la Sección Ecodoppler Abdominal y Pélvico. Vascular Integral.
Docente de SAUMB.
Miembro del Grupo Iberoamericano de Estudio Pélvico.
• Carolina Chacon. firstname.lastname@example.org.
Médica de planta. Jefa de Sección de Ecografía. Coordinadora del Área de Imágenes en Ginecología. Servicio de Diagnóstico por Imágenes. Hospital Italiano de Buenos Aires, Argentina.
• Carlos D´Alotto. email@example.com.
Médico Especialista en Diagnóstico por Imágenes. Coordinador Área de Doppler vascular. Diagnóstico Maipú. Buenos Aires, Argentina.
• Marcelo Dándolo. firstname.lastname@example.org
Cirujano Vascular. Expresidente de la Asociación Argentina de Angiología y Cirugía Cardiovascular (AAAyCCV).
Expresidente del Colegio Argentino de Cirugía Venosa y Linfática (CACVyL).
Subjefe del Servicio de Flebología y Linfología. Fundación Favaloro.
Staff de la Unidad de Cirugía Vascular. Hospital Perón y Sanatorio Itoiz.
Miembro Titular del Colegio Argentino de Cirujanos Cardiovasculares (CACCV).
Miembro Titular de la Asociación Argentina de Cirugía.
Miembro Titular del Grupo Iberoamericano de Estudio Pélvico.
• Guillermo Eisele. email@example.com.
Médico Especialista en Diagnóstico por Imágenes UBA y homologación España.
Jefe de Radiología Intervencionista. Hospital de Niños Dr. Ricardo Gutiérrez.
Miembro fundador Colegio Argentino de Radiología Vascular e Intervencionista (CARVI).
Docente del Curso Superior de Diagnóstico por Imágenes. Hospital de Clínicas, UBA.
• Santiago Gil. firstname.lastname@example.org.
Médico ginecólogo especialista en fertilidad y jefe de Sección de Patología Pelviana Benigna. Hospital Italiano de Buenos Aires.
• Néstor Giráldez. email@example.com.
Cirujano Vascular. Miembro titular del Colegio Argentino de Cirujanos Cardiovasculares de la Sociedad de Flebología y Linfología Bonaerense y de la Asociación Argentina de Angiología y Cirugía Vascular.
Docente de la Carrera Universitaria de Flebología y Linfología. Universidad de Morón.
Unidad de Cirugía Vascular. Sanatorio Municipal Dr. Julio Méndez.
• Sebastián Gogorza. firstname.lastname@example.org.
Doctor en Medicina. Jefe honorario de Ginecología. Hospital Italiano.
Profesor Titular de Ginecología. Instituto Universitario Hospital Italiano de Buenos Aires.
• Oscar Gural. email@example.com.
Especialista en Cirugía Cardiovascular. Intervencionismo Venoso. Jefe de Servicio de Flebolinfología. Flebología Intervencionista. Fundación Favaloro.
Vicepresidente del Grupo Iberoamericano de Estudio Pélvico.
• Alberto Kenny. firstname.lastname@example.org.
Médico Especialista en Diagnóstico por Imágenes y Radiología Intervencionista. Hospital Italiano, UBA.
Becario Hospital Georges Pompidou, París, Francia.
Staff de Radiología Intervencionista de Sanatorios de Galeno Argentina y Swiss Medical Group, Buenos Aires, Argentina.
• Esteban Mendaro. email@example.com.
Radiólogo Intervencionista. Director Médico de Investigaciones Vasculares.
Jefe del Servicio de Hemodinamia. Sanatorio de la Providencia.
Médico asociado de Diagnóstico por Imágenes. Hospital Italiano de Buenos Aires.
• Noelia Napoli. firstname.lastname@example.org.
Médica Asociada. Servicio de Diagnóstico por Imágenes. Hospital Italiano de Buenos Aires, Argentina.
• Juan Nigro. email@example.com.
Cirujano Vascular Periférico. Director del Curso Superior Universitario de Flebología y Linfología, Universidad de Morón.
Director del Curso Superior de Ecodoppler Vascular Periférico e Intervencionismos Ecodirigidos (CACCV).
Vicepresidente de la Asociación Argentina de Angiología y Cirugía Cardiovascular (AAAyCCV).
Jefe de Unidad de Flebología y Linfología, Hospital Eva Perón, provincia de Buenos Aires.
Secretario general del CACCV.
• Juan Paolini. firstname.lastname@example.org.
Cirujano Vascular Sanatorio Dr. Julio Méndez, Policlínico del Docente.
Presidente Colegio Argentino de Cirujanos Cardiovasculares (CACCV).
Presidente Argentine Chapter of Society of Vascular Surgery (SVS).
Secretario General Asociación Latinoamericana de Cirugía Vascular (ALCVA).
Secretario General Sociedad de Flebología y Linfología Bonaerense (SFLB).
Subdirector Curso Universitario de Flebología y Linfología, Universidad de Morón (UM)
• Eugenio Piraino. email@example.com.
Médico ginecólogo especialista en Laparoscopia e Histeroscopia, Jefe de Servicio de Ginecología del Sanatorio San José.
• Damián Simonelli. firstname.lastname@example.org.
Médico Cirujano General e Intervencionista, Servicio de Hemodinamia y Cirugía de CEMIC.
Staff de Radiología Intervencionista. sanatorios de Galeno Argentina y Swiss Medical Group, Sanatorio Mater Dei, Sagrado Corazón, Otamendi, Clínica San Camilo, Buenos Aires, Argentina.
• Thiago Vasconcellos. email@example.com.
Médico, especialista en Diagnóstico por Imágenes. Centro Rossi, Buenos Aires, Argentina.
• Eduardo Eyheremendy
• Alejandro Kornberg
• Javier Leal Monedero
• Sergio Sierre
• Ernesto Torresani
• Santiago Zubicoa Ezpeleta
• Arturo Fernández Murga
• Juan Manuel Ponce
• Guillermo Eisele
PARTICIPANT MEDICAL SOCIETIES
• AAAyCCV: Argentine Association of Angiology and Vascular Surgery
• CACI: Argentine College of Interventional Cardioangiologist.
• CACCV: Argentine College of Cardiovacular Surgeons.
• CACVyL: Argentinian College of Venous and Lymphatic Surgery.
• CARVI: Argentine College of Vascular and Interventional Radiology.
• SAR: Argentine Society of Radiology.
• SFLB: BonaerenseSociety of Phebology and Lymphology.
• Iberoamerican Working Group of Pelvic Studies.
TABLE OF CONTENTS
1. Methodology used in the Consensus
3. Summary of Consensus
3.3 Clinical signs
3.4 Differential diagnosis
3.5 Imaging diagnosis
3.6 Medical treatment
3.7 Endovascular treatment
3.8 Surgical treatment
3.9 Additional extrapelvic management
4. Intersocietal Argentine Pelvic Congestion
4.1 History and epidemiology
4.2 Pathophysiology and clinical signs
4.2.1 Pathophysiological mechanisms
4.2.2 Clinical signs
220.127.116.11 Differential Diagnosis
18.104.22.168 Clinical evaluation of the results of the
22.214.171.124 AVLS Classification
4.3 Diagnostic imaging modalities
4.3.1 Color-coded Doppler echocardiography
126.96.36.199 Study protocol
188.8.131.52 Access routes and patient preparation
184.108.40.206 Diagnostic criteria for vascular compression
• The nutcracker syndrome
• The May-Thurner syndrome
220.127.116.11 Diagnostic criteria for pelvic varicocele
• Gonadal veins
• Hypogastric or internal iliac veins
18.104.22.168 Pelvic leak points
22.214.171.124 Color-coded Doppler echocardiography
during postoperative management
4.3.2 Computed axial tomography and magnetic resonance imaging
126.96.36.199 How to request the study
4.3.3 Gonadal and iliac dynamic phlebography
188.8.131.52 Gonadal and iliac dynamic phlebography
4.4 Importance of medical treatment
4.4.2 The pelvic congestion syndrome as the cause of
chronic pelvic pain
4.4.3 Medical therapy
184.108.40.206 Non-steroidal anti-inflammatory drugs
220.127.116.11 Drugs to suppress ovarian function
18.104.22.168 Venoactive drugs
22.214.171.124 Compression treatment for pelvis and LL
4.5 Endovascular treatment
4.5.2 Transcatheter embolization
126.96.36.199 Technique and materials
188.8.131.52 Postembolization care, complications,
184.108.40.206 Recommendations for transcatheter
embolization of gonadal veins
and hypogastric vein branches
4.5.3 Angioplasty in venous obstruction syndromes
220.127.116.11 Obstruction of inferior vena cava
and May-Thurner syndrome
• Pathophysiology and clinical presentation
18.104.22.168 The nutcracker syndrome
• Phenomenon, syndrome and differential
4.6 Surgical Treatment
4.6.2 Conservative surgical treatment
22.214.171.124 Selective gonadal vein ligation through open
or conventional approach
126.96.36.199 Laparoscopic selective gonadal vein ligation
• Complications of laparoscopic gonadal
• Limitations of laparoscopic gonadal ligation
4.6.3 Non-conservative surgical treatment
188.8.131.52 Hysterectomy and salpingo-oophorectomy
184.108.40.206 Complications of open surgery
4.6.4 Vascular and endovascular surgical treatment of compression syndromes as the cause of the pelvic congestion syndrome
220.127.116.11 The May-Thurner syndrome
18.104.22.168 The nutcrackersyndrome
• Pathophysiology and clinical signs
• Treatment options of the nutcracker syndrome
o Conservative treatment
o Interventional behavior
4.6.5 Analysis and considerations
4.7 Additional extrapelvic treatment
4.7.2 Comprehensive diagnostic evaluation of pelvic venous incompetence
22.214.171.124 Clinical-semiological examination
126.96.36.199 Semiological examination under
188.8.131.52 Color-coded Doppler echocardiography
184.108.40.206 Computed axial tomography and
magnetic resonance imaging
220.127.116.11 Gonadal and iliac dynamic phlebography
of pelvic floor leak
4.7.3 Therapeutic arrangement of lower limb venous incompetence originated from pelvic varicose veins reflux
18.104.22.168 Common pelvic floor leak points
22.214.171.124 Treatment techniques of pelvic floor leak
• Percutaneous extrapelvic embolization
• Section and selective ligation of leak points
associated with intra- and extrapelvic sclerosis
4.7.4 Postoperative management
4.7.5 Treatment of intrafascial and epifascial vessels
126.96.36.199 Conventional surgery
188.8.131.52 Minimally invasive procedures
4.7.6 Analysis and considerations
1. Methodology used in the
Chronic pelvic pain (CPP), which mainly affects women in reproductive age, shows a significant incidence rate in the general population, where pelvic congestion syndrome (PCS) is the second leading cause of CPP.
Several medical societies have drafted documents to unify concepts seeking international consensus for the diagnosis and management of PCS.
Based on the existing evidence and experience managing PCS, a group of Spanish-speaking experts on Vascular Surgery, Phlebology, Gynecology and Obstetrics, Diagnostic Imaging Modalities, Radiology, and Interventional Cardiology has drafted the first edition of the Argentine Pelvic Congestion Consensus.
Using the Levels of Evidence and Recommendation established by the American College of Cardiology (ACC) and the American Heart Association (AHA) and accepted by the European Society of Cardiology (ESC), the validity of the diagnostic and treatment methodologies is established with proven utility in the lasting resolution of PCS cases (Table 1).
According to this classification, the strength of the evidence and the degree of recommendation associated with a specific diagnosis or treatment option are based on the data available (https://www.wikidoc.org/index.php/ACC_AHA_guidelines_classification_scheme).
The process of elaborating this consensus document was carried out following the algorithm of the Consensus area of the Argentine College of Interventional Cardioangiologist (CACI). Once the topic of discussion is determined, the consensus coordinators are summoned; they are responsible for the supervision, distribution of the project, and coordination of the discussion during during the stages of analysis of the document at stake. The consensus office together with the coordinators invite experts that will participate as authors, who will be carrying out a thorough, systematic search of the literature available, conduct the critical analysis, and write the draft or preliminary document. Once the preliminary document is completed, it is posted on the CACI website long enough for it to be analyzed by all the authors and coordinators involved. To this end, a tool called FORO has been created by CACI that grants access limited only to participant members. It allows the document to be viewed and triggers participation in the discussion via comments that will appear chronologically and will be supervised by the coordinators. This tool increases author participation and saves time. Once this stage is completed, the coordinators collect all the opinions and suggestions to draft the final document with the same writing style. Afterwards, the final document should be submitted for external review and to this end, it is once again posted on the FORO (replacing the previous version) and access is granted to those who will act as judges for final approval purposes; these people will make up the Review Committee. We should mention that at this stage it is also possible to make corrections as long as an agreement is reached between the authors and the review committee. Its members (coordinators, authors, and reviewers) are responsible for ensuring the rigor and quality of the document generated. Once the document has been approved, the consensus is presented in scientific sessions at different CACI societies and is published in scientific journals.
- AMC: aortomesenteric clamp
- BMI: body mass index
- CAT scan: computerized axial tomography scan
- CDE color-coded Doppler echocardiography
- CHIVA: outpatient conservative haemodynamic correction of vein incompetence
- CPP: chronic pelvic pain
- DVT: deep venous thrombosis
- GIDP: gonadal and iliac dynamic phlebography
- IVC: inferior vena cava
- IVUS: intravascular ultrasound
- LL: lower limbs
- LRV: left renal vein
- MPA: medroxyprogesterone acetate
- MRI: magnetic resonance imaging
- MTS: May-Thurner syndrome
- NCS: Nutcracker syndrome
- PCS: pelvic congestion syndrome
- PFL pelvic floor leak
- PTA: percutaneous transluminal coronary angioplasty
- PTE: pulmonary thromboembolism
- PV: peak velocity
- PVI: pelvic vein incompetence
- QOL: quality of life
- SMA: superior mesenteric artery
- SPJ: saphenofemoral junction
- TE: transcatheter embolization
- TV: transvaginal
- TVCDE: Transvaginal color-coded Doppler
- VAS: visual analogue scale
3. SUMMARY of CONSENSUS
The first description of ovarian varicose veins was made by Richet back in 1857. In 1949, Taylor, who associated anatomical changes with the symptoms of ovarian vein incompetence, proposed the concept of PCS suggesting ovarian vein ligation as the treatment of choice.
At the beginning, the etiology of pelvic venous incompetence (PVI) as the main pathophysiology of PCS was unknown and led to the psychological-psychiatric theory as an explanation of the symptoms of patients with pelvic pain disorders without an apparent cause.
Back in the 1980s, Lechter defined female gonadal vein incompetence as the causative of lower limb (LL), pelvic, and vulvar varicose veins. At that time, Beard proposed hysterectomy and bilateral oophorectomy as the treatment of PCS. In 1993, Edwards treated utero-ovarian varicose veins through transcatheter embolization (TE) for the first time ever, and in 2003, Chung proved the significant benefits of this therapy when compared to surgery and hormone therapy.
PCS is, after endometriosis, the second leading cause of CPP in 30% to 40% of the cases. PVI is the etiological element that characterizes PCS. Costs derived from the management of CPP are high (over 880 million dollars each year in medical appointments and 2 billion dollars each year in overall costs in the United States). Although numerous medical journals find a correlation among PVI, PCS, and LL vein incompetence, no articles have been published of good methodological quality confirming it.
PCS is defined by the presence of 2 or more of the following conditions: 1) utero-ovarian varicose veins with or without gonadal vein incompetence, 2) congestive pelvic pain, 3) hypogastric vein incompetence, 4) pelvic floor reflux, typical or atypical LL varicose veins of pelvic origin. Although the term PCS cannot cover all forms of presentation and causes, it has been agreed to be used because it appears in the International Classification of Diseases and the VEIN TERM Transatlantic Interdisciplinary Consensus Document.
The origin of PCS is multifactorial with valvular insufficiency, venous obstruction, and hormonal changes as the most common causes. The cause of pain in PCS is distension due to venous stasis, which stimulates the release of local inflammatory and pain mediators.
Pathophysiologically speaking, the causes of PCS can be categorized into:
Primary, due to ovarian venous valve agenesis or incompetence; these are the ones most commonly associated, or not, with LL vein incompetence and the ones that best respond to TE procedures.
Secondary, due to the occlusion of iliac veins (May Thurner syndrome [MTS]), inferior vena cava (IVC) or left renal vein (LVR) known as the nutcracker syndrome (NCS). The typical symptoms of PCS are associated with the venous obstruction area, and the ideal endovascular resolution combines percutaneous transluminal coronary angioplasty (PTA) and TE.
CPP-related nonvenous PCS are mainly due to non-vascular pelvic causes, basically correspond to endometriosis and other pelvic causes. PVI, though present, is not the main cause of the clinical signs. In these cases, therapies target the causative lesions detected.
3.3 Clinical signs
PCS has 4 clinical presentations:
1. Typical pelvic clinical signs compromising the uterus, ovaries, bladder and/or rectum.
2. Typical LL, vulvar, and gluteal (saphenous) or atypical (non-saphenous) varicose veins.
3. Edema and LL pain due to venous obstruction.
4. Renal clinical signs with pain and hematuria.
Pelvic damage often occurs with pelvic pain and LL varicose veins.
The dull, deep, prolonged, non-cyclical pain is exacerbated by movements and postures that increase abdominal pressure. It is a unilateral or bilateral, asymmetric and, chronic pain that lasts over 6 months. Associated with dyspareunia, dysmenorrhea and post-coital pain, it can cause nausea, distension or abdominal cramps, rectal discomfort, dysuria, and greater urinary frequency. It is often associated with neurological and psychosomatic symptoms like reactive depression.
The clinical signs of PVI are tenderness to palpation at ovarian, uterine, and cervical sites, as well as utero-ovarian, vulvar, gluteal, atypical of thigh and leg varicose veins associated with pelvic floor leak (PFL). It is often associated with symptomatic hemorrhoids. Varicose veins are predominant in the left LL for 2 reasons: gonadal and utero-ovarian PVIs are more significant on the left side of the body as well as PFL sites; also, in MTS cases, the left side is the common side where this condition develops. Clinical detection of the pelvic origin of LL varicose veins is not always evident and it requires performing a color-coded Doppler echocardiography (CDE); it should be suspected in the presence of post-saphenectomy relapses.
The predisposing factors of PCS are multiple pregnancies, sedentary lifestyle, increased estrogenic activity and low body mass index.
The clinical signs associated with venous obstructions (MTS, NCS, IVC occlusion, etc.) only occur when they are the causes of PCS.
3.4 Differential diagnosis
Both questioning and physical examination should cover the different possible causes of PCS. Suspected conditions will jutify performingearly clinical evaluationsthat will eventually lead toorderingadditional laboratory tests, invasive imaging modalities or not selectedbased on their effectiveness, invasiveness, and availability.
In this diagnostic stage, 2 etiological groups should be defined:
1. Patients with typical (primary or obstructive) PCS who will benefit from TE and/or PTA therapies. Medical (and rarely surgical therapies) cancomplement these therapies. This consensus document focuseson the management of these patients.
2. Non-venous PCS with clinical signs and CPP and PCS combined additional studies should be especially considered by the heart team. It is mandatory to confirm differential diagnosis where the main cause of CPP does not correspond to PCS. Also, specific treatment should be offered for each condition, especially if gynecological or extra-gynecological
3.5 Diagnostic imaging modalities
Color-coded Doppler echocardiography
It is the first-line diagnostic assessment tool thanks to its non-invasive, non-radiation capabilities. It performs real-time studies of vein hemodynamics and allows performing maneuvers and position changes that improve its sensitivity and specificity (> 90% in PCS).
The assessment protocol should include: 1) evaluation of compression syndromes such as NCS and MTS covering IVC, left renal vein (LRV), and primitive iliac veins; 2) gonadal veins; 3) internal iliac veins, and 4) PFL points at the pelvic PVI and LL junction.
Access routes are transabdominal, transvaginal (TV) and PFL. The latter is used to study inguinal, gluteal, obturator and perineal points.
There are proven diagnostic criteria in the CDE of the different PCS components that should be confirmed for diagnostic precision purposes. The CDE of Vein Compression Syndromes facilitate the detection of Direct Signs of stenosis that have a high diagnostic value and are associated with a reduction of vein diameters and speed changes; and Indirect Signs that originate inthe circulatory changes occurred on both sides of the stenosis and the development of collateral circulation.
The diagnostic criteria of Pelvic Varicocele include findings of gonadal veins (proximal with diameters >6 mm, Valsalva reflux, and distal with increased number and caliber with tortuous, ecstatic morphology, and ecstatic flow) and hypogastric veins.
Also, the CDE is key in the postoperative management of PTA (to treat compression syndromes) and TE (to treat insufficient pelvic varicose veins, PFL points, and LL).
Computed axial tomography scan and magnetic resonance imaging
Both imaging modalities should cover from the renal veins to the pelvis and the roots of the thighs to characterize incompetent pelvic veins, different drainage routes, and possible vein compression causes.
The MRV has a 88% sensitivity and a 67% specificity for the identification of gonadal vein PVI compared to conventional phlebography in the diagnosis of PCS. Also, it can characterize other causes of CPP (endometriosis, adenomyosis, myomas). It requires dynamic evaluation with IV contrast with images in the arterial phase (to view venous reflux and arterial vascular causes) and in the venous phase (venography, for the measurement of vascular diameters, for the assessment of compromised veins, to identify venous obstruction).
Both the computed axial tomography scan (CAT scan) and the MRI are used for monitorization purposes after the endovascular treatment. Also, it is necessary to first assess the residual clinical signs and identify the possible redistribution of venous drainage to another (hypogastric) territory. There can be artifacts in the MRI due to TE material and venous PTA that should be taken into account.
Gonadal and iliac dynamic phlebography
It is the diagnostic study with the highest sensitivity and specificity (80% to 100%) and it is considered the gold standard in the diagnosis of PCS. It provides a high-precision venous dynamic assessment with confirmation of incompetent veins, varicose veins, and their collateral leaks and even obstructive lesions. It is the imaging modality used to guide endovascular treatments.
Performed via brachial, internal jugular or femoral venous catheterization, it requires minor sedation (or not) and short hospital stays. It should include the assessment of IVC and LRV, gonadal, primitive iliac, hypogastric veins and their pelvic floor branches. It allows us to measure venous pressures from renal and primitive left iliac stenoses. The details of these findings are essential to confirm the diagnosis of PCS.
Gonadal and iliac dynamic phlebography (GIDP) is indicated in 3 situations mainly: 1) in the presence of inconclusive findings of non-invasive studies; 2) when it is necessary to confirm the findings of non-invasive studies; and 3) at the proposal and planning of endovascular treatment for the PCS.
3.6 Medical therapy
To start medical therapy for the management of PCS, first it is necessary to rule out other causes of CPP. Once the PCS has been confirmed as the causative agent and since it is often multifactorial, medical therapy will be targeted at controlling the symptoms derived from PVI and dilatation (pain killers, venotonic drugs, and elastic compression bandages) and the hormone condition involved in the development of the syndrome (hormone medication).
Non-steroidal anti-inflammatory drugs
Used as a first-line therapy, they provide temporary relief, are limited by adverse events, and are often used temporarily until specific therapies are implemented.
Vasoconstrictor that, when administered IV, improves the clinical signs of PCS pain in 30% of the patients. Limited use due to its short effect and multiple contraindications.
Progestogens. Used to treat CPP due to endometriosis; to treat the PCS, medroxyprogesterone acetate (MPA) of antiestrogenic and gonadotropic effect is used. They partially improve the symptoms of PCS.
GnRh agonists. Using synthetic analogs, menopause-like hypoestrogenism is achieved. Although they can improve the clinical signs of PCS, they use is limited due to the associated rate of climacteric symptoms and osteoporosis.
Diosmin, hesperidin. They improve vein and lymphatic circulation by reducing venous stasis and symptoms in PCS and LL, with optimal tolerance.
Compression treatment of pelvis and LL
The use of compression pants and stockings has shown clinical improvement in over 80% of the cases of patients with PCS and LL varicose veins.
3.7 Endovascular treatment
The main purpose of this therapy is to eliminate PVI components (varicose veins, pelvic refluxes, and collateral circulation), which happen to be the major contributors of the PCS clinical signs. Endovascular treatments of venous catheterization that are performed at the cath lab are outpatient procedures or have a very short hospital stays and are technically successful in over 95% of the cases with clinical improvement in 68% to 100% of the cases and evidence level 1B. The endovascular treatment techniques used are the TE of choice for the management of gonadal or hypogastric vein incompetency, while the PTA and venous recanalization often solve venous obstructive phenomena.
Based on the information provided by imaging modalities, but mainly the GIDP, the TE should occlude previously found incompetent gonadal, utero-ovarian varicose veins and hypogastric branches with the use of sclerosing liquids and metal devices (coils, plugs). TE is usually performed outpatiently with 24-hour hospital stays.
TE related complications often occur in less than 3% of the cases and are associated with postembolization syndrome; coil migration can be treated via an endovascular approach.
Postoperative management is clinical and based on scales like the visual analog scale (VAS) and the quality of life (QOL) scale. CAT scans or MRI, but especially the DCE, are important to assess the post-TE results at PFL points and LL varicose veins level.
Vein compression syndrome angioplasty
The vein obstruction-compression causes of PCS are often underestimated; however, the MTS has been reported in up to 80% of all PVI cases with PCS.
When PCS is associated with IVC obstruction or MTS, the PTA will initially be performed with astent, and in the presence of persistent PCS clinical signs, a TE will be performed at a second stage on the pelvic varicose veins. However, when PCS is associated with the NCS, a TE on the veins with reflux will be performed first, and the PTA of the LRV will be spared for cases of hematuria, severe lumbar pain or varicose veins persistent after the TE. The ilio-caval PTA shows primary, assisted primary and secondary patency of 79%, 100%, and 100%, respectively, in non-thrombotic disease, and 57%, 80%, and 86% respectively, in DVT at the 7-year follow-up.
3.8 Surgical therapy
It includes the ligation of ovarian veins by laparotomy or laparoscopy where the main benefit is diagnostic confirmation, especially for the management of gynecological conditions. The PCS of around 70% of patients is controlled using both techniques, but these are short-term experiences and with frequent relapses. These therapies have a 2B level of evidence recommendation and they are indicated only in cases of unavailability or therapeutic failure of less invasive techniques because they are associated with common major complications and high costs.
The resection of uterus and/or annexes (uni- or bilateral) contributes a significant benefit in the control of PCS, in addition to the patients’ psychological and social recovery. However, the high dispersion of results, common relapses due to lack of VPI control, but above all, the higher morbidity and mortality rates and recovery time reported have turned these therapies into last-line alternatives, only after other simpler, less bloody, and effective treatments have failed.
Vascular and endovascular surgical treatment of compression syndromes as cause of PCS
Basically, it includes the management of the MTS and the NCS.
The May-Thurner Syndrome. Before 2000, 75% of the procedures were performed by open surgery (25% via endovascular approach). Bypass surgical techniques were used that would create arteriovenous fistulas with unsatisfactory results due to high rates of DVT and restenosis. After 2000, this ratio reversed and 4.1% of the patients are operated on through open surgery and 95.9% via endovascular approach with balloon PTA and stenting and, sometimes, coadjuvant thrombolytic drugs (33.2%) or not (53.2%). As medical therapy (7%), anticoagulation and elastic compression are used.
The Nutcracker Syndrome. Conservative treatment is based on a 24-month wait-and-see approach in patients under 18 and a 6-month wait-and-see approach in adult patients, in whom weight gain and the development of collateral circulation improves clinical signs in 70% of the cases.
Intervention is decided once this period of time has elapsed or if there is clinical worsening with hematuria and severe recurring pain, an impaired kidney function, and significant varicocele.
Surgical therapy includes complex and different techniques such as renal vein transposition, renal autotransplantation, gonado-caval or splenorenal bypass and LRV ligation with bypass to iliac vein. Although a suitable resolution of the symptoms is initially achieved initially in over 80% of the patients, recurrence can occur at the end of follow-up due to venous restenosis. The complications reported require hospitalization and long controls, which is why this therapeutic decision is made by expert surgical teams in the management of these cases.
Endovascular treatmentis performed using PTA with LRV stenting. A PCS comprehensive treatment is recommended, first performing TE on gonadal and hypogastric varicose veins, and then renal PTA. With clinical improvement >95%, it requires a careful PTA technique and stent selection.
3.9 Additional extrapelvic management of PCS
The rates close to 80% reported in the association among pelvic, vulvar, perineal, and LL varicose veins are in correlation with the PCS, which represents nearly 35% of the cases of vulvovaginal varicose veins, and 90% of the cases of LL varicose veins. After the endovascular treatment of PCS with PFL related-LL vein incompetence, at times, relapses or varicose vulvoperineal remnants can occur and are responsible for the persistence of postoperative LL vein incompetence.
The study protocol of LL vein incompetence due to PCS is similar to the one used in LL varicose vein relapse after TE. It includes detailed clinical assessment of persistent LL varicose veins associated with the CDE study (enhanced with the use of augmented reality) of the PFL points and LL varicose veins involved. TV-CDE, CAT scans, MRI, GIDP, and varicography are used based on the need for more detailed anatomical and/or functional information on refluxes or their possible causes.
Special attention should be paid to the coexistence of superficial and/or deep LL vein incompetence, either essential or post-thrombotic associated with PCS, which should be properly detected and treated.
The management of these relapses if defects or omissions of already-embolized hypogastric or gonadal refluxes are reported, starts by correcting these errors with a second TE that should be as complete as possible, especially on the tributaries that irrigate the relapsed venous territories.
Several ultrasound-guided sclerosis techniques (with or without augmented reality) used on perineal varicose veins and PFL points are described with promising early results. Hower, studies long enough follow-up periods are still needed on this regard.
Finally, the procedure will be performed on the LL vein incompetence (saphenous trunks or accessories, leak perforator vessels) using conventional techniques (conventional major or minor saphenectomy, staged removal of varicose segments, perforator vessel surgery).
4. INTERSOCIETAL ARGENTINE PELVIC CONGESTION SÍNDROME CONSENSUS
4.1 History and epidemiology
Authors: Juan Esteban Paolini, Néstor Omar Giráldez.
Back in 1857, Albert Richet (1) came up with the very first anatomical description of ovarian varicose veins. Almost a century later, in 1949, Taylor (2) associated the anatomical variables with the physiological disturbances generated by ovarian vein incompetence. Also, he was the first to propose the concept of pelvic congestion syndrome (PCS) and suggested the ligation of ovarian veins as a therapy. He also considered that the origin of that vein incompetence was somehow connected with changes of the autonomous gonadal nervous system, suggesting a possible psychosomatic association with the symptoms. Since the true pathophysiology of gonadal vein incompetence was not known, the disease was treated as a psychological/psychiatric disorder in patients who presented with pelvic pain without an apparent cause (3).
In 1954, Dixon and Michell performed the first radiological studies of circulatory anomalies with phlebographic findings. In 1964, Clark described the right ovarian vein syndrome (4)
In the 1980s, Colombian gynecologist Abraham Lechter coined the term female gonadal system incompetence or pelvic vein incompetence (PVI) as the causative agent of lower limb (LL), pelvic, and vulvar varicosity. Afterwards, he conducted studies in cadavers and was able to determine the anatomical variants and proposed the open bilateral ligation of ovarian veins (5,6). British gynecologist Beard redefined PCS (7-9) by proposing hysterectomy with bilateral oophorectomy as the best way to treat this condition (10).
Taking into account the complexity of this procedure and the hormonal changes it causes, it was considered mutilating surgery, one that is hard to accept by young women with reproductive potential, a presentation characteristic of this disease (3).
In 1993, Edwards (11) proposed for the first time, the endovascular treatment option with embolization of ovarian veins, thus changing the paradigm of the female pelvic varicocele therapy. Leal Monedero et al. (12,13) determined the pathophysiological basis of sub-diaphragmatic refluxes and embolizing treatment. This highly effective, low morbidity and minimally invasive therapy replaced almost all the remaining conventional open surgical procedures considered mutilating treatments to this date (3).
In some Latin-American countries the laparoscopic treatment of pelvic varicocele is still an elective procedure (14-16) but thanks to the development of diagnostic means and technological advances,compared to the remaining therapies, endovascular treatment has been issued a Class II b Recommendation by the American Venous Forum and the Society of Vascular Surgery (17,18).
Chungy et al. (19) conducted a comparative study about PCS therapies available today and confirmed the statistically significant benefits of endovascular treatment.
According to Candance (20), PCS is associated with chronic pelvic pain (CPP) in 30% to 40% of the cases where PVI is the characteristic etiological component of PCS.
CPP is a complex nosological entity consisting of pelvic pain of over 6 months of clinical evolution often affecting one or several systems (gynecological, urinary, osteo-arthro-muscular, digestive, nervous) in different order and intensity, and followed by often emotional repercussions. Mainly, it affects women (but not exclusively), and it is highly prevalent among the general adult population. Still, according to the reports available its incidence rate is variable: 15% in the USA and 43% in other countries (21,22).
Zondervan et al. (23) reviewed a UK primary healthcare database that included the medical histories of 162 women from 12 to 70 years old. They found an incidence rate of 38/1000 per patients-year going to doctors’s offices due to CPP.
CPP amounts to 20% of all outpatient gynecological appointments. An annual US$ 881,5 million are spent in outpatient care and the overallannual cost in the United States is US$ 2 billion and £158 million in the UK (21,24). Several epidemiological studies, both prospective and retrospective, despite their different scientific quality, have proved that PVI is responsible for between 11.2% and 28% of the cases of CPP (25-27). Similarly, other PVI assessment studies in women with recurrent LL varicose veins have found prevalence rates of 20% to 76% (28).
4.2 Pathophysiology and clinical signs
Authors: Guillermo Eisele, Marcelo Dándolo.
Note: Angiographic imaging in this chapter are the sole property of the authors; the diagrams have been reproduced with the authors’ permission.
4.2.1 Pathophysiological mechanisms
The characteristics of the pelvic congestion syndrome (PCS) were initially described and published by Richet back in 1857 and then associated with chronic pelvic pain (CPP) in 1947 by Taylor (1,2) In recent years, it has been observed that there is not a unanimous consensus on the denomination of PCS to designate this entity; however, it is included in the International Classification of Diseases and VEIN-TERM of the Transatlantic Interdisciplinary Consensus (3). Although it is considered that there can be a multiple origin of PCS (hormonal, anatomical, post-partum, etc.), gonadal, hypogastric pelvic vein incompetence (PVI) and its spread towards the lower limbs (LL) is without a doubt the determinantpathophysiological factor. The coexistence of other participant mechanisms in PCS make the causal relation of PVI, though proven, not categorically established as the main causative agent of PCS (4-8). In this well-known underestimation of PCS (9,10), 2 elements of conceptual confusion are involved, both highly frequent among the female population of reproductive age. On one hand, utero-ovarian varicose veins can be found in up to 10% of the women. In 40% of these women, they are not associated with PCS, and it is an anatomical finding without any associated clinical signs. This means that no specific therapy or follow-up is required. On the other hand, 30% of the women’s CPP is due to PVI only, while another 15% of the patients with PCS have other additional non-venous related pelvic etiology. This makes the isolate identification of main and secondary problem ambiguous when it comes to administering evidence-based therapies (11).
To properly distinguish when the PCS is mainly associated with PVI or in what patients PCS is only a minor component is often ideally approached by multidisciplinary working groups, both at the diagnostic stage and when having to decide the most suitable approach or therapy (12).
It is commonly accepted that the origin of PCS is multifactorial, and vein incompetence, venous obstruction, and hormonal changes are the most usual suspects.
The cause of pain in PCS is associated with the progressive venous distension that together with vascular stasis stimulate the release of local inflammatory and pain mediators (13). In fact, the histopathological examination of these pelvic varicose veins shows fibrosis of tunica intima and tunica media with muscular hypertrophy and capillary endothelium proliferation (14). It is likely that the pain mechanism of PCS in its chronification process also acquires characteristics of growing complexity as it happens with CPP (15).
PVI, the leading cause of PCS, is anatomically characterized by the abnormal and insufficient dilatation of veins in the ovarian, uterine, hypogastric territories and very commonly in the LL too. The interconnection of ovarian and hypogastric venous systems mainly occurs in the broad ligament of the uterus and its fixation elements. From there, PVI can transmit venous reflux towards the hemorrhoidal, sacro and vesical circuits. More frequently, PVI of internal pudenda veins, round-ligament venous plexuses and parametrium lead to PCS per se; the predominant compromise of obturator and femoral circumflex veins often occurs as vulvar varicose veins and LL vein incompetence; and ovarian vein incompetenceoften occurs with both clinical signs (16). Back in 1997, Leal Monedero introduced the accurate concept of sub-diaphragmatic vein incompetence (a denomination that unified PCS and LL chronic vein incompetence), which is in turn pathophysiologically divided into “centrifugal reflux” (the predominant anti-physiological pelvic venous flow or in the direction of pelvic and LL varicose veins) and “centripetal reflux” (the utero-ovarian flow has a physiological direction, but with increased volume, and it is part of the collaterality of central venous obstruction, post-thrombotic syndrome (PTS) with ilio-caval compromise, May-Thurner syndrome (MTS).
The hormonal involvement of PCS can be seen in the different clinical instances. The regularity of PCS pain is associated with menstrual cycle in women whose symptoms decrease after the menopause, and with the response to treatment with anovulant drugs and human chorionic gonadotropin agonists where estrogen depletion, among other mechanisms, would be decreasing the response of the smooth musculature of the venous wall (9).
The pathophysiological mechanisms of PCS show a close correlation between its origin, frequency and response to the different modalities of treatment. They have been schematically categorized by different authors (9,17-19): Primary PCSs are due to ovarian venous valvular incompetence, venous anatomical variants, venous kinking following uterine malappositions, and structural and hormonal changes associated with pregnancy and labor. Primary PCSs occur most often with typical clinical signs of PCS associated, or not, with LL vein incompetence and they are the group that best responds to transcatheter embolization of endovascular treatment (ET).
Secondary PCSs are associated with severe stenosis or occlusion of drainage veins at the pelvic or suprapelvic level. Often chronic post-thrombotic, it compromises the iliac veins (primitive or internal) or the inferior vena cava (IVC) and they are often called, respectively, iliac compression syndrome or MTS and IVC syndrome. Some authors include congenital causes in this group (while other authors place them separately) like pelvic vascular malformations, IVC agenesis and aplasia, iliac veins and changes to the left renal vein (LRV) like compression due to aorto-mesenteric clamp (AMC) (or anterior nutcracker syndrome) or its retro-aortic location (posterior NCS) (9,20-22). Although they show clinical signs of PCS, in this group, patients often show symptoms associated with the territory of the venous obstruction that originate it. Endovascular resolution, properly associating percutaneous transluminal coronary angioplasty (PTA) with stent and TE is often the optimal therapeutics.
Finally, there is a group of non-venous pelvic causes that can come from different origins, mostly endometriosis. Other etiologies can correspond to myomas, benign uterine and ovarian tumors, adenomyosis, post-partum uterine retroversion, postoperative and post-infectious adhesions and staples, Master-Allen syndrome (lesion of uterine fixation elements), and non-obstetric trauma. In these cases, the presence of varicose veins and PVI often responds to a coincidental epiphenomenon rather than a direct causal relation. Therefore, once the differential diagnosis has been confirmed, if the cause of CPP is not associated with PCS, the specific treatment of each particular condition should be provided. In these cases, there is no indication for a TE procedure on the utero-ovarian varicose veins since they are rarely involved in the mechanism of pelvic pain.
In conclusion, the pathophysiologyof PCS is due to several venous circulatory and functional changes mainly determined by PVI. Understanding the correlation between clinical signs and changes of pelvic vein dynamics is essential regarding the endovascular and sclerosing therapies. Similarly, cases of primary and secondary PCS of strictly non-venous conditionswhere multi-disciplinary action would be required should be differentiated.
4.2.2 Clinical signs
CPP is a common cause of consultation in women between 18 and 50 years old. Actually, 39% of women have reported it at one time or another during their lifetime (12). Pain is by definition, a sensory and emotional experience associated with a real or potential damage of some tissue. CPP is an incapacitating situation that not only has physical repercussions, but also psychological and emotional. Several studies have discussed this issue and it is estimated that it causes the loss of jobs in 15% of the population and a 45% productivity reduction (8). In the United Kingdom, 18% of women report CPP and between 2% to 10% of all gynecological appointments are due to this cause (9,23).
Of the many causes of CPP, in this consensus document we have been focusing on PCS. In patients without any other apparent cause for CPP, it was confirmed that 30% had PVI and 10% of dilatation of one or both ovarian veins. Back in 2001, Soysal et al. studied women with CPP by conducting pelvic assessments, laparoscopies, ultrasounds and venographies. Thirty-one per cent of them had PCS as the only abnormality while 12% showed PVI associated with another condition like endometriosis, pelvic inflammatory disease, postoperative adhesions, and uterine disease (myoma or adenomyosis) (23-25). PCS is the second leading cause of CPP after endometriosis (26).
Although CPP is a common problem in premenopausal multiparous women, there are several etiologies that lead to these clinical signs and diagnosis can be complex. Fortunately, the evermore precise diagnostic tools available provide accurate information. However, in certain cases the differential diagnosis is complex since multiple conditions can coexist during a woman’s reproductive age.
PCS symptoms and signs are varied and some of them are unspecific of PVI. Patients with PCS report a dull, deep, non-cyclical pain that is exacerbated by movements, postures, and activities that increase abdominal pressure. Pain can be unilateral or bilateral and it is often asymmetrical and chronic of > 6-month clinical evolution (27,28). It does not have an obvious source and it can be associated with dyspareunia (71% of the cases), dysmenorrhea (66%), and post-coital pain (65%). Less often, it can cause nausea, distension or abdominal cramps, rectal discomfort, dysuria and an increased urinary frequency. Symptoms of the neurological and psychosomatic spectrum such as reactive depression are also prevalent (24,28).
Physical findings suggestive of PCS include sensitivity at ovarian, uterine and cervical points that are exacerbated during bimanual uterine palpation. The presence of utero-ovarian and pelvic varicose veins in vulva, gluteus and legs are characteristic signs associated with pelvic leak points, which will be dealing with below (9). Association with symptomatic hemorrhoids is common.
As we have already mentioned among the causes of CPP, PCS ranks second as the entity causing it. PCS can be due to different causes that determine PVI and, in turn, develop more or less signs and symptoms depending on the degree of dilatation and personal factors such as physical build, activity, number of pregnancies or hormonal status thatcan have a negative or positive impact.
The factors that favor the development of PCS include normal or slim body builds with a low body mass index (BMI). A study conducted by Labropoulos et al. categorizes patients into normal weight (BMI < 25), overweight (BMI, 25-29.9), and obese (BMI > 30). It was confirmed that most of the patients with PCS have a low BMI (24.1 kg/m2±5.6 kg/m2) (27). Regarding activity, those with a sedentary lifestyle or an increased intra-abdominal pressure are considered harmful (8). The number of pregnancies also has a negative impact and PCS is more common and severe among multiparous women. However, nulliparous women can also develop PCS (29). There is also a correlation between vein dilatation and estrogen level since symptoms decrease with the arrival of the menopause (28).
Venous circulation patterns in PVI are closely associated with the different causes of PCS (30). In primary PCSs due to venous wall insufficiency, centrifugal flow is established (which is inversely proportional to the usual flow). When the situation is perpetuated due to reflux and varicose veins, venous hypogastric and pelvic floor collaterality that do not stabilize and increase over time are established.
In secondary obstructive PCSs due to MTS, NCS or IVC occlusion, centripetal flow is established and, in this case, venous collaterality tries to compensate the venous hypertension established by the stenosis or the obstruction. If pressures are equalized, venous collaterality finally stabilizes.
Concomitant PCSs due to local causes (endometriosis, tumor mass, postoperative of gynecological surgeries) can coexist with utero-ovarian varicose veins; in these cases, we should see whether the PVI, the gynecological condition or both are determinant factors of the painful clinical signs described.
This venous collaterality, contained in the pelvis as pelvic varicose veins, will seek pelvic floor leak points (PFL). These are preexisting anatomical connections between pelvis and LL through extrapelvic branches of the hypogastric vein that end up developing hemorrhoids, vulvar varicose veins, testicular varicocele in men, and LL varicose veins. Leak points are well-known and established by Dr. Leal Monedero and Dr. Zubicoa who spare the following veins with extrapelvic communication (see diagram below).
Vulvar varicose veinsare more common during the fifth month of second pregnancies. However, they often disappear during the first month after delivery (31). They are more prevalent on the left side, but they can occur on both sides and in some cases the obstetrician prefers to perform a C-section incision since episiotomy, if required, could bleed heavily.
LL varicose veins of pelvic origin should be assessed with special care since they do not respect the saphenous axes and their appearance and distribution in the LL responds to the above-mentioned leak points. When they appear semiologically, we should complete the diagnostic tests while suspecting the presence of a possible PCS in case this was not the reason for the patient’s consultation. Otherwise, relapses and failed phlebological therapies can be a common thing here. Another indirect cause for patient’s consultation regarding PCS can precisely be LL varicose relapse. Perrin et al. showed PVI in 17% of 170 patients with relapse (32). Another study of 100 women with PVI (as seen on the venography and confirmed in the clinical examination in 71% of the cases) revealed the presence of leak points on the LL (in 58% of the cases). Actually, two thirds of these women showed varicose relapse after previous stripping (8,32).
Finally, obstructive PCS associate typical symptoms of venous compressions. Regarding CNS, pain on the left side and macro- or micro-hematuria can occur, sometimes intermittently. For several authors, when it is present it is the ultimate indication for treatment and to abandon a conservative treatment stand. The cases of MTS can present with edema associated with atypical left LL varicose veins. However, let us rememeber that compressions on the right iliac veins are also present.
184.108.40.206 Differential diagnosis
The diagnosis of PCS is achievingafter discarding other causes, mainly gynecological ones of CPP since these are common in the age group afflicting patients with PCS.28Similarly, PCS as cause of CPP is very common and it represents the second etiology of CPP after endometriosis.26 PCS is an misdiagnosedcondition, one on which we should use all diagnostic tools available since, in some cases, the PCS can be associated with other gynecological conditions and make clinical signs confusing (24,26). That is why the interdisciplinary differential diagnosis of this condition should be the rule of thumb. The assessment of patients should include initially a thorough clinical examination with questions and physical examination oriented to PVI and other gynecological and non gynecological causes (33-35). This early assessment will lead to the selection of laboratory and invasive and non-invasive imaging tests, which will eventually guide the ultimate diagnosis and therapeutic decision-making process.
In this diagnostic stage, it should be possible to differentiate 2 groups of patients:
In order to be able to achieve the differential diagnosis of PCS, the Royal College of Obstetricians and Gynaecologists (RCOG) (23) classification of the Gynecological and Extra-gynecological causes of CPP should be cited:
220.127.116.11 Clinical evaluation of the results
of the therapies applied
Clinical Severity Score
In the clinical assessment, it is important to know the scores that quantify the symptoms and signs of PCS and group the patients in comparable diseased groups. This way it is feasible to obtain greater accuracy regading the estimation of the results obtained with the therapies applied. Regarding clinical assessment, several authors have used indices such as quality of life (QOL) or the visual analogue score (VAS) validated to be used to measure PCS (7,36). The classification of patients based on the clinical signs and pathophysiology has been proposed recently (37-39), seeking to differentiate subgroups of patients with PCS and observe the response to the therapies prescribed. To this date, a clinical severity score PCS (PCS-CSS) has been presented to assess these patients’ pain with a score and also be able to unify criteria (40) (Table 1).
Regarding this clinical severity scale (PCS-CSS), different scores are assigned to the study patient:
• Minimum score: 0 points.
• Maximum score: 30 points.
o Absent or unlikely: 0-3 points
o Mild: 4-7 points
o Moderate: 8-15 points
o Severe: >15 points.
Several medical societies proposed years ago standard forms to report findings and results (7), and despite the important worldwide experience in the diagnosis and treatment of PCS, studies of high scientific quality are still scarce. To this end, important efforts are being made, worldwide, by different medical societies involved in the management of PCS. The International Union of Phlebology (IUP) has recently presented a very comprehensive consensus document on this regard (37) and other societies are evaluating unified ways to study and classify patients with PCS (38,39), among them the American Vein and Lymphatic Society (AVLS) (37-40).
18.104.22.168 AVLS Classification (40)
This anatomo-clinico-pathophysiological classification considers a gradation of symptoms (S), venous signs (V), and damaged venous territories (P), where the latter concept is in turn sublcassified into anatomy (A), type of hemodynamic alteration (H), and etiology (E).
S0: No clinical signs of venous disease.
S1: Renal symptoms (pain on the side, hematuria).
S2: Chronic pelvic pain due to vein incompetence (pain, dyspareunia, dysuria).
S3: Transitional symptoms (leak points: gluteal, pudendal, vulvar).
S4: LL symptoms:
a. Unspecified symptoms (pain, edema).
b. Venous claudication.
Signs – varicose reservoirs involved: “V”
V0: No varicose veins in the imaging clinical assessment.
V1: Renal hilar varicose veins.
V2: Pelvic varicose veins.
V3: Transitional varicose veins (leak points) (included in CEAP).
V4: LL varicose veins (included in CEAP).
LRV (left renal vein).
OV (ovarian veins:).
IV (common iliac veins; (external iliac veins;) (hypogastric veins;).
P (perforator pelvic veins).
Hemodynamic Anomalies: (H)
R (reflux) – decompensated.
RC (reflux) – compensated.
O (occlusion) – decompensated.
OC (occlusion) – compensated.
Thrombotic: due to DVT (reflux/obstruction).
Non-thrombotic: primary reflux or compression syndromes (reflux/obstruction).
Congenital: vascular malformations; venous or mixed.
In conclusion, PCS poses a diagnostic and therapeutic challenge. Therefore, integrating the knowledge of several medical specialties is required.
4.3 Diagnostic imaging modalities
4.3.1 Color-coded Doppler echocardiography
Authors: Luis Miguel Catalina, Carlos D´Alotto
Inside the diagnostic algorithm of patients with CPP and/or suspected PCS, the color-coded Doppler echocardiography (CDE) is in the first diagnostic line. This is mainly due to the fact that it is a non-invasive, low-cost imaging modality that provides real-time hemodynamic information and allows the assessment of the patient in different positions (decubitus, standing) using provocative maneuvers (Valsalva), which increase the sensitivity of this imaging modality regarding the detection of pelvic varicocele (1-3)
The main objective of this segment in this consensus document is to establish the study protocol and diagnostic ultrasound and Doppler criteria to identify pelvic varicocele and be able to select patients who will be examined using an abdominopelvic phlebography to plan endovascular therapeutics.
22.214.171.124 Study protocol
We believe that the study protocol should be comprehensive and include all the possible sectors where abdominal and pelvic veins can be affected and develop pelvic varicocele including pelvic leaks and development of LL varicosities.4
For this reason, the study protocol has been divided into 4 different steps:
1. Assessment of compression syndromes: NCS, MTS.
2. Assessment of gonadal axes.
3. Assessment of internal iliac axes.
4. Pelvis-LL connection: Assessment of pelvic leak points.
126.96.36.199 Access routes and patient preparation:
Currently, there are 3 ultrasound-guided access routes used to assess PCS:
1. Transabdominal (3-5 Mhz convex transducer). This access route is used to study the abdomen and the pelvis in the search for high (NCS) and low (MTS) compression syndromes, view the proximal and medial sectors of gonadal axes, and the iliac confluence (external and internal iliac veins). Also, it is useful at pelvic level to obtain a panoramic general view of the periuterine and periadnexal venous structures prior to transvaginal (TV) assessment.
It allows us to examine the patient in different positions (decubitus and standing) by increasing the sensitivity of the Doppler study to detect vascular venous compressions. If possible, the operating table should be in an inclined plane (about 30°) to increase the study sensitivity.
2. Transvaginal (TV) (intracavitary transducer). It is the access route of choice to assess periuterine plexuses and the cervicovaginal plexus. It provides a focalized visualization of them and a correct performance of the Valsalva maneuver looking for refluxes. If it is possible to incline the operating table, we suggest doing so at approximately 30° to increase the sensitivity of the study.
3. Pelvic leaks (7-12 Mhz linear transducer). We use this access route to assess the 4 main pelvic floor leak (PFL) points where the pelvic reflux connects to the LL (inguinal point: I; perineal point: P; obturator point: O; gluteal point: G). This study should be performed whenever there is clinical evidence of subdermal varicosities at inguinal or vulvo-vaginal level, on the thigh inner and posterior side, which is known as atypical varicosities.
There are two ways of study leak points: with the patient standing in a position similar to that used during the venous assessment of LL or else with the patient lying in decubitus in the lithotomy position; the latter is the one the authors prefer because it exposes the pelvic floor much better for the evaluation of the 4 leak points and it is often a more comfortable position for the patient. The drawback here is that venous refill is usually better in the standing position so when in doubt, the patient should be examined while standing keeping in mind that this often increases detection sensitivity since all the venous structures increase their caliber while standing.
Regarding the patient’s preparation for transabdominal examination, we recommend an 8-hour fasting preferably with an empty bladder, so it does not compress the vascular structures and alters the findings. The anthropometric profiles of most patients with PCS are often favorable for ultrasound examination since they usually have a low body mass index, which in 80% of the cases is < 25.5 The TV access route and pelvic floor assessment not used to look for leaks do not require any special preparation.
188.8.131.52 Diagnostic criteria of vascular compression syndromes
In every stenosis or vascular compression, whether arterial or venous, vascular flow changes occur at the compression site together with secondary changes that are distal to of compression site. These vascular flow changes are findings we look for on the Doppler echocardiography to achieve the diagnosis.
The changes that occur at the compression site are called direct signs and those that are distal to the compression site are called indirect signs. Direct signs are often more sensitive and specific compared to the indirect ones since they involve direct ultrasound imaging of the compression site and flow assessment at this level.
The nutcracker syndrome (6-11)
Direct signs (Figure 2):
• Aortomesenteric clamp (AMC) < 3 mm (normal: 4 mm to 5 mm).
• Absence of flow in the left renal vein (LRV) in the AMC.
• Diameter ratio; caliber of the LRV hilar segment/caliber of the AMC segment: ≥ 5.
• Velocity ratio; peak velocity at AMC/peak velocity of hilar segment: ≥ 5.
• AMC angle < 30º to 35°.
• Dynamic assessment while the patient remains standing or seated: in the true compression there may not be changes with respect to decubitus or an even greater closure of the AMC can occur with increased dilatation of the LRV hilar segment. In pseudo-NCS there is ofen decreased compression and, therefore, a reduction of the LRV caliber in the hilar segment.
Indirect signs (Figure 3):
• Collaterality through renal hilum and paravertebral veins.
• Collaterality through left gonadal vein (derivative flow type).
The May-Thurner syndrome
Direct signs (Figure 4):
• Total collapse of left primitive iliac vein in the junction with the right primitive artery with absence of flow in the Doppler examination.
• Diameter ratio: Caliber of segment prior to crossing/caliber of iliac crossing: ≥ 5.
• Velocity ratio: peak velocity at iliac crossing/peak velocity at segment prior to crossing: ≥ 5.
Indirect signs (Figure 5):
• Flow asymmetry in external iliac veins (the left external iliac vein loses its respiratory phasicity and spectral flattening occurs).
• Flow reversal in left internal iliac vein.
• Increased flow in right internal iliac vein.
• Presence of left ascending lumbar collaterals and uterine and cervicovaginal plexuses.
184.108.40.206 Diagnostic criteria of pelvic varicocele
There are 2 venous axes that can be assessed on the CDE that can lead to developing pelvic varicocele. They are the gonadal veins (left and right) and the internal iliac or hypogastric veins (right and left).
The assessment of gonadal veins begins from their proximal sector using a transabdominal transducer while trying to locate the ostial sector that will be found on the right side, in the inferior vena cava (usually between hours 9 and 11), and on the left side at the middle third of the LRV level. Then, the gonadal veins run anteriorly and in close correlation with the psoas-iliac muscle, which should be our main anatomical reference since it can be easily identified on the ultrasound exam (Figure 6).
We use the following reference measurements in the cross-sectional view of gonadal veins:
• 4 mm: normal.
• 4 mm to 6 mm: borderline.
• >6 mm: dilated.
Regardless of venous caliber, it is always important to functionally assess the vein using the Valsalva maneuver to rule out venous reflux; once we see it dilated on the cross-sectional tracking, we perform a longitudinal cut of the vein and assess the presence of venous reflux using a color-coded spectral Doppler echocardiography. We are interested in the proximal (ostial) and medial sectors of the gonadal veins via transabdominal access (Figure 7).
The distal sector at pelvic level is often more difficult to assess via transabdominal access; gonadal veins at this level usually look plexiform with multiple connected venous structures, which is why we prefer to use the TV access to perform this assessment (Figure 8).
Venous reflux, whether from the internal gonadal or iliac axes, will unload at plexoperiuterine and cervicovaginal vein level (Figure 8). The definition of PCS consists of an increase in both number and caliber of intrapelvic venous structures. These are veins with a varicose, tortuous, and ectatic morphology and with an ectatic slow flow.
From this definition the diagnostic criteria for PCS via TVaccess can be established:13,14 (Figure 9):
• Tortuous periuterine plexus veins of between 5 mm and 6 mm caliber.
• Dilated veins of the intrauterine arcuate plexus crossing the body of the uterus towards the contralateral side (pelvic collaterality).
• Confirmation of > 5 dilated venous structures.
• Slow ectatic flow (sometimes it is identified as venous smoke or rouleaux flow).
• Presence of reflux after the Valsalva maneuver.
The visualization of dilated periuterine venous structures bilaterally can occur as a sign of collaterality of an incompetent gonadal axis, generally the left one, where the reflux seeks a return pathway towards the IVC through the contralateral gonadal axis. Or else, it can be a sign of bilateral gonadal incompetence, where both axes reflow contributing to pelvic varicocele. This situation is often differentiated using the Valsalva maneuver where the incompetent axes will show reflux, while the axis that acts as physiological return with hyperflow of centripetal direction will not show reflux after the same maneuver. We should mention that Dr. Leal Monedero’s group describes 2 possible situations that can occur in the flow of venous structures of the periuterine plexus and the gonadal axis in the presence of pelvic varicocele (1).
• Continuous venous flow interrupted after the Valsalva maneuver. According to the author, this finding is highly indicative of a derivative type of flow as it occurs in vascular compression syndromes (NCS and MTS) (Figure 10).
• Slow or absence of flow in pelvic varicosities showing reflux after the Valsalva maneuver. This is indicative of primary gonadal or iliac internal incompetence.
Internal iliac or hypogastric veins
According to the PCS study protocol, internal iliac veins are the most difficult vascular structures to assess through CDE. This is due to the fact that they are short veins that emit multiple segmental branches, they are located in the depth of the pelvis, they are hard to assess functionally with dynamic maneuvers whether transabdominal or transvaginally. They are often valveless veins, unlike their segmental visceral or parietal branches (15). Studying the iliac confluence via transabdominal access is advised. For all this and contrary to what occurs with diagnostic signs in gonadal axes and compression syndromes, the indirect signs seen in the iliac axes are often more important than the direct signs when studying these veins.
Internal iliac veins have visceral (uterine, vaginal, vesical, rectal) branches, and parietal tributaries (gluteal, perineal, obturator); the latter have an extrapelvictrajectory towards the LL.
The assessment protocol of internal iliac axes would be next.16 (Figure 11):
• After gonadal axes have been assessed and they appearnormal, and there are no periuterine or cervicovaginal intrapelvic venous dilatations but atypical varicosities in the LL, it is assumed that they are fed by venous pressure from the internal iliac and/or parietal segmental branches.
• After gonadal axes have been assessed and they appear normal, and there are no intrapelvic venous dilatations but atypical varicosities in the LL, it is assumed that they are fed by venous hypertension from the internal iliac and/or parietal segmental branches.
• In both cases, whenever there is clinical evidence of atypical varicosities in the vulvo-vaginal, perineal, gluteal regions the PFL points should be assessed.
From what has been explained we should mention that, in the TV study, the periuterine-periadnexal plexus (high plexus) and the cervicovaginal plexus (low plexus) should be routinely evaluated (Figure 12 A and B). The latter is assessed by removing the TV transducer almost up to the vaginal introitus by performing the Valsalva maneuver. In the absence of significant periuterine plexus dilatation, but in the presence of low cervicovaginal plexus dilatation, it is highly indicative of segmental incompetence of the internal iliac branches, mainly the internal pudendal and obturator veins.
220.127.116.11 Pelvic leak points
Pelvic leak points are the connections between the intrapelvic venous system and LL veins. From the comparative point of view, they would be similar to LL perforator veins that connect the superficial to the deep venous system. These are the points through which the venous hypertension originated at the pelvis seeks to unload towards the LL superficial venous system.
Traditionally, 4 leak points are examined and described (Figure 13) (16,17):
• Inguinal point (Point I)
• Perineal point (Point P)
• Obturator point (Point O)
• Gluteal point (Point G)
We should mention that there are no ultrasound anatomic landmarks that can be used to look for them. Therefore, they are searched for based on the clinical examination of the subdermal varicose trajectories on the perineal and LL regions. They are followed in ascending direction until the area where they enter the pelvis can be seen. This is how it is suspected that a leak point may be involved in the origin of the varicose trajectory.
Inguinal point (Figure 14)
It is the re-patency of the round ligament vein; varicose trajectories are seen close to the pubis and groin that bringing their reflux into the aortic arch of the great saphenous vein or its tributaries (superficial inguinal veins). The varicosities come from above the inguinal fold and drain into the LL.
Perineal point (Figure 15):
It is located on the pelvic floor and is responsible for vulvo-perineal varicose trajectories towards the inner thigh; it is searched by moving the transducer parallel to the vulva. It is usually one of the most common leaks, it is associated with internal pudendal vein incompetence, a segmental branch of the internal iliac vein.
Obturator point (Figure 16):
The obturator point can be found underneath the inguinal fold, close to the SFJ. It holds an anterior location to the perineal point. Reflux comes from the obturator vein, which is a segmental intrapelvic branch of the internal iliac vein. Due to its intermediate location between the inguinal and the perineal points, it is responsible for varicosities both at vulvo-vaginal and inner thigh level.
Gluteal point (Figure 17)
The reflux leaks through the superior and inferior gluteal veins, both extrapelvic branches of the internal iliac vein. Two points are described: leaks through the superior gluteal vein are topographically located at the middle third of the gluteus and leaks through the inferior gluteal vein are topographically located through the inferior gluteal fold. The compromised territory is the thigh posterior side that follows the trajectory of the sciatic nerve.
18.104.22.168 Color-coded Doppler echocardiography
during postoperative management
Afer completing the diagnostic part (study protocol and diagnostic criteria for PCS), in the last section of this consensus document we will be describing the utility of CDE during the postoperative management of patients with PCS.
Among the endovascular therapeutic possibilities used today, there are 2 excellent choices available (18-20): percutaneous transluminal coronary angioplasty (PTA) with venous stent for the management of compression syndromes; and transcatheter embolization (TE) for the management of gonadal and internal iliac axe incompetence. In both cases, the CDE becomes an excellent diagnostic tool for postoperative management purposes both to detect complications and to assess therapeutic success. The same study systematics and approaches described should be used during the postoperative and diagnostic stage alike to assesss the results of the different endovascular and/or surgical therapies implemented.
The control CDE should be performed every 6 months for follow-up purposes and assessment of the PTA with stenting and TE (18,19).
During stent control we will be looking for (Figures 18, 19, 20, 21, and 22):
• Input, in-stent, and output velocities.
• Correct positioning (migration).
Using the mixed TE technique (coils and foam) we should become familiar with post-embolization findings. These are often the findings we are talking about (Figure 23):
• Complete occlusion of the venous axis treated, whether gonadal, internal iliac or its segmental branches.
• Findings of small refringences (hyperechoic comet-tail artifact images) along the vascular axis treated correspond to the coils and the foam artifact often present at adnexal area level.
Within the diagnostic algorithm of patients with CPP and clinical suspicion of PCS, ultrasound studies and CDE are among the first diagnostic imaging modalities of choice. Using the diagnostic criteria described, this imaging modality has acceptable percentages of sensitivity and specificity that are often above 90%. There is no doubt that this study has limitations since among the PCS diagnostic studies it is the most operator-dependent one. Therefore, to a greater extent, its diagnostic performance will depend on the operator’s skill and training. Other limitations can be the significant presence of abdominal and pelvic gas that will be limiting the visualization of vascular structures and the scarce penetration of ultrasound waves senn in some obese patients, but these are less common. We consider that the comprehensive approach is very important in the study of PCS. It should include a transabdominal, transvaginal, and pelvic floor approach to rule out leaks towards the LL. This is how all the possible causes that trigger pelvic varicocele and the atypical varicosities it can cause on the LL can be studied comprehensively.
Authors’ note: the ultrasound images contained in this chapter were obtained from patients actually. examined by the authors. The diagrams shown are modifications made by Dr. Guillermo Eisele from the references cited in each case.
22.214.171.124 Recommendations (21)
4.3.2 Computed axial tomography scan
and magnetic resonance imaging
Authors: Thiago Vasconcellos, Carolina Chacon, Pamela Inés Causa Andrieu, Noelia Napoli
Imaging studies have diagnostic value in patients with suspected of. Not all patients with venous dilatationare symptomatic,1 the history of pregnancy and vaginal delivery is often an example of this situation, that does not cause any symptoms or require treatment. Computed axial tomography scan (CAT scan) and magnetic resonance imaging (MRI) are non-invasive imaging modalities that are used to identify venous dilatations, look for signs of venous reflux and, eventually, determine the compromised territory and presence of venous compression/obstruction, diagnose vascular or non-vascular conditions that cause the syndrome, and rule out other causes of chronic pelvic pain or establish differential diagnoses.
Both imaging modalities obtain a number of images that can be reconstructed in both 2D and 3D. The CAT scan uses radiation (the amount of radiation used can be controlled using radiation modulation techniques provided by state-of-the-art scanners) and IV iodinated contrast with potential nephrotoxic risk. Therefore, it is important to assess the kidney function before performing this examination. The MRI uses radiofrequency pulses in a given magnetic field plus IV gadolinium as contrast agent without the risk of allergic reaction to iodine. Eventually, the MRI can reveal pelvic venous dilatation with better resolution compared to the CAT scan, without IV contrast, and characterize other causes of chronic pelvic pain (endometriosis, adenomyosis, myomas). In both imaging modalities the patient remains in the supine position during the examination and the varicose veins can be less prominent.
The study covers the abdomen (from the renal veins) and the pelvis in order to characterize different drainage paths and possible compression causes.
Dynamic assessments with IV contrast are required to obtain images in the arterial (to see venous reflux and assess eventual vascular arterial causes) and the venous phase (venography, to measure vascular diameters, assess the venous territory compromised, and identify the venous vascular obstruction). Acquisitions in apnea without inspiration are advised here (to avoid changing the aortomesenteric angle, which could eventually alter the correct interpretation).
126.96.36.199 How to request the study
The request these studies we should specify the type of examination, abdominal and pelvic venous angiography with IV contrast, the imaging modality required, whether tomography or magnetic resonance imaging and the presumed diagnosis so that the radiologist can select the study protocol correctly, for example: chronic pelvic pain, suspected pelvic venous congestion. The study of the lower limbs is associated with the clinical context and it is necessary to specify it in the medical request as an additional region to be assessed.
The magnetic resonance venography (MRV) seems to have good diagnostic precision compared to conventional selective venography, but the evidence available on this regard is limited are there are very few good quality studies (Evidence level II-1: controlled non-randomized clinical trials), which is indicative of an 88% sensitivity rate and a 67% specificity rate for the identification of gonadal vein incompetence (2,3).
CAT scan venography does not have proper correlation studies with conventional venography (Evidence level II-3 / III: multiples series compared in time, descriptive studies) (3).
The main pathophysiological mechanism of PCS is the valvular insufficiency of gonadal veins, and it basically affects adult, multiparous women. The findings suggestive of a diagnosis of PCS in the CAT scan and the MRI are:
• Gonadal venous reflux: visualization in the arterial phase of the gonadal vein.
o Grade I: left ovarian vein opacification (it does not reach the parauterine veins).
o Grade II: retrograde flow advanced towards the left parauterine veins.
o Grade III: retrograde flow crosses the midline through the uterus towards the right parauterine plexus (2-5) (Figure 24).
• Gonadal veins: >8 mm in diameter (6-9). They are measured using IV contrast in the venous phase, where the vascular diameter is more visible. We suggest taking the transversal diameter on the coronal plane (Figure 25).
• Parauterine veins: 4 or more tortuous homolateral veins with one of them > 5 mm (6-9) (Figure 25).
We suggest considering and assessing the presence of perivaginal, periurethral, presacral, venous plexus dilatation, drainage towards the hypogastric territory, and venous leak point assessment.
Rare pathophysiological mechanisms associated with extrinsic venous compressions affect a generally younger, nulliparous population, and they can be suitably assessed using these imaging modalities, such as the nutcracker syndrome (Figure 26), where there is left renal vein compression between the aorta and the superior mesenteric artery; we should see whether there is a reducedaortomesenteric angle (normal >25°) and aortomesenteric distance (normal >10 mm).
In the May-Thurner syndrome (Figure 27) we find the left primitive iliac vein compression exerted by the right primitive iliac artery; other compression causes can be associated with a large uterus (due to the presence of myomas or adenomyosis).
The following is a list of the aspects that we should study in the images of patients wuth suspected PCS (8)
In the abdomen:
• Left renal vein (LRV): the nutcracker syndrome.
• Left primitive iliac vein: the May-Thurner syndrome.
• Gonadal veins: normal anatomy (right gonadal vein draining into the IVC and left gonadal vein draining into the LRV). Diameter >8 mm. Retrograde flow.
In the pelvis:
• Pelvic varicose veins: tortuosity/dilatation (diameter >5 mm)/amount.
• Involvement of internal iliac veins.
• Varicose veins of atypical location (examine the leak points: inguinal, gluteal, obturator, etc., and their association with pelvic varicose veins and PCS).
During imaging monitorization that follows the endovascular treatment (Figure 28) it is particularly adviseable to assess the clinical signs and identify the possible redistribution of venous drainage into other territories (eg, the hypogastric region). Both imaging modalities are useful for monitoring purposes, there may be an artifact associated with the embolization material when using MRI images.
Imaging studies have diagnostic value in patients with suspected PCS. Both imaging modalities, the CAT scan and the MRI, complement the Doppler echocardiography (the modality of choice) and have the proper diagnostic precision to be able to diagnose PCS; the latter recommendation has a II-1 level of evidence for the MRI (controlled non-randomized clinical trials in association with the conventional selective venography) and a II-3/III level of evidence for the CAT scan (multiple series compared across time, descriptive studies). Both imaging modalities are adequate for monitoring purposes of the therapy administered; also, the MRI is a useful imaging modality to diagnose other causes of non-chronic pelvic pain (eg, endometriosis, adenomyosis, myomas). Finally, we always recommend examining the patient’s clinical signs and working together with the physician who requested the study for a correct interpretation of the imaging modality at stake.
188.8.131.52 Recommendations (10)
4.3.3 Dynamic gonadal and iliac phlebography
Authors: Damián Simonelli, and Guillermo Eisele
The pelvic congestion syndrome (PCS) is part of an even greater syndrome called chronic pelvic pain (CPP) described by Richet back for the first time back in 1857 (1).
Although the progress made in non-invasive diagnostic imaging modalities (CDE, CAT scan, and MRI) facilitates the diagnosis of PCS with satisfactory sensitivity and specificity rates, the gonadal and iliac dynamic phlebography (GIDP) is still considered essential for the diagnostic stage in the presence of inconclusive findings of the non-invasive diagnosis of PCS and in the assessment prior to endovascular treatment.
Technical aspects will be detailed in this chapter as well as the importance of GIDP in the venous catheterization of PCS.
Pelvic organs drain into their venous flow both to right and left through the large internal left and right iliac veins.
These veins meet in the pelvic wall with the external iliac vein, continuation of the femoral vein, making up the primitive (right and left) iliac vein; the convergence of these primitive iliac veins at the level of the right anterolateral side of the body of the fifth lumbar vertebra make up the beginning of the inferior vena cava (IVC).
Another drainage path of venous flow is both gonadal veins which, by deriving the blood flow coming from the ovaries and the homolateral Fallopian tube, ascend parallel to the spine to drain directly into the IVC from the right gonadal vein and into the left renal vein (LRV) in the case of the homolateral gonadal vein.
Didactically speaking, if we divide pelvic veins into 2 groups, on the one hand we will find veins running through the pelvic wall. On the other hand, we will find veins comings from the pelvic organs, called parietal and visceral, respectively. Among the parietal veins we can identify the gluteal, sciatic, pudendal, obturator, and lateral sacral veins. The first 4 branches mentioned have a high capacity of showing anastomosis with LL veins. Therefore, they are indicative points of probable flow reversal for the outflow of venous content from the pelvis called pelvic leaks.
In the case of gluteal veins, anastomoses will occur on the gluteus medius and gluteus maximus inferior lines and edges, and they will be noticeable on the thigh posterior side. Obturator veins can anastomize with the inner thigh branches. Internal pudendal veins can anastomize with the external pudendal veins through perivulvar and sciatic venous branches with deep femoral vein perforator branches in the LL thighs (2,3).
On the other hand, lateral sacral veins can often show an important number of anastomoses with contralateral lateral sacral veins, which is why this is a very effective path to derive flow from left to right or vice versa. Congenitally speaking, unlike gonadal and iliac veins, sacral veins are valveless.
Perivesical plexus is among the visceral veins, which in its lateral portion drains into the vesico-prostatic plexus and it can also drain through numerous branches of the internal iliac vein (4).
The medial hemorrhoidal veins run along the sides of the rectum making up a plexus that ends in 1 or 2 bundles that eventually drain into the internal iliac vein. They run across the sacro-lateral ligaments, contrary to the superior hemorrhoidal veins (branches of the portal system).
Vaginal and uterine veins drain into the internal iliac vein through bundles located at the wide ligament (5) (Graphic 1).
We can have a single (in 50% of the patients) or a duplicated internal iliac vein or it can have a venous plexus presentation (6). According to Champaneria, the incidence rate of internal iliac vein duplication or its plexus presentation is 30% (7). In a study of 42 cadavers, Le Page determined that the internal iliac vein was present as a single trunk in 73% of the cases, and as 2 trunks in 27%. Another interesting fact revealed by this study is the presence of valves in the external iliac vein in 26% of the cases, and the presence of valves in this vein was 3 times more common on the right compared to the left side (4).
The main venous trunks and their ostia are subject to multiple anatomical presentations that vary regarding their location, duplication, etc. (Figures 29 and 30).
All of these are valved veins, except for the diseased ones that generate leak points and presacral plexus.
184.108.40.206 Gonadal and iliac dynamic phlebography
GIDP is the most sensitive and specific diagnostic imaging modality (80% to 100%) and it is considered the gold standard in the diagnostic tests of PCS.8-10 This minimally invasive study provides a highly precise dynamic venous assessment, confirms the presence of obstructive lesions, and facilitates the endovascular therapeutics guidance. During the same procedure it is possible to perform both the diagnosis and the therapeutics. With a sensitivity similar to transvaginal (TV) CDE, it provides a graphic representation of the affluent and effluent varicose trajectories. To a large extent pelvic phlebography is based on performing the technique correctly: it is necessary to attemptvery distal catheterizations in the internal iliac veins tributary branches and inject the contrast agent while using the Valsalva maneuver to properly visualize the pelvic leaks.
Both the CCTA (coronary computed tomography angiography) and the angio-MRI are highly sensitive and specific imaging modalities to study PCS and compression and/or occlusive syndromes. Still, they need accurate measurements of venous times in the contrasted series and avoid artifacts that can degrade the diagnostic quality significantly. However, it does not quite work like this inleak point or reflux assessmentswherethe selective and distal GIDP is obviously more effective.
The invasive character of GIDP as a diagnostic imaging modality is justified by the fact that within the same procedure it is possible to continue with the endovascular treatment.
Before performing a GlPD, the operator should already be familiar with the patient’s clinical signs and findings obtained from non-invasive studies.
This study can be performed outpatiently under local anesthesia or, if required, adjuvant neuroleptoanalgesia. We should remember that, for a proper dynamic phlebography assessment, the patient should cooperate and remain lucid. In addition to the informed consent, we teach patients to perform the maneuvers of deep inspiration accurately (Valsalva and apnea). General anesthesia is exceptional and spared for certain clinical or mood conditions of the patient.
The GIDP prior assessment includes lab tests withcoagulogram and kidney function tests since the average iodinated contrast volume injected is 120 ml.
With the patient in the dorsal decubitus position and after the administration of proper antiseptics, venous access is often obtained through an ultrasound-guided puncture or with anatomical landmarks by inserting a 5-Fr-or-6-Fr vascular guidewire using the Seldinger technique.
Vascular access can be obtained via femoral or internal jugular access route, although jugular and/or brachial accesses are more comfortable and easier to perform regarding the study of both sides of the pelvis (Figure 31).
Access via jugular vein makes it difficult to use the radiation protection screen (Figure 31) and, statistically speaking, it is associated withmore complications (pneumothorax, inadvertent puncture of the carotid artery).
In most cases some authors often use the arm basilic vein. Also, we always shave and administer antiseptics on the right groin in case right femoral vein access is required when the retroaortic access has gone unnoticed on previous tests or when the right gonadal vein catheterization is impossible from the superior vena cava. Occasionally, it is more accessible via femoral vein for catheterization purposes with the Simmons 1 catheter using a distal microcatheter to administer microcoils therapy or see the right gonadal vein ostium. Also, a multipurpose catheter from the brachial vein is often use for catheterization purposes.
After vascular access is obtained, the purpose of the study is to obtain dynamic images of the 4 main venous axes of pelvic drainage.
In the early studies of hypogastric veins and their eventual leaks it would not be necessary to use a vesical probe; on the contrary, if the diagnostic study starts with gonadal and LRV assessment or diagnosis and endovascular treatmentare anticipated within the same procedure, vesical probingshould come before the GIDP (11,12).
The GIDP protocol of some authors consists of studying the left renal vein and the left iliac vein to rule out any possible compression syndromes. Then, move on to study the right gonadal vein because its access is more complicated. Afterwards, the left gonadal vein should be studied since the LRV has already given us an idea of the morphology that we will eventually find. Lastly, we should study the internal iliac veins to see any possible refluxes (13). Vesical probing is recommended since diagnostic procedures are always complemented by a TE therapeutic procedure.
If the catheterization of the right gonadal vein with the multipurpose catheter is not possible, we should move on with the next step in the work protocol, thus studying the remaining veins. Once the study has been completed, the catheter should be changed to access the right gonadal vein.
Using a 5-Fr 100 cm or 125 cm-long vascular preformed multipurpose catheter with distal orifice (Figure 32) and under the protection of a Terumo hydrophilic guidewire (curved tip, 0.035 in, 150 cm-to-180 cm long) (Figure 33), the goal was to catheterize and draw a sketch of both renal veins, the gonadal veins, the primitive and hypogastric iliac veins, the femoral veins, and their most significant bundles. With the current material, it is not usually necessary to use long introducer sheaths.
Contrast injections are performed manually with 10 mL-syringes using pure idonated contrast or at 20% dilution.
Ideally, the best thing to do is to use low radioscopic doses and radioscopic registers to reduce secondary radiation. A full study should not exceed 50 mGy.
For the registration of series in digital substraction, cadence does not require more than 2 images per second. Ideally, the panoramic fields also minimize the need for multiple focal registrations of small fields. To improvethe quality of the images, compression syndromes are studied in apnea to obtain more precise assessments of the compression notches and the collateral circulation.
Left renal phlebography has 2 different purposes: to assess renal vein stenosis and the presence of venous reflux towards the homolateral gonadal vein. Its other objective is to assess the possible presence of other collateral branches that may develop in compression syndromes (perirenal, paralumbar, paravertebral, and pararenal) (Figure 34).
We shall begin by positioning the catheter in the LRV trunk and performing a renal phlebography with and without the Valsalva maneuver (6) (Figure 35).
In the cases of NCS, stenosis of the LRV trunk can be confirmed (6). Afterwards, the hemodynamic study will be completed with the measurement of venous pressures through the stenosis, first looking into proving the existence of a pressure gradient >3 mmHg according to Greiner (6) or else ≥5 mmHg according to Monedero (13) between the proximal and distal portions to the stenosis.
Pressure should always be measured (Figure 36), not only in case of NCS.
Secondly, we will try to catheterize the compression of the LRV. The most common one is to reduce the aortomesenteric clamp (AMC) angle. However, the retroaortic renal vein runs behind the aorta in its way towards the IVC, which also generates anterior compression (much less common); the third option is to have a circumaortic renal vein to reduce the caliber in the anterior and posterior passage towards the aorta (even less common).
These anatomical variants open the possibility of compressing the LRV in the aortomesenteric space or between the spine and the abdominal aorta, thus causing syndromes (NCS) with a lower back pain component, possible hematuria, and syndromes derived from venous collateral circulation while trying to decompress renal venous drainage, which can originate PCS or left testicle varicocele when flow is diverted by the gonadal vein through reflux with flow reversal.
The gonadal vein develops PCS usually in thin women who could even be nulliparous. As a matter of fact, in these cases, we can see how the right gonadal vein has a normal morphology as opposed to the left one that appears clearly dilated and valveless.
The right renal vein often has a shorter trajectory and is also more angled with compared to the vena cava. Its possible variants do not have so much repercussion regarding pelvic venous conditions. Although, at times, we can say that the right gonadal vein drains directly into the renal one, some other times the gonadal branch drains into the renal vein, often accessory, of smaller caliber, thus communicating directly with the gonadal vein. Also, there are times that the right gonadal vein drains into the angle formed between the right renal vein and the inferior vena cava (Figures 37, 38, and 39).
Another aspect that should be characterized during renal phlebography is the presence of stenosis-related thrombosis and/or myofibrotic bands due to the myointimal aggression generated in the venous wall by the superior mesenteric artery arterial beat.
Performing a LRV phlebography with the Valsalva maneuver also gives us the possibility to diagnose left gonadal vein reflux. Although with this maneuver its presence is a conclusive sign of gonadal vein incompetence, its absence does not rule out the diagnosis. The explanation is that the GIDP is usually performed in the decubitus position since very few radiology machines at the cath lab have remote control tables capable of achieving the supine position. We do not know of any publications that show better sensitivity or specificity rates compared to phlebography in the supine position (Figure 40).
Left gonadal phlebography should be completed with its selective catheterization and opacification in order to specifically look for the following data:
• Diameter of the gonadal vein. Most authors estimate that a diameter of up to 5 mm is normal: mild incompetence (between 5 mm and 8 mm); major incompetence (between 8 mm and 10 mm); and severe incompetence with diameters >10 mm. Still, we should mention that the association between the diameter of the gonadal vein and the presence of reflux is not always so linear, as Dos Santos confirmed in his study conducted back in 2014 (14).
• Development of high paravertebral collaterals diverting renal flow towards the paravertebral plexus. This can be considered an indirect sign of misdiagnosed LRV stenosis or thrombosis. Still, the LRV thrombosis associated with the NCS is rare.
• Number and height of bifurcations. The gonadal vein commonly undergoes divisions at different heights that should be known on the GIDP since this can lead to relapses in all of its branches if not properly embolized (6).
• Presence of contrast retained for 30 seconds after the injection of contrast, which is strongly indicative of the presence of gonadal reflux and stasis.
• Flow reversal: the GIDP allows us to confirm incompetent gonadal drainage both spontaneously and thanks to the Valsalva maneuver. This incompetence can be so significant that it determines left gonadal flow diversion towards the contralateral side through the pelvic veins (lateral sacral) or towards the internal contra- or homolateral iliac vein. (Figures 41, 42, and 43).
Right gonadal phlebography looks for the same findings as it happens with the left side. However, we should mention that, if not dilated, its selective catheterization can be more difficult compared to the left side.
Iliac phlebography consists of catheterizing both the iliac and femoral veins by performing bilateral ascending phlebografies with and without the Valsalva maneuver (Figures 44, 45, and 46).
The study of the left iliac vein is often performed by injecting contrast into the external iliac vein proximal to the common femoral vein to obtain an ascending phlebography while in apnea and analyze the possibilities of compression syndromes, May-Thurner Syndrome (MTS) or other abnormalities. Afterwards, a selective study of both internal iliac veins and their anatomical variants is conducted with the C-arm in counterclockwise rotation (25º) and using the Valsalva maneuver to assess the possibilities of reflux or leak through the tributary veins.
In their origin the internal iliac veins are valveless and, as we have already said, so are the presacral veins. We do find valves in the internal iliac vein tributary branches except for those with incompetence consistent with pelvic leaks. In our work methodology we always perform a proximal injection that works as a general map for the selective distal catheterization of incompetent veins (Figure 47).
It is adviseable to start on the left side since the disease is more prevalent here. In these GIDPs we will try to rule out:
A. Significant stenosis of the primitive iliac vein. In case of MTS, the disease is significantly more prevalent on the left side and the stenosis corresponds to the primitive iliac artery crossing on the left primitive iliac vein trunk anterior side. Iliac venous thrombosis can be associated. The development of collaterals is also feasible (in general they are ilio-lumbar, ascending lumbar, and perivertebral).
According to Neglen, there is not such a thing as a standard pattern to determine the significance of a MTS venous obstruction, and athough small transtenotic pressure gradient differences (from 2 mmHg to 5 mmHg) can be hemodynamically significant, normal gradient measurements do not rule out the presence of critical venous obstruction. Therefore, the measurement of transtenotic pressures should be taken into consideration together with phlebographic, CDE, CAT scan, MRI images, and clinical signs. Arbitrarily, the intravascular ultrasound (IVUS) is capable of showing superior iliac vein stenoses > 50%. It may very well be the most precise imaging modality to define significant stenosis eligible for treatment with angioplasty and stent therapy (15).
We were able to confirm that thrombosis associated with the iliac stenosis of the MTS amplifies the existing obstructive symptoms. In cases of right MTS, the right primitive iliac vein is the compressed vein, and stenosis is often more longitudinal (not so cross-sectional) and it can expand quite a few centimeters from the proximity of the iliac confluent to the caudal. When the arteries are more elongated, we may find compressions on the right side too. We believe that there is MTS when the right iliac artery compresses the left iliac vein.
B. The GIDP of the MTS can also show membranes of myointimal hyperplasia caused by the vascular damage due to the arterial beat on the venous wall in the form of longitudinal negative images (without flow) of different lengths and without a precise distribution pattern. They are also called central synechiae, fibrous adhesions between the venous walls. When treatment is performed, the vein hourglass morphology can be seen directly when balloon dilatation is performed. In the presence of thrombosis, manipulation can be risky (Figures 48 and 49).
These synechiae are often associated with occlusive thromboses already diagnosed on the CDE, CAT scan or MRI (Figure 50).
There are times that the primitive iliac vein catheterization via jugulo-brachial access may become difficult. If so, this should inform us of the presence of severe stenosis that should eventually be studied from the corresponding LL.
C. Also, we should not rule out the possibility that a stenosis, thrombosis or anomaly reported on the IVC difficults the iliac veins catheterization. In general, in these cases, non-invasive diagnostic studies usually inform us on this situation, meaning that the patient should be studied from the LL.
D. Stenoses following extrinsic, asymmetrical and non-circumferential compressions with smooth edges and obtuse angles can be due to adenopathies. They should be distinguished from tissue invasion/infiltration of the venous wall as stenoses show irregular surfaces, acute or variable angles, and heterogeneous images. As in the previous point made, the patients’ clinical data and non-invasive studies should inform us on this diagnostic possibility.
E. It is important to confirm the presence of venous reflux towards the pelvis through the internal iliac veins, which can be the case because 80% of them are valveless. Unlike their tributary branches, internal iliac (and presacral) vein ostia are valveless.
F. Also, we should confirm whether there is flow diversion towards the contralateral side through different pelvic plexuses and whether after contrast injection some contrast remain for over 30 seconds in the pelvic veins (unequivocal sign of PCS). If so, this would be associated with postural mechanisms.
The last stage of the GIDP consists of the phlebography of both internal iliac veins and their branches. This part of the study is usually time-consuming, requires training, and the proper anatomical knowledge. In order to optimize the time that it takes to perform the study, the use of iodinated contrast and radiation exposure should be minimized, and the targets of this study should be crystal clear. Dr Zubicoa Ezpeleta’s study protocol includes the assessment of the possible compression syndromes first. Afterwards, the study of gonadal veins, and finally the internal iliac veins selective catheterization.
Clinical assessments and non-invasive studies should have already informed us on the presence of pelvic, vulvar, gluteal and/or LL varicose veins and suspect the presence of pelvic venous leak points (see section on anatomy). At this point, we should diagnose suprapelvic obstructive causes (NCS, MTS, thrombosis, hypoplasia, extrinsic compression of both the iliac vessels and the IVC), venous incompetence and reflux at both gonadal and iliac levels.
Uterine veins and internal iliac vein branches can also be involved in the development of periuterine varicose veins of the PCS. Therefore, in the study of internal iliac veins, added to the possible existence of leaks, we should rule out the presence of uterine branch incompetence and perform a TE procedure on this branch, when applicable (Figure 23).
In conclusion, the GIDP performed on internal iliac veins and their branches should inform on the following findings:
• Presence of incompetent pelvic varicose veins in uterine veins and internal iliac vein branches (internal pudendal, obturator, etc.).
• Diagnose hypogastric vein anomalies (duplications, and/or anomalous drainages or trajectories).
• Map and quantify the significance of major venous leaks into the homo- or contralateral LL and establish which will eventually require endovascular treatment (Figure 24).
Although the multipurpose catheter facilitates the acquisition of highly precise images of the leaks without having to use balloon catheters, it can be useful to use high-flow catheters or perform injections with balloon catheters that will partially occlude the internal iliac vein lumen facilitating the detection of venous leaks.
The main PFL points that should be studied are the superior and inferior pudendal, gluteal, obturator, and round ligament veins. The round ligament vein is a gonadal vein branch, unlike the others, that are internal iliac vein branches.
The systematic study of these pelvic veins and their annexes will be crucial to confirm or rule out diagnoses and make the corresponding therapeutic decisions. It is important to take into account that the information provided by the GIDP cannot be isolated from the patient’s clinical signs. Otherwise, it will just be an image and the results will not be the expected ones.
Several studies have established the phlebographic findings that define patients with PCS and PVI based on morphological and hemodynamic parameters. Still, their scientific quality is not good enough to confirm the sensitivity and specificity rates of GIDP. These findings are:
• Gonadal vein/s incompetence and dilatation.
• Utero-ovarian plexus varicose veins.
• Prolonged retention of contrast in the gonadal vein/s and the utero-ovarian plexus.
• Reflux from the utero-ovarian varicose veins towards the hypogastric and/or gonadal contralateral drainage.
• Reflux towards vulvo-vaginal varicose veins and/or LL varicose veins (16-18).
Back in 1984, Beard was the first one to describe a diagnostic score using pelvic phlebography via transuterine puncture by comparing patients with PCS symptoms to case-control studies whose findings had been extrapolated to the GIDP (9,12). This imaging modality has been totally abandoned today because it does not allow us to perform TE procedures when incompetence has been confirmed.
The 3 phlebographic criteria with which this score is used are:
• Larger diameter of the ovarian vein (< 5 mm; 5 mm to 8 mm; or >8 mm).
• Delayed contrast washout times in the ovarian vein (to 0 s, 20 s, and 40 s).
• Significance of utero-ovarian varices (normal, moderate or severe).
Each criterion measures a severity level that goes from 1 to 3, and the sum of 5 or more points in the score is indicative of the presence of PCS with a 91% and 89% sensitivity and specificity rates, respectively (10-12).
In his systematic review, Champaneria assigns scores ≥5, and odds ratio = 31 to the GIDP (7).
Therefore, it seems clear that the GIDP plays a key role in 3 different situations to achieve a definitive diagnosis of PCS:
• When findings from non-invasive studies are inconclusive.
• When it is necessary to confirm the findings from non-invasive studies.
• When endovascular treatment has been proposed as a possible alternative (1).
For these reasons, and despite these limitations, the GIDP is still considered the gold standard (17) imaging modality for the diagnosis of PCS.
Note: both the images and phlebographies showed in this chapter are actual cases managed by the authors and reviewers of this consensus document.
220.127.116.11 Recommendations (20)
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Revista Argentina de Cardioangiología intervencionista
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