The vasculature of the human body is variegated and complex. In 1616, William Harvey improved upon the works of Ibn al-Nafis and Michael Servetu with his description of a continuous circulatory system. In An Anatomical Exercise on the Motion of the Heart and Blood in Animals, Harvey eloquently and historically linked the veins and arteries in circuit with the heart. Beginning with these early writings, the pelvic veins and their disorders have been less well understood and often enigmatic in their pathologic presentation, partly because of to the variety and complexity of the descriptions of pelvic venous anatomy. The more recent focus on and understanding of this portion of the vasculature is exemplified by the revision of pelvic venous terminology within this decade after its early characterization in the 1800s.

Diseases of the pelvic veins have much to do with the heterogeneous anatomy of these vessels, with imaging advancements leading to vast improvements in diagnosis and directed treatment. Pelvic venous disease has been well described in women given the complex anatomy of the female genital system and the social and emotional association with conception and birth. This chapter focuses on the intricate anatomy and physiology of the pelvic venous system, including a description of congenital abnormalities, iatrogenic injuries, thrombotic disorders, and maladies associated with pregnancy.

The pelvic veins and plexuses have great variability in unions, pathways, and size. Although the pelvic vein anatomy was first described in the 1800s by Brechet and Bock, it was Batson (for whom the vertebral venous system is named) who recognized the correlation between the spread of disease and the venous system.¹ Richet described pelvic varicosities, a currently well-publicized women’s health issue, as early as 1857.² Bajka et al³ extended our understanding of not just the complexity of the venous system but also cleverly visualized the entire human anatomy in their Virtual Human Female (VHF) wherein segmentation software created detailed digital images from cryosections of human cadavers. Adjustments in terminology were made in 2004 so that the daily clinical terminology became standardized.⁴ The 21st World Congress of the International Union of Angiology with the participation of International Union of Phlebology, the International Federation of Anatomical Association, and the Federative International Committee for Anatomical Nomenclature met in Rome to refine vein terminology, especially that of the pelvic veins. The description of the pelvic veins has undergone much refining through the years. An understanding of the origin of the pelvic veins and tributaries is necessary for the pathophysiology of disease entities in upcoming sections of this chapter.

Superficial

The pelvic veins are formed from the superficial and deep veins of the lower extremities (Tables 29-1 and 29-2). The superficial veins originate as the superficial dorsal digital veins and join together to form the common digital veins, which then collectively make up the dorsal venous arch of the foot. The medial and lateral marginal veins are composed of many deep branches as well as the dorsal venous network and arch of the foot and plantar venous subcutaneous network. The great saphenous vein (not the long saphenous vein, which could be misconstrued as the lesser saphenous vein if abbreviated LSV) starts at the medial marginal vein.⁴ The small saphenous vein starts at the lateral marginal vein.

Communicating Veins Eponyms

Communicating veins direct blood flow from the superficial to deep veins. The following are eponyms (historical significance only) for such communicating or perforating veins, although they are located from the foot to the gluteus: superior to the knee (Dodd’s perforator), inferior to the knee (Boyd’s perforators), adductor canal (Hunterian perforator), medial calf (Cockett’s perforators), and the inframalleolar perforators.⁵ Additional eponyms are included here as they have been publicized in worldwide journals. Giacomini’s vein, more correctly called the posterior thigh circumflex vein, is the union along the medial thigh of the great and small saphenous veins. The posterior arch vein, the posterior accessory vein of great saphenous vein, lies on the medial aspect of the leg. Santorini’s plexus, or the pudendal plexus, is familiar to urologists.⁴

Deep Veins

These deep veins, which accompany the arteries with whom they share a name, contain more valves than superficial veins and are more numerous in the lower limbs of the body. They arise from the plexuses that form the deep plantar digital veins of the foot, which join together to form the deep metatarsal veins just shortly after a contribution (intercapitular veins) to the dorsal digital veins. Veins of the dorsum of the foot unite with metatarsal veins to form the deep plantar venous arch from which the medial and lateral plantar veins arise. The great and small saphenous veins from the superficial set join the medial and lateral plantar veins behind the medial malleolus and unite to form the posterior tibial vein. The anterior tibial vein courses over the interosseus membrane of the lower leg to join the posterior tibial vein at the lower border of the popliteus muscle to originate the popliteal vein. Thin-walled sinusoids or venous lakes are embedded in the soleus muscle bellies.⁵ Muscle contraction then empties these sinusoids into the posterior tibial and peroneal (fibular) veins. Accompanying each tibial artery are two to three venae comitantes. The next landmark is the aperture of adductor magnus after which popliteal vein becomes the femoral vein. Early in its course, the femoral vein is positioned laterally and then moves behind the femoral artery. The deep femoral vein (the union of the medial and lateral femoral circumflex femoral veins with contribution from the inferior gluteal vein) joins the femoral vein at the inguinal ligament. At the fossa ovalis, the femoral vein gets a contribution from the great saphenous vein via the saphenofemoral junction (SFJ) (Figure 29-1).⁴ The SFJ is a series of four valves that function to prevent backflow into the great saphenous vein when a terminal valve (outlet into the great saphenous vein) is closed. The femoral vein is now positioned medial to the artery (thus the popular NAVEL mnemonic for the femoral central venous line) and becomes the external iliac vein. The external iliac vein unites with the internal iliac vein (hypogastric vein that drains the pelvis) to become the common iliac vein at the sacroiliac joint within the pelvis. At lumbar vertebra five (L5), the left and right common iliac veins form the inferior vena cava (IVC).

The left external iliac vein is medial to the artery, and the right external iliac vein begins medially and then finds a position behind the same-named artery.⁶ The inferior epigastric vein, deep iliac circumflex vein, and pubic veins contribute to the external iliac vein after it passes the inguinal ligament. The left and right inferior epigastric veins communicate via superficial suprapubic veins.

The many tributaries of the internal iliac vein cover the pelvic sidewall lateral to the internal iliac artery.⁷ The superior gluteal veins are usually the largest tributary to the internal iliac veins except during pregnancy, when the uterine veins assume this role.⁸ The internal iliac vein’s entry into the common iliac vein may be varied. In the fresh cadaveric studies, Baque et al⁹ noted that the internal iliac vein was single in 50% of cases, double in 30% of cases, and plexiform in 20% of cases. Bleich et al⁷ examined the anatomy of the internal iliac artery and its posterior division branches and noted the usual location of the internal iliac vein important for clamp placement during surgery. The internal iliac vein is positioned dorsal and lateral to the artery 93.3% of the time on the left and 70.6% of the time on the right; otherwise, the vein is medial and inferior to the same named artery (Figure 29-2).⁷ In terms of valves, however, the right internal iliac vein has the greater percentage (28% compared with 14% on the left).¹⁰ The right common iliac vein begins its vertical ascent posterior to then lateral to the right common iliac artery, and the left common iliac vein (which drains the middle sacral veins) is longer and starts off medial and then moves posterior to its same-named artery.⁶ A significant anatomic peculiarity exists wherein the left common iliac vein is positioned posterior to the right common iliac artery and anterior to L5, creating potential for venous outflow obstruction.¹¹

Advanced imaging techniques have allowed us to clarify and classify anatomical abnormalities. Congenital anomalies of the IVC, a risk factor for deep venous thrombi (DVT) in young adults, and pelvic venous variation possibly portend difficulty during surgery.¹² Morita et al¹² used computed tomography (CT) examination to radiologically classify eight types of pelvic venous variations of congenital IVC abnormalities and noted a higher occurrence in men (Figure 29-3).¹² Type 1 shows normal iliac connection. The five variants of type 2 show the absence or presence of an interiliac connection (vein that drains the common iliac, internal iliac, or external iliac vein into the contralateral side) between double IVCs.¹² Type 3 shows the IVC’s connection to a renal vein. Type 4 reflects the absence of the infrarenal IVC, which may develop into parametrial phlebectasia (large tortuous dilated varicosities) and collateralization of surrounding veins that eventually drain into the azygous system.²

Pelvis

The pelvis is primarily drained by the internal iliac veins but also by the superior rectal, medial sacral, and ovarian veins.⁸ The internal iliac (hypogastric) vein receives both visceral and parietal drainage from within the pelvis: the superior gluteal veins (at the greater sciatic foramen), inferior gluteal veins (which unite with medial femoral circumflex veins and enter at the lesser sciatic foramen), internal pudendal veins (which primarily terminate as the pudendal plexus), obturator veins (via the obturator foramen along the lateral wall of the pelvis), lateral sacral veins (anterior surface of the sacrum), middle rectal vein (contributions from the bladder, prostate, and seminal vesicle), vesical, uterine, and vaginal veins.¹³ As seen from the aforementioned list, an incompetence of an internal iliac vein tributary may affect lower extremity varicosity.¹⁰ Pubic veins form as the union of the obturator veins and external iliac veins. The perineal vein, a nebulous term, is better separated as the deep perineal veins and superficial perineal veins. The deep perineal veins are actually the part of the pudendal plexus atop the internal face of the perineum.⁴ The superficial perineal veins comprise the subcutaneous veins of the urogenital and perianal region. The rectal veins are housed in very loose connective tissue incapable of increased pressures and have two components: an internal plexus, which is a circular arrangement above the anal orifice (termed the hemorrhoidal plexus), and an external plexus outside of the muscle (termed the rectal plexus).⁴ This external plexus (or rectal plexus) communicates with the portal system via the superior hemorrhoidal vein (origins of the inferior mesenteric vein leading to the portal vein).⁵ Behind the symphysis pubis is the pudendal plexus, which is the union of the bladder veins via the vesical plexus at the lower part of the bladder and prostate veins via the prostatic plexus. Accepted synonyms for this pudendal plexus are the vesico-prostatic plexus and the retropubic plexus (of Santorini).⁴ The vesical venous plexus also drains an unpaired vein, the deep dorsal vein of the clitoris and of the penis. The other part of the dorsal veins of the penis, the superficial vein, contributes to the great saphenous vein. The uterine and ovarian vein or plexuses, which are quite enlarged during pregnancy, are housed in the broad ligament along the lateral and superior aspects of the uterus. The left ovarian plexus first drains into the left ovarian vein and then the left renal vein before finally draining into the IVC. The right ovarian vein, however, drains directly into the IVC just below the renal vein on that side.¹⁴ The uterine and vaginal plexuses drain into the uterine veins and ultimately into the internal iliac vein.¹⁵ The male counterparts, the spermatic veins, open directly into the IVC. Venous hypertension in the pelvic plexus is transferred to the abdomen and inguinal superficial veins lower limb via veins of the broad ligament.⁴ Vaginal plexuses are at the lateral aspects of the vagina. The pampiniform plexus surrounds the ovarian or testicular artery and cools that blood. This is the main component of the spermatic cord, which is the combination of the spermatic veins off the back of the testis with the epididymal tributaries. The rectal, vesical, prostatic, uterine, and vaginal plexuses are collectively known as pelvic venous plexuses and drain mainly into the internal iliac vein; they have clinical significance, especially in women.⁸

Presacral Venous Plexus

Of surgical significance is the presacral venous plexus (PSVP), which covers the anterior sacral body and brings together the lateral and median sacral veins.⁹ Nearby elements include the presacral fascia, piriformis, coccygeal muscle fascia, and sacrospinous ligament as well as the hypogastric plexus and the pelvic sympathetic trunk. The location of the PSVP makes it especially vulnerable during surgery unless staples are placed at the avascular corners (as in a Well’s operation). Failure to do so could cause massive bleeding owing to the lack of valves in the pelvic venous system.⁹

Craniospinal Venous System

Of special interest is the craniospinal venous system (CSVS) of valveless veins comprised of the intracranial veins and the vertebral venous system.¹⁶ The intracranial veins include cortical veins, dural sinuses, cavernous sinuses, and ophthalmic veins. The vertebral venous system or Batson’s veins are subdivided into the internal vertebral venous plexus (in the spinal canal outside the dura), external vertebral plexus (external to the vertebral column), and basivertebral veins (within the vertebra).¹ The CSVS has close communication with the sacral and pelvic veins and the prostatic venous complex. Because these are valveless, this provides bidirectional flow and spread of tumors, infections, and emboli. There is a direct relationship between the prostatic veins and the valveless venous system in the metastasis of prostate cancer to the spine.

Venous circulation operates in a closed system and is impacted by arterial flow and inspiratory negative pressure. When becoming erect from a reclined position, an individual’s volume of blood in the lower extremities increases by 0.5 L, thereby exerting hydrostatic pressure in the order of about 100 to 120 mm Hg.⁵ This pressure must be dissipated to avoid pooling in the lower extremities, which serves to decrease venous return to the heart. Unidirectional flow toward the heart depends on the capacity of the venous system within the lower extremities, which in turn is affected by smooth muscle contractility, valves, and perforators. Thin-walled soleal sinusoids rely on the Venturi effect to draw these walls closer together and siphon blood toward the heart.⁵ Numerous venous valves help direct flow proximally; however, they decrease cranially such that there are none in the IVC. Increasing vein lumen diameter and insufficiency in this area, either because of a paucity of or damage to valves, leads to reflux, stasis, and varicosities and provides the physiological basis of pelvic vein abnormalities discussed in upcoming sections.

The retroperitoneal venous system is derived from three parallel sets of veins (subcardinal, postcardinal, and supracardinal veins) during weeks 6 through 10 of gestation.¹³ Failure of the postcardinal and supracardinal veins to develop results in IVC or renal vein agenesis.¹⁷ The right and left postcardinal veins drain the lower limb buds, pelvis, body wall, and derivatives of the mesonephroi.¹³ Adult derivatives of this embryologic vein include the root of the azygous vein, iliac bifurcation, and the iliac veins as the subcardinal vein later assumes its drainage.¹³ Infrarenal IVC anomaly occurs in less than 2% of the population, and an even smaller number of individuals have IVC agenesis or absence.¹⁷ The subcardinal veins cross anterior to the aorta and later make up part of the adult left renal vein. The left common iliac vein is derived from an anastomosis between the iliac veins. The left renal vein develops from the embryonic circumaortic venous ring and normally forms ventral to the aorta; otherwise, retroaortic renal veins develop in 1.7% to 3.4% of the population.¹⁸ Left renal vein anomalies are either classified as type 1 or 2. Type 1 is formed from the persistence of the left subsupracardinal anastomosis, the intersupracardinal anastomosis, and the dorsal left renal vein. Type 2, which is seen at the level of L4–L5, is the persistence of the left subsupracardinal anastomosis and left supracardinal vein.¹⁸

Arteriovenous Malformations

Congenital arteriovenous malformations (AVMs) are rare with locations in the extremities, lung, head, and neck; those in the pelvis are especially uncommon, with only 71 known cases between 1867 and 2001.¹⁹ They are more commonly seen in women than men.²⁰ Whether pelvic AVMs occur as congenital anomalies or are acquired through trauma, childbirth, or pelvic surgery is debatable.

AVMs have been termed angiodysplasia with arteriovenous shunts.²¹ Arterial, capillary, venous, and lymphatic vessels maldevelop and form tangled networks that are especially problematic during treatment. Dysplastic arteries and veins but not capillaries are seen in the histologically.²²

AVMs reroute a high volume of blood to low-resistance veins, which provide the basis of the symptoms such as dyspareunia, pelvic or flank pain, and leg swelling. Signs that may be elicited include palpation or auscultation of a pulsatile mass or thrill and pain in the abdomen, high-output cardiac failure, distal leg ischemia, hematuria, impotence, and (very rarely) ureteral obstruction.²² Some AVMs may be asymptomatic but still compress adjacent organs. Early recognition is paramount because these pelvic AVMs carry a risk of significant hemorrhage.

Use of imaging modalities has increased awareness of these AVMs. Conventional CT angiography is the most common modality for diagnosing the size and source of pelvic AVMs; however, this diagnostic mode carries the risk of radiation and nephrotoxicity. A typical pelvic CT dose is 9.9 mSv, about half of that received daily by those employed by the nuclear industry. Nephrotoxicity is an issue in those with diabetes, preexisting renal insufficiency, and dehydration. The procedure is fairly well tolerated by the patient because there is no placement of an arterial catheter. Radionuclide angiography in pelvic AVMs shows an abnormal network of vessels with engorged feeding arteries, collateralization, early venous drainage, and stasis of contrast medium within this area.²⁰ Success of color Doppler ultrasonography (CDUS) is highly dependent on patient habitus and thus can miss many anomalies.

Magnetic resonance angiography (MRA) offers the benefit of cross-sectional imaging and multiplanar imaging capabilities without the risk of nephrotoxicity or radiation.²⁰ This imaging tool cannot be used by those with metallic clips or implants or embolization materials. Diaz Candamio et al²⁰ examined its utility in pelvic AVMs because MRA has been proven a diagnostic tool in brain, extremity, and pulmonary AVMs. This method provided an excellent study because of less flow and motion artifact and saturation effects, subsequent findings may suggest a type 3 AVM bypassing the need for surgery.

Olcott et al²¹ classified three types of AVMs for treatment purposes. Type 1 is a small, easily removed area. Type 2 involves embolization before surgical removal. Type 3 AVMs are addressed with repeated embolization only. Treatment is often a challenge. Initial therapy should be percutaneous embolization (coils, balloons, Gelfoam, autologous muscle, polyvinyl acetate emulsion, isobutyl cyanoacrylate), which reduces bleeding and increases successful intervention.²³ Castaneda et al²³ investigated the use of ethylene vinyl alcohol copolymer, an investigational radiopaque nonadhesive liquid casting agent successful in brain AVMs and in pelvic AVMS. They found this agent to be easily conformable and delivered, although rapid injection caused vasospasm. A unique feature of this compound allows that particular vascular segment to be surgically resected at a later date, an unavailable option with more conventional adhesives.²³ A complication of embolotherapy is intractable hematuria because of bladder ischemia.²²

When the AVM is too large or accompanied by innumerable feeding vessels, surgical resection remains the sole option. Surgery, which involves devascularization or excision of a mass of arterial feeders, is associated with increased injury to adjacent tissues, morbidity, and high AVM recurrence rate.²³ McCready et al²² described a case wherein deep hypothermic circulatory arrest complemented surgical resection of pelvic AVM because this has been previously successful in mandible and posttraumatic pelvic AVM. The risk of hemorrhage associated with extensive dissection within the pelvis is attenuated by hypothermic circulatory arrest. The bloodless field allows surgeons to identify neighboring structures for safe and complete surgical resection.

Although pelvic AVMs are certainly more prevalent and described more often in women, a British case study reported on a recurrent scrotal AVM secondary to pelvic fracture 4 years prior. Their literature review reports on only seven described cases, only one congenital, which overwhelmingly involved the scrotal skin.²⁴ These patients present with scrotal swelling, ulceration, and bleeding. Although these authors recognized that CDUS may diagnose this entity, they found CT and magnetic resonance imaging (MRI) more useful in directing their chosen intervention. Angioembolization carries the risk of testicular and lower extremity ischemia; therefore, a more detailed vascular assessment in the form of CT or MRI is necessary.

Inferior Vena Cava Anomalies

The IVC is composed of hepatic, suprarenal, renal, and infrarenal segments. IVC abnormalities are found in 0.3% to 0.5% of healthy persons and 0.6% to 2.5% of those with other cardiovascular defects.²⁵ A Canadian study concluded that major venous and renal anomalies were present in 5.65% of the population.²⁶ Congenital anomalies of the IVC, a risk factor for DVT in young adults, and pelvic venous variation possibly portend difficulty during surgery.¹² Congenital anomalies of the IVC present as a palpable abdominal mass.

Morita et al¹² used CT examination to radiologically classify eight types of pelvic venous variations of congenital IVC abnormalities and noted a higher occurrence in men (Figure 29-4). Type 1 shows normal iliac connection. The five variants of type 2 show the absence or presence of an interiliac connection (i.e., vein that drains the common iliac, internal iliac, or external iliac vein into the contralateral side) between double IVCs.¹² Type 3 shows the IVC’s connection to a renal vein. Type 4 reflects absence of the infrarenal IVC, which may develop into parametrial phlebectasia (large, tortuous, dilated varicosities) and collateralization of surrounding veins. Aljabri et al²⁶ showed these venous anomalies in their random review of CT scans: circumaortic left renal vein in 1.62% (nonregression of the dorsal segment of an anastomosis), left-sided IVC without situs inversus in 0.17% (persistence of the left subcardinal and supracardinal veins with abnormal regression of the right-sided system), and duplicate IVC in 0.39% (persistence of bilateral supracardinal and subcardinal veins) of 1788 abdominopelvic CTs they reviewed.

Type 4 has an incidence 0.0005% to 1% in the general population and is found in 5% of those with a DVT.²⁵ This absence of the infrarenal IVC is one of three entities that are classified as agenesis of the IVC. The other two are absence of the suprarenal IVC (failure to form the right subcardinal vein) and absence of the entire IVC (all the paired venous systems failed to form) (see Figure 29-4).²⁵ These patients likely require lifelong anticoagulation along with elastic stocking support and leg elevation.²⁵ CT and MRI are better imaging modalities to expose collateral flow and prominent azygous and hemiazygous systems. Some authors promote the use of MRI such that it visualizes associated congenital abnormalities and offers a superior vascular picture.²⁵

An infrarenal aortic aneurysm may erode and cause a fistula between the IVC and aorta dependent origin, size, location (most often located between the infrarenal aorta and IVC), patient’s age, and comorbid cardiopulmonary or renal disease.²⁷ Only 0.22% to 6.04% of abdominal aortic aneurysms ever develop a fistula.²⁷ Another cause for this type of fistula is iatrogenic injury. The likeliest physical examination finding is an abdominal bruit, pulsatile mass, or dyspnea in these cases.²⁷

Miscellaneous

Ureteral obstruction (medial deviation and dilatation of the right ureter) is most often caused by an IVC abnormality.²⁸ Ovarian vein syndrome can pinch the ureter at the point where the right ovarian vein crosses the ureter at L3; otherwise, enlarged abnormal gonadal, spermatic, or lumbar veins cause compression.²⁸ Examiners in Hong Kong prospectively reviewed patients before total extraperitoneal inguinal hernioplasty and noted the iliopubic artery and vein present all of the time, with an aberrant obturator vein present 27% of the time.²⁹

Most epidemiologic studies of vascular injuries involve those sustained by the military and civilians in war-torn areas.³⁰ In addition to the iliolumbar and pelvic vein abnormalities attributable to congenital causes or disease, operative injury also occurs. Linton and White³¹ reported the first case of arteriovenous fistula status after laminectomy in 1945. In fact, these iatrogenic injuries are responsible for the increasing incidence of vascular injury, which is presently at 0.9% to 2.3% per 100,000 population.³⁰ The incidence has a variable range of less than 4% to 15.7%.³² The left common iliac vessels course through the L4–L5 and L5–S1 region, which leaves the left common iliac vein the most vulnerable during anterior spinal injury.³² These life-threatening injuries require rapid diagnosis and immediate attention for appropriate repair.

Summary of Reviews

Oderich et al³⁰ performed a retrospective review of the types, management, and outcome of iatrogenic operative injuries of the abdominal and pelvic veins on a small number of patients at the Mayo Clinic between 1985 and 2002. Of the 713 patients with vascular injury, 40 sustained abdominal and pelvic operative injury, most commonly during elective general surgery and tumor debulking. The partial laceration of the iliac veins was most often involved caused by sharp or blunt dissection; 95% had immediate bleeding.³⁰ Injury to the IVC had a varied origin: resection of a retroperitoneal mass, right hepatectomy, laparoscopic cholecystectomy, and radical cystectomy (which necessitated a medial rotation of the right colon with extended Kocher maneuver in some cases).³⁰

Fantini et al³² specifically looked at anterior lumbar spinal injury that occurred during treatment of spinal deformity, bony or discogenic infection, trauma, tumor, and degenerative disease from 2001 to 2005. Anterior lumbar spinal surgery is preferred for a thorough discectomy and release; implantation, removal, and success of devices; and debridement and excision of necrotic tissue. With the advent of total disk arthroplasty (TDA) and prosthetic disc nucleus replacement, anterior spinal access is increasingly sought.³² Of the 338 patients in this retrospective review, there were 10 cases of vascular injury to the iliac vessel (predominance of left common iliac vein injury), vena cava, and aorta for an incidence of 2.9%.³² Injury occurred during initial spinal exposure or maintenance of exposure for corpectomy, distraction, osteotomy, and spinal reconstruction when these at-risk vessels were mobilized within dense inflammatory tissue.³²

Predisposing Factors

Possible predisposing factors for iatrogenic pelvic vein injury are oncologic operations, distorted anatomy, previous operation, tumor recurrence, previous radiation therapy, and chronic inflammatory changes.³⁰ Risk factors specifically related to anterior spinal injury include current or previous osteomyelitis or discogenic infection, previous anterior spinal surgery, spondylolisthesis, osteophyte formation, transitional lumbosacral vertebra (the last mobile segment is dorsal to the iliac vein), and anterior migration of interbody device point.

Management

Management of patients with iatrogenic vein injuries may be by traditional open surgery or endovascular techniques. The gold standard is open surgical repair with or without the vascular team, with a mortality rate of 10% to 66%.³¹ Venous repair is strongly advocated; however, ligation is acceptable in cases of hemodynamic instability, local injury, and extensive anesthesia requirements. Adverse effects of venous ligation include DVT, compartment syndrome, postoperative edema, and thrombophlebitis. Operative repair involves digital or sponge compression, a method used when attempting to rapidly address the significant blood loss.³⁰ Other interventions included aortic cross clamping (although this can result in additional injury), Pringle maneuver, intravenous (IV) fluids, extension of the original incision, and open packing with scheduled repeat operation for hemostasis. The Trendelenburg position and proximal venotomy suction are helpful adjuncts.³² The injured vessel is carefully addressed with mobilization (release traction every 20 minutes) and debridement so as not to adhere to healthy neighboring tissue because there is a limit to their deformation.³² Liberal application of clot (Gelfoam, Surgicel) and hemostatic (Vitagel, FloSeal, and Tisseal) agents with or without vascular clips was used.³² Lateral venorrhaphy with a single 5-0 Prolene suture in a figure-of-eight fashion was adequate.³² Those undergoing technically difficult vascular reconstructions have better patency rates.³¹ The internal iliac vein is most often partially lacerated during pelvic oncologic procedures, requiring approximately 20 units of packed red blood cells for the 7-L blood loss.³⁰ Lateral venorrhaphy, patch angioplasty, and end-to-end anastomosis most often addressed this issue (Figure 29-5).

Postvenorrhaphy DVT surveillance with MRI of pelvic veins and IVC with or without IVC filter is an important adjunct because this procedure is considered thrombotic. The question of whether to use autologous saphenous vein or externally supported expandable polytetrafluoroethylene graft (ePTFE) is valid. Although the graft is certainly more timely and helpful for complex injury with segmental loss, it does carry a risk of infection and poor long-term patency rates.³⁰

Sequelae

The vascular complication is expressed as hemorrhage or thrombosis. The ensuing complications may be as early and obvious as organ system failure, disseminated intravascular coagulation (DIC), acute respiratory distress syndrome (ARDS), or later complications such as chronic venous insufficiency. A patient may remain hospitalized for 41 days, and perioperative mortality is as high as 18% and caused by venous rather than arterial injury.³⁰ Unfortunate sequelae of these iatrogenic operative injuries of abdominopelvic veins include prolonged intensive care unit stay, ventilator-dependent respiratory failure, sepsis, dilutional coagulopathy, ARDS, pneumonia, cardiac arrest, myocardial infarction, dialysis-dependent renal failure, and DIC. Symptoms of iatrogenic vein injury may be as benign as “unexplained anemia” (tamponaded bleeding) or malignant as exsanguinating hemorrhage. Atrioventricular fistula is the most common delayed presentation of unrecognized venous injury, but classical symptoms are only present half the time.³¹ The patient may complain of dyspnea and palpitations. The physical examination may yield an abdominal bruit on auscultation. Pelvic hypertension is manifested as lower extremity swelling, hematuria, renal insufficiency, scrotal edema, or rectal bleeding. This presentation is quite amenable to endograft repair, with the first reported by Zajko et al.³¹ The cause of death is multisystem organ failure with protracted hospital course, exsanguinating hemorrhage, and massive pulmonary embolus (PE) associated with iliofemoral venous thrombosis.³⁰ Mortality is directly associated with bleeding and its physiologic consequences.³⁰ A 3-year follow-up showed that late deaths were not attributable to the previous venous injury.³⁰

Conclusion

Advances in surgical technique and technologies allow for very precise treatments in the lumbar and abdominopelvic region. Expected increases in longevity demand that these modalities become more available. These are, however, accompanied by negative sequelae that impact the venous system in hemorrhagic or thrombotic fashion. Although these two retrospective reviews demonstrate that pelvic venous injury is uncommon, its effects may be catastrophic. Serious complications are minimized or avoided with recognition and repair of low-pressure and high-flow venous systems encased in inflammatory tissue. Vascular surgeons have a challenge with the anatomic site, amount of blood loss, and regional edema.

Chronic pelvic pain (CPP), an emerging medical entity, affects 10% to 40% of the obstetric/gynecologic outpatient population.³³ According to some authors, the prevalence of CPP at 38 of 1000 individuals is comparable to those of asthma, low back pain, and migraine.³⁴ The terms CPP, pelvic congestion syndrome (PCS), pelvic venous congestion, pelvic varices, pelvic varicocele, pelvic varicosities, pelvic venous incompetence, and ovarian vein syndrome are synonymous, although some terminology is more specific and anatomically sound. CPP is a dull and heavy pressure of at least 6 months duration that is noncyclical in occurrence and leads to functional disability. There are various causes of this disorder, including urogenital, gastrointestinal, musculoskeletal, neurologic, and psychological causes. The location of pain may be in the pelvis, anterior abdominal wall inferior to the umbilicus, lumbosacral area, and buttocks.³⁴ The pain may be referred, as in the case of visceral pelvic pain often sensed about the umbilicus, or well-localized somatic sacroiliac pain corresponding to posterior buttock discomfort.³⁵ Other authors propose that this pain must be nonresponsive to narcotics and exclude menstrual or sexual symptoms in the definition of CPP.³⁶ CPP appears to affect women to a greater extent than men, which is thought to be attributable to anatomical differences between men and women.

The cause of CPP is unclear, but many associations have been implicated. The following are a sampling of causes and associations: PCS, endometriosis, adenomyosis, pelvic adhesions, trauma, atypical menstrual pain, pelvic inflammatory disease, urological problems, irritable bowel syndrome (IBS) or spastic colon syndrome, portal hypertension, trapped or residual ovaries, congenital or acquired anomalies, and neuropsychosomatic disorders.^33,37,38 Causes may also be subdivided into visceral or somatic sources. Potential visceral sources include reproductive, genitourinary, and gastrointestinal tracts, and potential somatic sources include the pelvic bones, ligaments, muscles, and fascia.³⁵ The symptomatology most often experienced is that of urinary or gastrointestinal complaints, and it is these women that get a more thorough workup.³⁴ Few disease entities, such as endometriosis, interstitial cystitis, and IBS, have evidence for a causal relationship with CPP. Up to 80% of women who present with complaints of pelvic pain are found to have symptoms consistent with either IBS or interstitial cystitis.³⁵ Risk factors include hereditary factors, hormonal influence, pelvic surgery, retroverted uterus, varicose veins, and multiple pregnancies.³⁸ Careful questioning or obstetric history may reveal lumbar lordosis, delivery of a large infant, muscle weakness and poor physical conditioning, cesarean delivery, or a difficult delivery with or without the assistance or tools or stirrups.³⁵ These factors cause significant trauma to the musculoskeletal system.

Varicose veins are particularly implicated because of the congestion brought about by increased blood flow and vein dilatation. Arnoldi’s³⁹ 1958 definition of varices is any dilated vein; the normal venous diameter of the utero-ovarian veins should be less than 5 mm. Pavkov et al³⁹ used von Hagen’s plastination technique to qualify and quantify the caliber of such veins and their anastomosis (utero-ovarian arcade) in postmenopausal deceased women. Their research shows there is no preferential draining of blood in these two vessels and highlights their role in CPP. This tubo-ovarian arcade is a common site for venous pooling. Treatment of CPP, as will addressed in upcoming sections, is therefore best addressed with targeted minimally invasive techniques rather than radical aggressive therapies because these sites can be directly targeted. Varicosities may be primary or secondary. Primary varicosities are the result of heredity, occupation, or avocation (e.g., a job or sport may predispose a person to standing for prolonged times). Secondary varicosities are of more concern because they are caused by DVT, catheter-associated DVTs, pregnancy, or trauma.

PCS is responsible for approximately 50% of CPP and is most often diagnosed in multiparas ages 30 to 40 years.^33,40 In the mid-1930s, Cotte⁴¹ made the association with CPP and reflux within ovarian and uterine veins causing pooling of blood in tortuous veins and subsequent pressure and heaviness. The role of vascular insufficiency in PCS was also described in 1949 by Taylor.⁴² He coupled abnormal ovarian morphology with utero-ovarian venous congestion accompanied by ascitic fluid.^39,43 Sixty percent of women with ovarian varices develop PCS,³⁸ and 56% of those with polycystic ovaries have PCS.⁴⁴ Modern terminology has given the moniker pelvic migraine to PCS.⁴⁵ PCS involves dilatation of the broad ligament, especially with pregnancy-related and premenstrual progesterone. Some authors exclude broad ligament varices in the definition of pelvic pain.⁴⁶ This incompetence within the ovarian plexus veins does not accommodate for increased blood flow and promotes PCA, thereby making activities such as coitus, walking, bending, heavy lifting, and prolonged sitting insufferable.³³ Note that vulvar varices are not present in these descriptions of PCS⁴³ because they are in descriptions of PCS by Bell et al.⁴⁷ This latter study examined vulvar varices samples that were initially misinterpreted as vulvar cyst, Bartholin gland, and Bartholin gland cyst. Vulvar varices may be associated with leg varices or labial, clitoral, or vaginal venous malformations or Klippel-Trenaunay-Weber syndrome and Parkes-Webber syndrome.⁴⁷ Pelvic varices may be associated with vulvar, perineal, and lower extremity varices.³³ A Turkish study by Gultasli et al³³ examined the incidence of lower extremity venous insufficiency in women with pelvic varices. The study found that 70% of women with pelvic varices also had lower limb venous insufficiency as evidenced by increased inverse flow per lower extremity Doppler examination.

Patients with varices often complain of a shifting lower quadrant abdominal pain, dyspareunia, lower extremity varices, and emotional disturbances.⁴² The exact definition and pathophysiology causing these complaints are difficult to discern and likely represent a spectrum of disease rather than a discrete entity. One author attempted to classify PCS into three main groupings: symptomatic pelvic congestion without venous reflux, nonpainful vulvar varices with ovarian vein reflux, and painful pelvic congestion with varices but without reflux.⁴³

Pregnancy not only injects hormonal influence into the pathophysiology of CPP but may also predispose vessels to mechanically kink, causing uterine malpositioning.^40,41 Ovarian steroid hormones, in mice at least, seem to specially affect uterine and ovarian vessels.⁴³ PCS is seen predominantly in premenopausal women who presume ovarian hormonal influence.⁴⁸ Women older than 35 years of age, toward the end of childbearing years, have lower odds of developing CPP.³⁸ A recent article reported complete regression of symptoms after menopause because a decrease in estrogen leads to less nitric oxide release, decreased smooth muscle relaxation, and vascular responsiveness.¹⁵ Gravity exacerbates the pooled blood and thus dull pressure and heaviness, which may reflect a systemic vasomotor instability.

Related Diagnoses

Ovarian Vein Syndrome. Ovarian vein syndrome comes about when an ovarian vein becomes so dilated that it compresses a ureter and the patient complains of renal colic.⁴² Although anatomy dictates that ovarian vein reflux would be more demonstrable on the left than the right because of an absence of valves more so on the left than the right, symptoms may be experienced bilaterally. About 10% of women have ovarian vein incompetence, and within this population, 60% go on to develop PCS. Secondary ovarian vein syndrome may be caused by portal hypertension or acquired IVC syndrome.⁴¹ Zhou et al¹⁷ describe a rare occurrence of ICV agenesis that results in an enlarged left ovarian vein to drain the pelvic collaterals.

Nutcracker Syndrome. Grant first gave this whimsical title to the left renal vein’s entrapment between the aorta and superior mesenteric artery (SMA), similar to a nut in the vise-like grip of a nutcracker (Figure 29-6).⁴⁹ De Schepper⁵⁰ made this association with hematuria. Nutcracker phenomenon may be anterior or posterior. In the first instance, the left renal vein lies between aorta and SMA, and in the latter, the left renal vein lies between the aorta and vertebral column. The addition of retrograde hypertension of the left renal vein, flank pain, hematuria, varicocele, and pelvic varices or PCS make up the nutcracker syndrome. A congenital cause of nutcracker syndrome is an abnormal steep caudal descent configuration of the SMA off the aorta, creating only a slit between the aorta and the SMA.⁴⁹ A rare congenital cause for PCS is double left retroaortic renal veins.¹⁸ The renal venous hypertension leads to the development of collateral flow and subsequent hematuria with ovarian vein congestion and development of varices. This situation is exacerbated by valvular incompetence, which may be caused by prior thrombophlebitis or pregnancy-induced or ovarian vein dilatation. Pelvic varicosities develop with congestion of the left ovarian vein as its drainage into the left ovarian vein is hampered, thus causing subsequent PCS or CPP and low back pain. d’Archambeau et al⁴² proposed a grading system for nutcracker syndrome. A different hypothesis is offered forth by Scholbach⁵⁰ in his study of nutcracker phenomenon and causality in “midline congestion syndrome,” wherein volume overload and venous dilatation in downstream abdominopelvic organs causes pain. This author proposes that organs that lie on the midline of the body and provide collateral circulation to the left renal vein are at risk for CPP.

Endometriosis. Cheong and Stones’ review³⁴ states endometriosis is the primary laparoscopic finding (based on the skill of the surgeon) in women suspected of having CPP. Endometriosis affects younger infertile women. Endometriosis is hormonally responsive tissue that resides outside of the uterus. Unfortunately, the extent of disease cannot be correlated with the amount of CPP one experiences, a testament to the complexity of visceral sensation and innervation. Although it has been found in multiple sites in the body, appearing in a spectrum from peritoneal deposits to burn-out lesions, endometriosis favors the ovary, uterine ligament, pouch of Douglas, pelvic peritoneum, fallopian tube, and uterus over the bladder, cervix, and vagina. There is a genetic predisposition to developing endometriosis with a 10-fold increased prevalence in those with an affected first-degree relative. Four theories have been proposed to explain the phenomena of endometriosis. They include the (1) implantation theory, which proposes retrograde menstruation through the fallopian tubes into the peritoneum; (2) direct theory wherein endometrial tissue is hypothesized to be transplanted during a surgical procedure; (3) dissemination theory in which endometrial tissue is thought to be transported via lymphatic and vascular channels; and (4) coelomic metaplasia theory, which speculates conversion of tissue.³⁸ Despite an obvious causal relationship to CPP, endometriosis has viscera–visceral interactions such that CPP continues to exist long after the endometriosis has been surgically addressed.³⁵

Adenomyosis is a special case of endometriosis wherein the uterine basal layer glands become embedded in the myometrium, causing painful uterine enlargement and CPP. These are often mistaken for leiomyomas or fibroids. About 70% to 80% of cases affect women aged 40 to 50 years.³⁸ Two forms of this disorder exist. The first is diffuse, which is disseminated throughout the myometrium. The second form is focal, demonstrating nodular adenomyomas. Adenomyosis is thought to come about either by an ingrowth of endometrium into the myometrium or endometrial tissue is thought to be transported via lymphatic and vascular channels.

Adhesions. According to a meta-analysis, 36% of women with CPP had adhesions.³⁴ It is unclear whether adhesions actually cause CPP because very little symptomatic relief is gained from adhesion lysis. Adhesions that are dense and vascular and contain nerve fibers are more likely to cause pain and additionally promote traction upon the peritoneum. Observational studies suggest that adhesiolysis involving the bowel is more likely to demonstrate decreased pain.³⁵

Trigger Points. Pelvic trigger points are a cause that is not often diagnosed in traditional physicians’ practices and are most likely to be diagnosed by a women’s health physical therapist or osteopathic physician. A “typical pelvic pain posture” is exaggerated lumbar lordosis and thoracic kyphosis that predisposes one to trigger points and areas of hypertonicity.³⁵ Pelvic muscle trigger points that are discrete areas of hyperalgesia,³⁴ lead to chronic holding patterns and may be a minor cause of CPP. Chronic holding patterns are an abnormal maintenance of muscle, predisposing one to fatigue and pain in that immediate area. The subsequent development of a trigger point shows a viscero–somatic relationship. This trigger point feels much like a ball of butter that will dissipate upon proper digital manipulation or injection of lidocaine or bupivacaine.

Psychological Factors

CPP may ultimately manifest as physical and psychological suffering, negatively impacting the lives of many women, although black women were less often affected in one study.³⁸ Patients with CPP report a high incidence of psychological disturbances such as anxiety, depression, physical worries, and marital or sexual abuse problems.⁵¹ Depression and sleep disorders are also often found in women with CPP, and a history of abuse is prevalent in some patients with PCS.³⁴ Women with a sexual abuse history are more likely to have nonsomatic pelvic pain, thus suggesting the link between CPP and abuse is neuropsychiatric because of a lower threshold for pain.³⁵ Stress may more overtly impact individuals who are socially or psychiatrically predisposed to have low premorbid stress intolerance. Unfortunately, this means more women are affected because their dual roles of career women and caretaker often clash. On a cellular level, stress disturbs the smooth muscles of the pelvic organs, and these organs respond by releasing vasodilators substance P and neurokinins A and B.⁴⁴ Vasodilation promotes increased blood flow and furthers congestion into that area. Whether these psychological dispositions are causal or the result of CPP is unclear, but they are nevertheless part of the multidisciplinary approach to evaluating patients with CPP.

Clinical Presentation

Multigravid women may offer complaints of pelvic pain, dyspareunia, menorrhagia, dysmenorrhea, increased vaginal discharge, and increased urinary frequency.⁴⁵ Pain is usually unilateral. Upon further questioning, they may admit to a postcoital ache in addition to a chronic and dull, constant ache. The complaints of postcoital ache and ovarian point tenderness are 94% sensitive and 77% specific for PCS on later imaging.¹⁵ The quality of the pain should be clearly specified, including such information as onset; location; character; radiation; aggravating or relieving factors; and associated bowel, bladder, or menstrual symptoms. Inquiries should be made regarding a family history or varicose veins and vulvar varices and a personal history of anxiety and depression.⁴⁴ IBS and dyspareunia often accompany CPP, so the physician may open up a discussion regarding pelvic heaviness and CPP. There may be cervical motion tenderness or a blue-appearing cervix on bimanual or speculum examination.⁴ Additional physical examination findings include vulvar varicosities or varicosities that extend into the medial thigh, along the path of the great saphenous vein.⁴¹ The uterus may feel soft and boggy upon palpation, or one may observe trigger points over the ovaries.

Although these patients are often classified as “head cases” or placed in the always easy chronic pain category, these negative attitudes toward this disorder add to the pain experienced by an already suffering individual. Although there are more common and easier diagnoses to make, CPP should also be considered in patients who present with pelvic pain. Because the cause of CPP is poorly understood and the definitions have a wide range, it is no surprise that the median symptom duration is 15 months.³⁴ CPP diagnosis and sequelae lead to a fair economic impact: 15% of those with CPP miss about 14.8 hours of work per month or $14 billion in lost productivity.³⁸

Diagnosis of Chronic Pelvic Pain

Most of the investigations into CPP involve a supine patient position, wherein only the most severe pelvic congestion will be revealed. These imaging modalities may be noninvasive or noninvasive. It has been noted that $128 million is wasted each year on unnecessary medical, surgical, and psychiatric costs because multiple tests are ordered, often returning normal results.³⁸

Multiple schemas have been proposed to define pelvic congestion. Chung and Huh⁵¹ enumerated the following criteria as diagnostic of PCS: (1) ovarian vein diameter of 6 mm or larger, (2) contrast medium retention time longer than 20 seconds, (3) existence of congestion in the pelvic venous plexus or opacification of an internal iliac vein, and (4) filling of vulvovaginal and thigh varices. Subsequently, Park et al⁴⁰ enumerated the following diagnostic factors: (1) dilated left ovarian vein with reversed caudal flow, (2) presence of a varicocele, (3) dilated arcuate veins crossing the uterine myometrium, (4) polycystic changes of the ovary, and (5) variable duplex waveform during the Valsalva maneuver, which accentuates a varicocele similar to scrotal disorders in men. Kuligowska et al³⁸ offered the following variation of the criteria: (1) tortuous pelvic vein with a diameter larger than 4 mm, (2) slow blood flow (~3 cm/sec), and (3) dilated arcuate vein in the myometrium that communicates between bilateral pelvic varicose veins.³⁸ Finally, Ganeshan et al⁴¹ offer these criteria for PCS: (1) tortuous pelvic veins with a diameter of greater than 6 mm, (2) slow blood flow (~3 cm/sec) or reversed caudal flow, (3) dilated arcuate veins in the myometrium that communicate between bilateral pelvic varicose veins, and (4) sonographic appearance of polycystic changes of the ovaries. Because of the novelty of this disorder, no one schema has emerged as a gold standard; however, common elements among the experts include a dilated vein of at least 4 mm and the presence of a congested venous plexus. Both imaging studies and diagnostic laparoscopy are used to confirm diagnosis.

Ultrasonography. Ultrasonography is a safe and economical mean of diagnosing CPP. Pelvic varices are most commonly diagnosed with transvaginal ultrasonography (TVUS) and appear alongside the atretic follicles within the ovaries or uterus as dilated and tortuous anechoic vascular structures measuring more than 5 mm in diameter.³³ This is in contrast to the hyperestrinergic cystic changes described in another study.⁴⁰ Endometriomas cannot be differentiated from malignancies and other conditions that cause cystic changes in the ovary. The overall sensitivity and specificity for the diagnosis of endometrioma are 83% and 89%, respectively.³⁸ In select populations, the addition of transabdominal sonography to measure the involved veins may be beneficial. Campbell et al³⁷ prospectively studied the utility of transvaginal power ultrasonography in its detection for increased motion sensitivity by raising the Doppler gain level until the next highest signal above the noise baseline appeared in the background noise. This adjustment allows for greater sensitivity to slow flow but remains poorly correlated with venography diagnostic capability. TVUS is an excellent medium to assess endometrioma and adenomyosis but less so for adhesions and smaller endometrial implants. Adenomyosis appears as an echogenic mass with ill-defined borders. Vascularity is seen inside the mass and for treatment purposes is much be differentiated from fibroids.³⁸ TVUS has 8% to 96% sensitivity and specificity, respectively, for diagnosis of adenomyosis.

Magnetic Resonance Imaging. MRI is the preferred noninvasive nondiagnostic imaging technique owing to the absence of radiation exposure and excellent exposure of pelvic anatomy. It shows pelvic varices as having no signal on T1-weighted sequences and high signal on T2-weighted images.⁴¹ MRI offers better characterization in circumstances in which ultrasonography is equivocal. Endometriosis, especially extraperitoneal lesions, appears as a cystic mass on MRI with high signal intensity on T1-weighted images and low signal intensity on T2-weighted images because of the iron contained in the endometrioma (Figures 29-7, 29-8, 29-9, 29-10).³⁸ The sensitivity and specificity of MRI for endometrioma are 92% to 98%, respectively.³⁸ If adenomyosis is suspected, MRI is the diagnostic choice, with a sensitivity and specificity of 93% and 91%, respectively. Contrast-enhanced magnetic resonance venography (MRV) may supersede MRIs; the pelvic venous system can be imaged in a breath using the same pulse sequence as conventional MRI.

Computed Tomography. CT can distinguish between pelvic varices and lymphadenopathy or adnexal masses; the varices appear isodense after contrast enhancement.⁴¹ This contrast and the lack of dynamic component limit the utility and safety of this imaging modality in symptomatic childbearing women.

Venography. Pelvic color Doppler ultrasonography, although a good screening method, presents a fair amount of false-negative results owing to the slow blood flow in pelvic varicosities⁴²; therefore, the “gold standard” for diagnosis of pelvic varicosities remains transuterine venography. Comparisons between these two popular options yield contrasting results: whereas Hobbs et al concluded that ultrasonography often missed varices otherwise identified on venography, Park et al⁴⁰ found the two modalities fairly consistent.

Transuterine venography is an invasive procedure that requires sedation and irradiation, which reveals venous clearance issues; otherwise, three-dimensional T1-weighted gradient-echo sequences with gadolinium may demonstrate these pelvic varices, where blood flow is shown by high signal intensity.³⁸ Venography was first described by Tavernier and Lange in 1965 and later refined by Ahlberg and Endlundh for use in PCS.⁴¹ Transuterine venography can be performed as an outpatient procedure and very rarely results in uterine rupture.¹⁵ Selective ovarian venography shows more detail and but requires an overnight stay in the hospital. Ideally, this is performed on a tilting table, and a jugular rather than femoral venous approach is preferred if embolectomy is a possible treatment.⁴⁴ In addition to TVUS, selective ovarian venography should be included in the evaluation for CPP; otherwise, these women will continue to be unnecessarily referred to psychologists and psychotherapists.⁴⁶

Imaging Qualifications. Radiographically, pelvic congestion is expressed as dilated uterine and ovarian veins alongside reduced venous clearance of contrast medium, without the need to demonstrate ovarian vein reflux.⁴³

Diagnostic Laparoscopy. Thirty five percent of diagnostic laparoscopies in the United States are to discern the cause of CPP because this is the most sensitive way to diagnose the disease.³⁸ Laparoscopic findings of CPP in one study included endometriosis (31%), adhesions (23%), chronic pelvic inflammatory disease (7%), ovarian cyst (4%), myomas (<1%), pelvic varicosities (< 1%), and others (4%).⁴⁵ A separate study showed endometriosis (60.2%), normal findings (21.2%), and pelvic congestion (13%).⁵² Congestion within the pelvic vessels is decreased in the recumbent position during laparoscopy, possibly minimizing a clinically impressive disorder.

Management

The ideal management of PCS would include a multidisciplinary approach. Involvement of a general practitioner, gynecologist, pain specialist, psychologist, and physical therapist in the ongoing evaluation and care of the patient is vital in conjunction with patient education. Patients with CPP are managed similarly to those with other chronic disease processes: optimize the patient’s functional status, use analgesics or other mediators of pain prudently, and promote psychological support (psychotherapy or counseling) with a multidisciplinary approach. This team approach seems to benefit some women as reflected in decreased pain scores shown by one group of investigators.³⁴ Given the amazing advancements in imaging, it is worth mentioning radiologists as an integral part of this multidisciplinary team because their diagnostic acumen may be heightened when provided with the patient’s clinical history. Because CPP is a poorly understood process, the number of interested specialists and reimbursement for such services is limited, perhaps another impediment for these already suffering women. Possible therapies are medical, surgical, or endovascular; this care costs up to $39 billion per year.³⁸

Medical Treatment. The overall success of medical treatments is approximately 70%,⁴⁶ although some studies report lesser degrees of success. The first recorded treatment for pelvic varices was in 1883 when Lawson Tait used ergots as a short-lived panacea.¹⁵ Medical treatment is aimed at mitigating estrogen effects on relaxation of smooth muscle using nitric oxide inhibitors or dihydroergotamine. An alternate treatment uses medroxyprogesterone acetate (MPA; 30–100 mg/d) or gonadotropin-releasing hormone (GnRH) agonists with or without estrogen to suppress ovarian function. GnRH affects hypothalamic–pituitary gland production and significantly reduces estradiol levels, and it is as effective as danazol in patients with endometriosis-associated CPP.³⁵ One investigation showed that an injectable GnRH agonist has a substantially longer effect than MPA. Adjunctive measures such as stress and pain management and counseling should always be included in the treatment of patients with CPP.⁴² MPA remains the mainstay of hormonal manipulation but provides only a short period of relief.⁴¹ IV dihydroergotamine may be used for patients with acute exacerbations.³⁴

The effects of hormonal therapy have been explored in small, randomized, controlled trials, and other interventions have been examined in observational studies.⁴³ Oral contraceptives suppress ovulation, reduce spontaneous uterine activity, stabilize hormonal levels, and reduce subjective pain associated with menses. Because of these actions, oral contraception is appropriate therapy for endometriosis-associated CPP.³⁵ Nonsteroid antiinflammatory drugs (NSAIDs) or oral contraceptive agents may symptomatically address endometriosis. NSAIDS alone relieve symptoms in 40% of patients, and when combined with psychotherapy help 60% of women.⁴⁴

Opioids are increasingly used, and although these agents address pain but do not improve functional or psychological status.³⁵ Selective serotonin reuptake inhibitors such as sertraline offered no improvement in pain scores.³⁴ Another antidepressant class, called tricyclics, however, have been shown to improve pain levels and tolerance in some patients but were limited by side effects at higher doses.³⁵ Aspirin at 15 to 200 mg/d may alleviate those symptoms, especially those associated with nutcracker phenomena.⁵⁰

Transcatheter Embolization. The first transcatheter embolization (TCE) of ovarian varices was in 1993 by Edward et al.^41,48 Transcatheter coil embolization brings about significant improvements in quality of life, with less postoperative pain and improved cosmesis as reported in 75% of patients of one study.⁵¹ The particular choice of TCE at the sacroiliac joint assures complete ovarian vein embolization without damage of the deep pelvic plexus. Because anastomoses develop upon congestion, embolectomy is a preferred solution to target the site of the enlarged vein.³⁹ Some centers report technical success rates of 96% to 99%.³⁸ Kwon et al⁴⁸ set forth the following criteria for coil embolization in their study of this intervention in multiparous women with CPP: dilatation of the ovarian vein larger than 5 mm, ovarian vein reflux into the pelvic cavity involving an incompetent valve, severe congestion of the pelvic venous plexus, significant stasis of the contrast medium in the pelvic veins, abnormal filling of the pelvic veins across the midline, and filling of vulvovaginal or thigh varicosities. They demonstrated that 82% of patients reported significant or complete reduction of pain after embolization.⁴⁸ The current norm and choice intervention for pelvic variceal disease is bilateral ovarian vein embolization.¹⁵ Internal iliac vein embolization may be undertaken if primary ovarian vein embolization is inadequate.⁴⁸

Chung and Huh⁵¹ explored treatments for patients with PCS and compared minimally invasive ovarian vein or internal iliac vein embolotherapy, total abdominal hysterectomy with bilateral salpingo-oopherectomy (TAH-BSO), and total abdominal hysterectomy with unilateral salpingo-oopherectomy (TAH-USO) after patients failed 4 to 6 months of medical therapy. Their research shows embolotherapy to be superior to the other interventions, especially in patients with lower stress scores or experiencing a typical amount of stress. TAH-USO was the least effective treatment.⁵¹ Nicholson and Basile⁴⁴ cite similar successes for ovarian vein embolization, showing a 73% to 78% cure or symptomatic improvement. Other investigators found that 83% of patients have clinical improvements up to 4 years after TCE.⁴⁸ Alternately, sclerotherapy via 5% ethanolamine oleate reduces blood flow to these varices and was successful in a pioneering case report, showing such improvement that the patient remained symptom free into pregnancy.⁴⁵ Sclerotherapy is a sound choice for addressing vulvar varices.⁴⁷

Stent Placement. Venous stent placement may be used for anatomic anomalies.³⁸ Endovascular therapy for the nutcracker syndrome was proposed by Neste in 1996 and has also been used in addressing May-Thurner syndrome.⁵³ Hartung et al⁵³ examined the utility of left ovarian vein stenting to alleviate nutcracker syndrome as the cause of CPP and found that three of five patients achieved modest relief.

Laparoscopy. Laparoscopy and adhesion lysis may also be used to treat patients with endometriosis, although a Cochrane review stated that “there is no evidence of benefit rather than evidence of no benefit.”³⁴ Conscious pain mapping, or patient-assisted laparoscopy, involves a sedated but conscious patient to assist the surgeon to the true cause of her CPP. This special sort of laparoscopy was first described in 1996 as an alternative to the traditional laparoscopy wherein 50% of the etiology of CPP remains undiagnosed.³⁶ Forty-three women with CPP underwent laparoscopy under local anesthesia and gave pain scores as a blinded examiner mobilized organs and adhesions before initiation of medical or surgical treatment. This small study showed that 74% of women experienced a statistically significant reduction in pain.³⁶ No substantial data exist to confirm improvement with diagnosis or clinical outcomes with pain mapping.³⁵ In 1984, Rundqvist⁴¹ found that extraperitoneal resection of the left ovarian vein decreased PCS symptoms. The more modern laparoscopic ovarian vein ligation boasts a 73% cure rate and a 78% improvement in patient symptoms.⁴⁴ Gargiulo et al⁴⁶ reported on the feasibility of transperitoneal laparoscopic ligation of ovarian veins, showing that 78% of patients had resolution of pain.