Pelvic venous disorders (PeVD): Spectrum of clinical disorders presenting with chronic pelvic pain and varicosities related to pelvic venous incompetence (PVI) caused by venous reflux or nonthrombotic venous obstruction
Syndromic nomenclature, sites of pathology, and common mechanism of disease
Nutcracker syndrome (NS): Left renal vein (LRV); compression between aorta and superior mesenteric artery
May-Thurner syndrome (MTS): Common iliac vein (CIV); compression between spine and contralateral common iliac artery
Pelvic congestion syndrome (PCS): Gonadal vein (GV) ± internal iliac vein (IIV); reflux due to incompetent valves
Varicocele: Pampiniform plexus dilation; reflux of spermatic/GV
PeVD/PVI etiology: Often multifactorial
Primary valvular insufficiency due to absent/incompetent valves
Venous outflow obstruction
Anatomic: Nutcracker or May-Thurner anatomy; causes venous compression, elevated venous pressure, possible thrombosis or reflux
Presence of anatomic findings does not always mean patient will be symptomatic (i.e., nutcracker phenomenon vs. NS)
Pathologic: Retroperitoneal or pelvic mass causing venous obstruction
Particularly of concern with new right unilateral varicocele
Anatomic variants
Retroaortic LRV may elevate venous pressure/may cause GV reflux
Dysfunctional vasomotor hormonal regulation [uterine/ovarian veins (OV) are hormonally sensitive]
Clinical Presentation
NS: Hematuria and left flank pain
Can lead to additional findings of PCS
Due to increased reflux/pressure within left GV (LGV), which acts to decompress obstructed LRV
May need additional work-up (such as cystoscopy) to exclude other causes of hematuria
PCS: Chronic pelvic pain lasting > 6 months due to PVI and associated venous distention
Most often due to retrograde flow through incompetent valves in dilated ovarian/IIVs, causing tortuous, congested pelvic/periuterine veins
Can occur primarily or as secondary result of other anatomic abnormalities (such as NS)
Risk factors: Heredity, hormonal influence, pelvic surgery, retroverted uterus, history of varicose veins, and multiple pregnancies
Pelvic pain, pressure, or heaviness that is often worsened with movement, posture, and activities that increase abdominal pressure
Symptoms often worsen throughout day and are relieved with supine positioning
Superficial varicosities of vulva or lower extremities
Dyspareunia (71%), dysmenorrhea (66%)
Bladder irritability, urinary frequency
Symptoms typically disappear after menopause
Varicocele
Asymptomatic (common)
Palpable lump ("bag of worms"), scrotal thickening
Testicular pain, heaviness
Infertility/subfertility
Most common abnormality found in infertile men
Affects 40% of infertile men but only 15% of general male population
75% of men with varicocele have normal fertility
Etiology: Elevation of testicular temperature
Other proposed causes: Reflux of toxic metabolites, hypoxia from venous stasis
Anatomic Considerations
Anatomic findings of venous compression may be incidentally present without associated syndrome
Left-sided disease significantly more common than right side due to pattern of drainage
LGV → LRV → inferior vena cava (IVC)
LGV may also communicate with inferior mesenteric vein
Right GV → IVC
Enters below right renal vein
Complex venous communications common between GVs and internal iliac varices
Between GV and IIV
May cause OVs to be decompressed despite significant reflux
Normal-sized OVs (< 10 mm) does not exclude venous incompetence
Between uterine veins and OV or right and left endometrial veins
Incomplete embolization may result in persistent symptoms via uteroovarian arcade pelvic varices
GV anomalies common (both ovarian and spermatic)
Absent GV valves on left (15%) more common than right (6%)
Multiple LGV branches draining into LRV in 10-20%
Multiple right GV branches draining into right renal vein in 20-25%
Alternative drainage pathways may develop after varicocele embolization
External pudendal vein → greater saphenous vein
Ductus deferens vein → superior vesicular vein → IIV
Broe MP et al: Spermatic vein embolization as a treatment for symptomatic varicocele. Can Urol Assoc J. 15(11):E569-73, 2021
Chait J et al: Nutcracker syndrome: how to diagnose it and when/how should it be treated in the pelvic venous disease population. Tech Vasc Interv Radiol. 24(1):100734, 2021
Gavrilov S et al: Stratification of pelvic venous reflux in patients with pelvic varicose veins. J Vasc Surg Venous Lymphat Disord. 9(6):1417-24, 2021
Joh M et al: Ovarian vein embolization: how and when should it be done? Tech Vasc Interv Radiol. 24(1):100732, 2021
Khilnani NM et al: Clinical presentation and evaluation of pelvic venous disorders in women. Tech Vasc Interv Radiol. 24(1):100730, 2021
Velasquez CA et al: A systematic review on management of nutcracker syndrome. J Vasc Surg Venous Lymphat Disord. 6(2):271-8, 2018
Borghi C et al: Pelvic congestion syndrome: the current state of the literature. Arch Gynecol Obstet. 293(2):291-301, 2016
Halpern J et al: Percutaneous embolization of varicocele: technique, indications, relative contraindications, and complications. Asian J Androl. 18(2):234-8, 2016
Lorenc T et al: The value of ultrasonography in the diagnosis of varicocele. J Ultrason. 16(67):359-70, 2016
Rotker K et al: Recurrent varicocele. Asian J Androl. 18(2):229-33, 2016
Favard N et al: Comparison of three different embolic materials for varicocele embolization: retrospective study of tolerance, radiation and recurrence rate. Quant Imaging Med Surg. 5(6):806-14, 2015
Lurvey R et al: Adolescent varicocele: a large multicenter analysis of complications and recurrence in academic programs. J Pediatr Urol. 11(4):186.e1-6, 2015
Alkaram A et al: Varicocele and its effect on testosterone: implications for the adolescent. Transl Androl Urol. 3(4):413-7, 2014
Koo S et al: Pelvic congestion syndrome and pelvic varicosities. Tech Vasc Interv Radiol. 17(2):90-5, 2014
Kwak N et al: Imaging and interventional therapy for varicoceles. Curr Urol Rep. 15(4):399, 2014
Masson P et al: The varicocele. Urol Clin North Am. 41(1):129-44, 2014
Vanlangenhove P et al: Pathophysiology, diagnosis and treatment of varicoceles: a review. Minerva Urol Nefrol. 66(4):257-82, 2014
Laborda A et al: Endovascular treatment of pelvic congestion syndrome: visual analog scale (VAS) long-term follow-up clinical evaluation in 202 patients. Cardiovasc Intervent Radiol. 36(4):1006-14, 2013
Iaccarino V et al: Interventional radiology of male varicocele: current status. Cardiovasc Intervent Radiol. 35(6):1263-80, 2012
Monedero JL et al: Pelvic congestion syndrome can be treated operatively with good long-term results. Phlebology. 27 Suppl 1:65-73, 2012
Diegidio P et al: Review of current varicocelectomy techniques and their outcomes. BJU Int. 108(7):1157-72, 2011
Black CM et al: Research reporting standards for endovascular treatment of pelvic venous insufficiency. J Vasc Interv Radiol. 21(6):796-803, 2010
Tu FF et al: Pelvic congestion syndrome-associated pelvic pain: a systematic review of diagnosis and management. Obstet Gynecol Surv. 65(5):332-40, 2010
Cayan S et al: Treatment of palpable varicocele in infertile men: a meta-analysis to define the best technique. J Androl. 30(1):33-40, 2009
Gandini R et al: Transcatheter foam sclerotherapy of symptomatic female varicocele with sodium-tetradecyl-sulfate foam. Cardiovasc Intervent Radiol. 31(4):778-84, 2008
Ratnam LA et al: Pelvic vein embolisation in the management of varicose veins. Cardiovasc Intervent Radiol. 31(6):1159-64, 2008
Reiner E et al: Initial experience with 3% sodium tetradecyl sulfate foam and fibered coils for management of adolescent varicocele. J Vasc Interv Radiol. 19(2 Pt 1):207-10, 2008
Koc Z et al: Association of left renal vein variations and pelvic varices in abdominal MDCT. Eur Radiol. 17(5):1267-74, 2007
Kwon SH et al: Transcatheter ovarian vein embolization using coils for the treatment of pelvic congestion syndrome. Cardiovasc Intervent Radiol. 30(4):655-61, 2007
Liddle AD et al: Pelvic congestion syndrome: chronic pelvic pain caused by ovarian and internal iliac varices. Phlebology. 22(3):100-4, 2007
Kim HS et al: Embolotherapy for pelvic congestion syndrome: long-term results. J Vasc Interv Radiol. 17(2 Pt 1):289-97, 2006
Pavkov ML et al: Quantitative evaluation of the utero-ovarian venous pattern in the adult human female cadaver with plastination. World J Surg. 28(2):201-5, 2004
Venbrux AC et al: Pelvic congestion syndrome (pelvic venous incompetence): impact of ovarian and internal iliac vein embolotherapy on menstrual cycle and chronic pelvic pain. J Vasc Interv Radiol. 13(2 Pt 1):171-8, 2002
Venbrux AC et al: Embolization of the ovarian veins as a treatment for patients with chronic pelvic pain caused by pelvic venous incompetence (pelvic congestion syndrome). Curr Opin Obstet Gynecol. 11(4):395-9, 1999
Dubin L et al: Varicocele. Urol Clin North Am. 5(3):563-72, 1978
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References
Tables
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KEY FACTS
Terminology
Preprocedure
Post Procedure
Outcomes
TERMINOLOGY
Definitions
Pelvic venous disorders (PeVD): Spectrum of clinical disorders presenting with chronic pelvic pain and varicosities related to pelvic venous incompetence (PVI) caused by venous reflux or nonthrombotic venous obstruction
Syndromic nomenclature, sites of pathology, and common mechanism of disease
Nutcracker syndrome (NS): Left renal vein (LRV); compression between aorta and superior mesenteric artery
May-Thurner syndrome (MTS): Common iliac vein (CIV); compression between spine and contralateral common iliac artery
Pelvic congestion syndrome (PCS): Gonadal vein (GV) ± internal iliac vein (IIV); reflux due to incompetent valves
Varicocele: Pampiniform plexus dilation; reflux of spermatic/GV
PeVD/PVI etiology: Often multifactorial
Primary valvular insufficiency due to absent/incompetent valves
Venous outflow obstruction
Anatomic: Nutcracker or May-Thurner anatomy; causes venous compression, elevated venous pressure, possible thrombosis or reflux
Presence of anatomic findings does not always mean patient will be symptomatic (i.e., nutcracker phenomenon vs. NS)
Pathologic: Retroperitoneal or pelvic mass causing venous obstruction
Particularly of concern with new right unilateral varicocele
Anatomic variants
Retroaortic LRV may elevate venous pressure/may cause GV reflux
Dysfunctional vasomotor hormonal regulation [uterine/ovarian veins (OV) are hormonally sensitive]
Clinical Presentation
NS: Hematuria and left flank pain
Can lead to additional findings of PCS
Due to increased reflux/pressure within left GV (LGV), which acts to decompress obstructed LRV
May need additional work-up (such as cystoscopy) to exclude other causes of hematuria
PCS: Chronic pelvic pain lasting > 6 months due to PVI and associated venous distention
Most often due to retrograde flow through incompetent valves in dilated ovarian/IIVs, causing tortuous, congested pelvic/periuterine veins
Can occur primarily or as secondary result of other anatomic abnormalities (such as NS)
Risk factors: Heredity, hormonal influence, pelvic surgery, retroverted uterus, history of varicose veins, and multiple pregnancies
Pelvic pain, pressure, or heaviness that is often worsened with movement, posture, and activities that increase abdominal pressure
Symptoms often worsen throughout day and are relieved with supine positioning
Superficial varicosities of vulva or lower extremities
Dyspareunia (71%), dysmenorrhea (66%)
Bladder irritability, urinary frequency
Symptoms typically disappear after menopause
Varicocele
Asymptomatic (common)
Palpable lump ("bag of worms"), scrotal thickening
Testicular pain, heaviness
Infertility/subfertility
Most common abnormality found in infertile men
Affects 40% of infertile men but only 15% of general male population
75% of men with varicocele have normal fertility
Etiology: Elevation of testicular temperature
Other proposed causes: Reflux of toxic metabolites, hypoxia from venous stasis
Anatomic Considerations
Anatomic findings of venous compression may be incidentally present without associated syndrome
Left-sided disease significantly more common than right side due to pattern of drainage
LGV → LRV → inferior vena cava (IVC)
LGV may also communicate with inferior mesenteric vein
Right GV → IVC
Enters below right renal vein
Complex venous communications common between GVs and internal iliac varices
Between GV and IIV
May cause OVs to be decompressed despite significant reflux
Normal-sized OVs (< 10 mm) does not exclude venous incompetence
Between uterine veins and OV or right and left endometrial veins
Incomplete embolization may result in persistent symptoms via uteroovarian arcade pelvic varices
GV anomalies common (both ovarian and spermatic)
Absent GV valves on left (15%) more common than right (6%)
Multiple LGV branches draining into LRV in 10-20%
Multiple right GV branches draining into right renal vein in 20-25%
Alternative drainage pathways may develop after varicocele embolization
External pudendal vein → greater saphenous vein
Ductus deferens vein → superior vesicular vein → IIV
Broe MP et al: Spermatic vein embolization as a treatment for symptomatic varicocele. Can Urol Assoc J. 15(11):E569-73, 2021
Chait J et al: Nutcracker syndrome: how to diagnose it and when/how should it be treated in the pelvic venous disease population. Tech Vasc Interv Radiol. 24(1):100734, 2021
Gavrilov S et al: Stratification of pelvic venous reflux in patients with pelvic varicose veins. J Vasc Surg Venous Lymphat Disord. 9(6):1417-24, 2021
Joh M et al: Ovarian vein embolization: how and when should it be done? Tech Vasc Interv Radiol. 24(1):100732, 2021
Khilnani NM et al: Clinical presentation and evaluation of pelvic venous disorders in women. Tech Vasc Interv Radiol. 24(1):100730, 2021
Velasquez CA et al: A systematic review on management of nutcracker syndrome. J Vasc Surg Venous Lymphat Disord. 6(2):271-8, 2018
Borghi C et al: Pelvic congestion syndrome: the current state of the literature. Arch Gynecol Obstet. 293(2):291-301, 2016
Halpern J et al: Percutaneous embolization of varicocele: technique, indications, relative contraindications, and complications. Asian J Androl. 18(2):234-8, 2016
Lorenc T et al: The value of ultrasonography in the diagnosis of varicocele. J Ultrason. 16(67):359-70, 2016
Rotker K et al: Recurrent varicocele. Asian J Androl. 18(2):229-33, 2016
Favard N et al: Comparison of three different embolic materials for varicocele embolization: retrospective study of tolerance, radiation and recurrence rate. Quant Imaging Med Surg. 5(6):806-14, 2015
Lurvey R et al: Adolescent varicocele: a large multicenter analysis of complications and recurrence in academic programs. J Pediatr Urol. 11(4):186.e1-6, 2015
Alkaram A et al: Varicocele and its effect on testosterone: implications for the adolescent. Transl Androl Urol. 3(4):413-7, 2014
Koo S et al: Pelvic congestion syndrome and pelvic varicosities. Tech Vasc Interv Radiol. 17(2):90-5, 2014
Kwak N et al: Imaging and interventional therapy for varicoceles. Curr Urol Rep. 15(4):399, 2014
Masson P et al: The varicocele. Urol Clin North Am. 41(1):129-44, 2014
Vanlangenhove P et al: Pathophysiology, diagnosis and treatment of varicoceles: a review. Minerva Urol Nefrol. 66(4):257-82, 2014
Laborda A et al: Endovascular treatment of pelvic congestion syndrome: visual analog scale (VAS) long-term follow-up clinical evaluation in 202 patients. Cardiovasc Intervent Radiol. 36(4):1006-14, 2013
Iaccarino V et al: Interventional radiology of male varicocele: current status. Cardiovasc Intervent Radiol. 35(6):1263-80, 2012
Monedero JL et al: Pelvic congestion syndrome can be treated operatively with good long-term results. Phlebology. 27 Suppl 1:65-73, 2012
Diegidio P et al: Review of current varicocelectomy techniques and their outcomes. BJU Int. 108(7):1157-72, 2011
Black CM et al: Research reporting standards for endovascular treatment of pelvic venous insufficiency. J Vasc Interv Radiol. 21(6):796-803, 2010
Tu FF et al: Pelvic congestion syndrome-associated pelvic pain: a systematic review of diagnosis and management. Obstet Gynecol Surv. 65(5):332-40, 2010
Cayan S et al: Treatment of palpable varicocele in infertile men: a meta-analysis to define the best technique. J Androl. 30(1):33-40, 2009
Gandini R et al: Transcatheter foam sclerotherapy of symptomatic female varicocele with sodium-tetradecyl-sulfate foam. Cardiovasc Intervent Radiol. 31(4):778-84, 2008
Ratnam LA et al: Pelvic vein embolisation in the management of varicose veins. Cardiovasc Intervent Radiol. 31(6):1159-64, 2008
Reiner E et al: Initial experience with 3% sodium tetradecyl sulfate foam and fibered coils for management of adolescent varicocele. J Vasc Interv Radiol. 19(2 Pt 1):207-10, 2008
Koc Z et al: Association of left renal vein variations and pelvic varices in abdominal MDCT. Eur Radiol. 17(5):1267-74, 2007
Kwon SH et al: Transcatheter ovarian vein embolization using coils for the treatment of pelvic congestion syndrome. Cardiovasc Intervent Radiol. 30(4):655-61, 2007
Liddle AD et al: Pelvic congestion syndrome: chronic pelvic pain caused by ovarian and internal iliac varices. Phlebology. 22(3):100-4, 2007
Kim HS et al: Embolotherapy for pelvic congestion syndrome: long-term results. J Vasc Interv Radiol. 17(2 Pt 1):289-97, 2006
Pavkov ML et al: Quantitative evaluation of the utero-ovarian venous pattern in the adult human female cadaver with plastination. World J Surg. 28(2):201-5, 2004
Venbrux AC et al: Pelvic congestion syndrome (pelvic venous incompetence): impact of ovarian and internal iliac vein embolotherapy on menstrual cycle and chronic pelvic pain. J Vasc Interv Radiol. 13(2 Pt 1):171-8, 2002
Venbrux AC et al: Embolization of the ovarian veins as a treatment for patients with chronic pelvic pain caused by pelvic venous incompetence (pelvic congestion syndrome). Curr Opin Obstet Gynecol. 11(4):395-9, 1999
Dubin L et al: Varicocele. Urol Clin North Am. 5(3):563-72, 1978
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