Vulvovaginal varicosities and pelvic congestion syndrome
- Natasha R Johnson, MD
Natasha R Johnson, MD
- Assistant Professor in Obstetrics, Gynecology and Reproductive Biology
- Harvard Medical School
- Section Editors
- Robert L Barbieri, MD
Robert L Barbieri, MD
- Editor-in-Chief — Obstetrics, Gynecology and Women's Health
- Section Editor — General Gynecology and Female Reproductive Endocrinology
- Kate Macy Ladd Professor of Obstetrics, Gynecology and Reproductive Biology
- Harvard Medical School
- John F Eidt, MD
John F Eidt, MD
- Section Editor — Vascular Surgery
- Professor of Surgery
- University of South Carolina School of Medicine Greenville
- Joseph L Mills, Sr, MD
Joseph L Mills, Sr, MD
- Section Editor — Vascular Surgery
- Professor of Surgery
- University of Arizona Health Sciences Center
- Deborah Levine, MD
Deborah Levine, MD
- Section Editor — Imaging
- Professor of Radiology
- Co-Chief of Ultrasound
- Beth Israel Deaconess Medical Center
The pelvic venous syndromes, which include pelvic congestion syndrome and vulvar varicosities, are poorly understood disorders of the pelvic venous circulations.
- Pelvic congestion syndrome (PCS) is characterized by chronic pelvic discomfort exacerbated by prolonged standing and coitus in women who have periovarian varicosities on imaging studies. The etiology of PCS is unclear and the optimum treatment is uncertain. Development of an evidence-based approach to managing these patients has been limited by the absence of definitive diagnostic criteria.
- Vulvar varicosities result from venous obstruction, increased venous pressure, and venous insufficiency, most commonly during pregnancy. They may be isolated or associated with varices of the lower extremity, and they may occur as part of PCS.
Epidemiology — It is estimated that 4 percent of women have had vulvar varicosities . They usually occur during pregnancy and typically regress spontaneously within six weeks postpartum. They are rare in nulliparous women. When they occur in non-pregnant women, they generally present in the second or third decade of life .
Pathogenesis/pathology — Vulvar varicosities are dilated venous channels that probably develop from a combination of proximal venous obstruction and valvular incompetence, which results in increased venous pressure. Their anatomy has been defined by direct injection and surgical dissection of the varices.
The vascular drainage of the female external genitalia includes the dorsal superficial clitoral, bulbovestibular, profundus clitoral, and posterior labial veins. Because these perineal veins do not have valves, they are susceptible to the development of varices. Up to one-half of vulvar varicosities arise from an incompetent great saphenous vein, which drains the superficial and deep external pudendal veins and posteromedial tributaries.
- Hobbs JT. The pelvic congestion syndrome. Br J Hosp Med 1990; 43:200.
- WILSON CE. MANAGEMENT OF VARICOSE VEINS IN PREGNANCY. Nebr State Med J 1964; 49:368.
- Scultetus AH, Villavicencio JL, Gillespie DL, et al. The pelvic venous syndromes: analysis of our experience with 57 patients. J Vasc Surg 2002; 36:881.
- Ashour MA, Soliman HE, Khougeer GA. Role of descending venography and endovenous embolization in treatment of females with lower extremity varicose veins, vulvar and posterior thigh varices. Saudi Med J 2007; 28:206.
- Royle JP, Macafee CA, Beischer NA. An unusual presentation of varicose veins of the vulva. Aust N Z J Obstet Gynaecol 1971; 11:122.
- LePage PA, Villavicencio JL, Gomez ER, et al. The valvular anatomy of the iliac venous system and its clinical implications. J Vasc Surg 1991; 14:678.
- Dixon JA, Mitchell WA. Venographic and surgical observations in vulvar varicose veins. Surg Gynecol Obstet 1970; 131:458.
- Marrocco-Trischitta MM, Nicodemi EM, Nater C, Stillo F. Management of congenital venous malformations of the vulva. Obstet Gynecol 2001; 98:789.
- Ninia JG, Goldberg TL. Treatment of vulvar varicosities by injection-compression sclerotherapy and a pelvic supporter. Obstet Gynecol 1996; 87:786.
- Veltman LL, Ostergard DR. Thrombosis of vulvar varicosities during pregnancy. Obstet Gynecol 1972; 39:55.
- Beard RW, Highman JH, Pearce S, Reginald PW. Diagnosis of pelvic varicosities in women with chronic pelvic pain. Lancet 1984; 2:946.
- Beard RW, Reginald PW, Wadsworth J. Clinical features of women with chronic lower abdominal pain and pelvic congestion. Br J Obstet Gynaecol 1988; 95:153.
- Venbrux AC, Lambert DL. 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 1999; 11:395.
- Bell D, Kane PB, Liang S, et al. Vulvar varices: an uncommon entity in surgical pathology. Int J Gynecol Pathol 2007; 26:99.
- Herman AR, Morello F, Strickland JL. Vulvar venous malformations in an 11-year-old girl: a case report. J Pediatr Adolesc Gynecol 2004; 17:179.
- Cordts PR, Eclavea A, Buckley PJ, et al. Pelvic congestion syndrome: early clinical results after transcatheter ovarian vein embolization. J Vasc Surg 1998; 28:862.
- Mathis BV, Miller JS, Lukens ML, Paluzzi MW. Pelvic congestion syndrome: a new approach to an unusual problem. Am Surg 1995; 61:1016.
- Giannoukas AD, Dacie JE, Lumley JS. Recurrent varicose veins of both lower limbs due to bilateral ovarian vein incompetence. Ann Vasc Surg 2000; 14:397.
- Wang S, Lang JH, Zhou HM. Venous malformations of the female lower genital tract. Eur J Obstet Gynecol Reprod Biol 2009; 145:205.
- Nassiri N, O TM, Rosen RJ, et al. Staged endovascular and surgical treatment of slow-flow vulvar venous malformations. Am J Obstet Gynecol 2013; 208:366.e1.
- Cebesoy FB, Kutlar I, Aydin A. A rare mass formation of the vulva: giant cavernous hemangioma. J Low Genit Tract Dis 2008; 12:35.
- da Silva BB, Lopes-Costa PV, Furtado-Veloso AM, Borges RS. Vulvar epithelioid hemangioendothelioma. Gynecol Oncol 2007; 105:539.
- Bava GL, Dalmonte P, Oddone M, Rossi U. Life-threatening hemorrhage from a vulvar hemangioma. J Pediatr Surg 2002; 37:E6.
- Lazarou G, Goldberg MI. Vulvar arteriovenous hemangioma. A case report. J Reprod Med 2000; 45:439.
- Gearhart PA, Levin PJ, Schimpf MO. Expanding on earlier findings A vulvar varicosity grew larger with each pregnancy. Am J Obstet Gynecol 2011; 204:89.e1.
- Rasmussen OO, Hamilton Jakobsen B. Post-partum persisting pudendal varicose veins--effect of local excision. Vasa 1987; 16:352.
- Leung SW, Leung PL, Yuen PM, Rogers MS. Isolated vulval varicosity in the non-pregnant state: a case report with review of the treatment options. Aust N Z J Obstet Gynaecol 2005; 45:254.
- Beard RW, Reginald P, Pearce S. Pelvic pain in women. Br Med J (Clin Res Ed) 1987; 294:124.
- Soysal ME, Soysal S, Vicdan K, Ozer S. A randomized controlled trial of goserelin and medroxyprogesterone acetate in the treatment of pelvic congestion. Hum Reprod 2001; 16:931.
- Fassiadis N. Treatment for pelvic congestion syndrome causing pelvic and vulvar varices. Int Angiol 2006; 25:1.
- TAYLOR HC Jr. Vascular congestion and hyperemia; their effect on structure and function in the female reproductive system. Am J Obstet Gynecol 1949; 57:211.
- Hobbs JT. The pelvic congestion syndrome. Practitioner 1976; 216:529.
- Reginald PW, Beard RW, Kooner JS, et al. Intravenous dihydroergotamine to relieve pelvic congestion with pain in young women. Lancet 1987; 2:351.
- Tropeano G, Di Stasi C, Amoroso S, et al. Ovarian vein incompetence: a potential cause of chronic pelvic pain in women. Eur J Obstet Gynecol Reprod Biol 2008; 139:215.
- Rozenblit AM, Ricci ZJ, Tuvia J, Amis ES Jr. Incompetent and dilated ovarian veins: a common CT finding in asymptomatic parous women. AJR Am J Roentgenol 2001; 176:119.
- Gargiulo T, Mais V, Brokaj L, et al. Bilateral laparoscopic transperitoneal ligation of ovarian veins for treatment of pelvic congestion syndrome. J Am Assoc Gynecol Laparosc 2003; 10:501.
- Edwards RD, Robertson IR, MacLean AB, Hemingway AP. Case report: pelvic pain syndrome--successful treatment of a case by ovarian vein embolization. Clin Radiol 1993; 47:429.
- Maleux G, Stockx L, Wilms G, Marchal G. Ovarian vein embolization for the treatment of pelvic congestion syndrome: long-term technical and clinical results. J Vasc Interv Radiol 2000; 11:859.
- Venbrux AC, Chang AH, Kim HS, 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 2002; 13:171.
- d'Archambeau O, Maes M, De Schepper AM. The pelvic congestion syndrome: role of the "nutcracker phenomenon" and results of endovascular treatment. JBR-BTR 2004; 87:1.
- Menard MT. Nutcracker syndrome: when should it be treated and how? Perspect Vasc Surg Endovasc Ther 2009; 21:117.
- Ahlberg NE, Bartley O, Chidekel N. Right and left gonadal veins. An anatomical and statistical study. Acta Radiol Diagn (Stockh) 1966; 4:593.
- Farquhar CM, Rogers V, Franks S, et al. A randomized controlled trial of medroxyprogesterone acetate and psychotherapy for the treatment of pelvic congestion. Br J Obstet Gynaecol 1989; 96:1153.
- Chung MH, Huh CY. Comparison of treatments for pelvic congestion syndrome. Tohoku J Exp Med 2003; 201:131.
- Belenky A, Bartal G, Atar E, et al. Ovarian varices in healthy female kidney donors: incidence, morbidity, and clinical outcome. AJR Am J Roentgenol 2002; 179:625.
- Tu FF, Hahn D, Steege JF. Pelvic congestion syndrome-associated pelvic pain: a systematic review of diagnosis and management. Obstet Gynecol Surv 2010; 65:332.
- Park SJ, Lim JW, Ko YT, et al. Diagnosis of pelvic congestion syndrome using transabdominal and transvaginal sonography. AJR Am J Roentgenol 2004; 182:683.
- Freedman J, Ganeshan A, Crowe PM. Pelvic congestion syndrome: the role of interventional radiology in the treatment of chronic pelvic pain. Postgrad Med J 2010; 86:704.
- Gloviczki P, Comerota AJ, Dalsing MC, et al. The care of patients with varicose veins and associated chronic venous diseases: clinical practice guidelines of the Society for Vascular Surgery and the American Venous Forum. J Vasc Surg 2011; 53:2S.
- Desimpelaere JH, Seynaeve PC, Hagers YM, et al. Pelvic congestion syndrome: demonstration and diagnosis by helical CT. Abdom Imaging 1999; 24:100.
- Nascimento AB, Mitchell DG, Holland G. Ovarian veins: magnetic resonance imaging findings in an asymptomatic population. J Magn Reson Imaging 2002; 15:551.
- Meneses LQ, Uribe S, Tejos C, et al. Using magnetic resonance phase-contrast velocity mapping for diagnosing pelvic congestion syndrome. Phlebology 2011; 26:157.
- Shokeir T, Amr M, Abdelshaheed M. The efficacy of Implanon for the treatment of chronic pelvic pain associated with pelvic congestion: 1-year randomized controlled pilot study. Arch Gynecol Obstet 2009; 280:437.
- Kwon SH, Oh JH, Ko KR, et al. Transcatheter ovarian vein embolization using coils for the treatment of pelvic congestion syndrome. Cardiovasc Intervent Radiol 2007; 30:655.
- Pieri S, Agresti P, Morucci M, de' Medici L. Percutaneous treatment of pelvic congestion syndrome. Radiol Med 2003; 105:76.
- Sichlau MJ, Yao JS, Vogelzang RL. Transcatheter embolotherapy for the treatment of pelvic congestion syndrome. Obstet Gynecol 1994; 83:892.
- Kim HS, Malhotra AD, Rowe PC, et al. Embolotherapy for pelvic congestion syndrome: long-term results. J Vasc Interv Radiol 2006; 17:289.
- Ghosh A, Shafie-Pour H, Ayers KJ. Laparoscopic sclerotherapy in a case of pelvic congestion syndrome. BJOG 2006; 113:610.
- Beard RW, Kennedy RG, Gangar KF, et al. Bilateral oophorectomy and hysterectomy in the treatment of intractable pelvic pain associated with pelvic congestion. Br J Obstet Gynaecol 1991; 98:988.
- Paraskevas P. Successful ultrasound-guided foam sclerotherapy for vulval and leg varicosities secondary to ovarian vein reflux: a case study. Phlebology 2011; 26:29.
- VULVOVAGINAL VARICOSITIES
- Clinical manifestations
- - Differential diagnosis
- - Pregnant women
- - Sclerotherapy
- - Ligation
- - Ablation
- PELVIC CONGESTION SYNDROME
- Epidemiology PCS
- Pathogenesis/pathology PCS
- Diagnosis and diagnostic evaluation PCS
- - Clinical manifestations PCS
- - Physical examination PCS
- - Imaging PCS
- Computed tomography and magnetic resonance imaging
- - Laparoscopy PCS
- Differential diagnosis
- Management of PCS
- - PCS without vulvar varices
- - PCS with vulvar varices
- SUMMARY AND RECOMMENDATIONS