Overview

 Vulvar varicosity is a term used for swollen, twisted veins present in the female external genitalia, the labia. These are mostly without symptoms but may become symptomatic and can cause discomfort during pregnancy. ; -However, they tend to resolve on their own about 40 days after delivery in most cases.¹

Keywords: Vulvar varicosities (VV), Pelvic congestion syndrome, Deep vein thrombosis (DVT).

How common is the disease?

The number of women affected by vulvar varicosities is found to be 4%; The rate is higher in pregnant women(18%-22%) and women with pelvic congestion syndrome (22%-34%). Usually, the vulvar varicose veins resolve by six weeks after birth but may persist in about 4%-8% of cases. The incidence of vulvar varicosities in non-pregnant women is rare and occurs in the second or third decade of life.¹

How the blood vessels in the vulva are built and how they function 

The vulva has a rich network of interconnected blood vessels. Blood flows from the veins in the vulva to the larger veins in the body( the great saphenous veins and internal iliac vein) Vulvar varicosities develop in this rich network of veins.^1,4

Causes and risk factors

Increased pressure in the pelvic region is the main cause of vulvar varicosities. This increase in pressure can be due to backflow of blood in the veins blockage.⁸

Risk factors for developing vulvar varicosities are;

Pregnancy

 a number of anatomic and physiologic changes contribute to developing vulvar varicosities during pregnancy, including

• A growing fetus can exert pressure on the veins, causing iliac vein compression and thus compromising blood drainage
• An increase in pelvic blood flow during pregnancy can result in venous congestion in the lower extremities and pelvis
• Hormonal changes like high estrogen and progesterone can cause dilation of the veins and thus causing stagnancy of blood^2,4

Increase in blood volume

 An Increase in blood volume in veins, either due to pregnancy or due to incompetent valves in non-pregnant women, can cause an increase in venous pressure.

Obesity

 obesity increases the risk of iliac vein compression, resulting in higher chances of developing vulvar varicosities.

Chronic constipation

straining due to chronic constipation when having bowel movements increases pressure on the veins, causing them to dilate.

Genetics 

Vulvar varicocities may be associated with abnormalities in genes like CASZ1, PIEZO1, PPP3R1, EBF1, STIM2, HFE, GATA2, NFATC2, and SOX9.³

Hormonal imbalance

 Elevated progesterone or estrogen in certain conditions like ovarian cysts, and adrenal gland dysfunction can cause veins to stretch and dilate.

Multiparity

History of two or more full-term pregnancies.²

Pathophysiology

Vulvar varicosities are tortuous and dilated veins in the vulva that develop from a venous obstruction, valvular incompetence, or both, resulting in poor drainage and pooling of venous blood in the pelvic region, causing increased venous pressure in the vulvar veins.^1,8 Perineal veins lack valves, making them susceptible to developing VV. Incompetent great saphenous veins, internal iliac, and ovarian veins may cause massive VV.⁴

Clinical presentation

Vulvar varicosities are often asymptomatic and mostly appear during pregnancy. Common symptoms of VV include discomfort, swelling, and pressure in the vulva that is aggravated by prolonged standing, exercise, and intercourse.

The symptoms may range widely from mild discomfort to difficulty walking, depending on the severity of the condition.^4,6

Other symptoms are;¹

• Pain and tenderness in vulva
• Heaviness and burning in the perineum
• Frequent or painful urination
• Pain during sex and after sex⁷
• Painful menstruation
• Itching and skin irritation surrounding varicosities

Diagnostic evaluation

Physical examination findings

The diagnosis of vulvar varicosities is usually made following a physical examination where VV appears as swollen, purple-bluish folds in labia that are tender on palpation or as soft grape-like clusters( bag of worms). These may or may not be associated with varicose veins in the lower extremities. The lining of the vagina is swollen and congested.^1,4

Imaging studies

Diagnostic tests used to evaluate vulvar varicosities and are helpful in therapeutic planning include;

• Ultrasound; Duplex ultrasound is done to determine vascular incompetency, connections with the saphenous vein, and iliac vein compression¹
• Computed Tomography(CT) and Magnetic Resonance Imaging(MRI) are done to blood flow patterns in detail
• Venography; This is an invasive imaging study done by injecting contrast media into the veins and obtaining an X-ray image. This is mostly performed if an invasive therapy like embolization is to be planned

Other similar diseases

The following are differential diagnoses of vulvar varicosities, that can be ruled out based on history, examination, and imaging studies.

• Venous malformations; these are rare congenital dysmorphic vessels in the vulva
• Hematoma; this should be suspected if there is a history of recent trauma
• Hernia; no blue-purple discoloration is present, unlike in vulvar varicosities
• Bartholin gland cyst; this appears as a soft, non-tender mass on either side of the vaginal opening⁵

Treatment options

Treatment options for vulvar varicosities can be:

• conservative
• pharmacological and
• minimally invasive
•  surgical procedures

Treatment depends on the 

• the severity of symptoms, 
• pregnant state, or
•  presence of other conditions like DVT or Pelvic congestion syndrome

Conservative management¹

This includes lifestyle modifications to reduce pressure in the veins, like;

• Leg elevation
• Avoiding prolonged sitting or standing
• Use of support garment
• Maintaining a healthy Body Mass Index(BMI)
• Applying cold compresses to the vulva

Minimally invasive procedures

They include sclerotherapy and embolization. They are preferred for isolated VV and considered when VV are symptomatic and persists after conservative treatment.⁶

Sclerotherapy

A solution of sodium tetradecyl sulfate or polidocanol is injected into the varices; this causes varices to fibrosis and shrink. Patients usually require two to three sessions and improvement begins to show up within seven days. This procedure can cause discomfort for three to four days, which can be managed with the help of oral analgesics. Patients need to wear a compression girdle for 48 hours to avoid the complications of thrombosis.¹

Embolization; A device like a metal coil is placed in a vein to block blood flow, in case of pelvic and gonadal vein incompetence, like ovarian vein reflux.⁷

Surgical intervention

 Phlebectomy (removal of venous segments via small incisions) is only performed in severe cases. Pelvic venous congestion, dilation and reflux in ovarian veins are an absolute indication of surgery.¹

Management during pregnancy^1,4,5

Management of vulvar varicosities during pregnancy is usually by a conservative approach like wearing specialized compression undergarments. harmacological agents like Detralex are approved for use in the second trimester of pregnancy, as vulvar varicosities are mostly evident in the second and third trimesters. 

Other symptoms, like itching around varices and skin irritation, can be relieved by H1 histamine receptor blockers and zinc oxide. 

The mode of delivery in cases of large vulvar varicosities is via cesarean section to avoid potential rupture and bleeding from the veins; however, the presence of vulvar varicosities is not a contraindication to vaginal delivery. Hence, the decision for the mode of delivery is made by a team of experts comprising an obstetrician and a vascular surgeon.

FAQ’s

What causes vulvar varicosities?

Vulvar varicosis happens when the veins in the vulva are unable to circulate blood efficiently from pethe rineum up to the heart, so the blood starts to pool up in the veins, increasing venous pressure and causing them to dilate and swell up. Any changes in the body that lead to increase in blood volume or pressure in the veins can cause this condition like hormonal changes, Gravid uterus or vascular incompetency.

What are some Dos and Don’ts to consider during pregnancy?

The following things should be kept in mind During pregnancy if you have vulvar varicosities:

Don’t:

• Stand, sit or squat for too long.
• Eat lots of salty food

Do:

• Keep yourself hydrated 
• Lie down with legs elevated 
• Lie on your left side
• Wear a compression garment like tight elastic underwear that is designed for VV.

Summary

Vulvar varicosities are common in pregnancy, especially in multiparous women, and are often associated with pelvic congestion syndrome, or lower extremity varicoses, but can also occur in isolation. These can cause chronic pain, discomfort, swelling, itching, and fullness in the vulva. To reduce the risk of developing vulvar varicosities, it is highly recommended to monitor weight, avoid prolonged standing, exercise regularly, and consult an obstetrician in case of experiencing any form of symptoms. Its management requires a collaborative approach that can make an early and correct diagnosis and treatment possible