Chapter: Consultation hour
Article: 10 of 12
Update: Feb 24, 2021
Author(s): Wohlgemuth, Walter A.
Questions about sexual life, contraception and possible changes in a vascular anomaly under the hormonal influence of pregnancy are highly relevant. However, appropriate detailed advice on these issues requires direct dialog and precise knowledge of the characteristics of the individual disease. A trusting doctor-patient relationship is the basis. Nevertheless, some important basics can be addressed here.
Many, although not all, women with larger venous malformations (VM) often experience a temporary worsening of symptoms around the time of their period, especially pain in the affected area. This is due to hormone-induced changes in the blood’s clotting ability, with increased painful thrombophlebitis or LIC within the venous malformation.
In this situation, as well as consistent compression therapy, it seems to make sense to avoid excessive physical exercise as well as prolonged standing and sitting without movement. This situation is of a temporary nature.
Extensive venous and combined venolymphatic malformations of the lower extremity often affect the entire body quadrant of one side, i.e., including the buttocks and also the genital area in women (labia) and men (scrotum).
Venous malformations, in particular, often extend to the ipsilateral scrotum in male patients, and somewhat less frequently to the penis. Even if more extensive, this is usually not very symptomatic in men and more cosmetically noticeable. Sexual function and fertility in men is not disturbed in most of these cases since the changes are usually epifascial and surround but do not involve the testis. An invasive therapy (sclerotherapy) is rarely necessary, but it is certainly possible and successful.
In women, the labium majus (usually on one side, more rarely on both sides) is often affected together with the ipsilateral leg, especially in the case of extensive venous or lymphatic malformations. In these cases, painful thrombophlebitis is more frequent, with increased swelling/increased volume, especially in connection with menstruation. Large thrombophlebitis can also lead to sometimes large local painful swellings, which then impair sexual function and are painful when sitting or riding a bicycle. For women, therefore, invasive therapy with sclerotherapy is more likely to be indicated. Since resection operations can cause local sensitivity disorders and also significant bleeding, this is a domain of sclerotherapy, which can be performed with good results. It is important to take a proper history that also includes this problem because patients are less likely to report this themselves.
Patients who have a vascular anomaly in the genital area sometimes express the fear that bleeding could be triggered there, especially during sexual intercourse. This danger does not usually exist, as these are slow-flow malformations that are not under arterial pressure. However, sexual intercourse can be painful if thrombophlebitis is present.
Since oral contraception (birth control pill) can have an influence on blood coagulation, and can particularly increase blood coagulation, increased pain and thrombophlebitis can occur in female patients with venous malformations. Furthermore it is possible that, under the influence of hormones (in a similar way to pregnancy), an arteriovenous malformation in particular can change into proliferation. The same applies to hormone replacement therapy in postmenopausal women. As the combination of estrogen and progestin contraceptives is primarily held responsible for these possible deteriorations, a low-dose mono-preparation should be chosen, if possible. It should be noted that a somewhat poorer contraceptive effect is achieved if the product is taken irregularly.
According to available studies, the tendency to deep vein thrombosis also increases to varying degrees depending on the progestin used. Therefore, when using a combined preparation containing an estrogen and a progestin, a preparation with a lower estimated risk of thrombosis (levonorgestrel, norethisterone or norgestimate) should be prescribed.
If mono-preparations are not tolerated, another alternative is a hormone-containing vaginal ring, which only releases low blood hormone levels. As an alternative, purely mechanical contraceptive methods (e.g., coil) or, if family planning is completed, sterilization of one of the partners may also be considered.
Most vascular anomalies occur spontaneously as a so-called genetic mosaic (not affecting all body cells, especially not the germ cells). This means they are not hereditary and therefore the risk of the same disease occurring in potential children of affected individuals is not increased. The few familial forms that are inherited must be diagnosed by a specialist. If this is the aim of couples who wish to have children, appropriate genetic counselling is advisable.
Probably under the influence of physiological hormones, however, some affected women may experience a worsening of symptoms during pregnancy, especially in the last trimester and in some cases in the weeks after birth, especially in those with venous and lymphatic malformations, but also in arteriovenous malformations. Pregnant patients with HHT Osler’s disease have a particularly increased risk. Recurrences in pretreated vascular anomalies are also more frequent after pregnancy. Therefore, appropriate consultation and, if necessary, prophylactic therapy before a planned pregnancy are certainly advisable.
Intensified compression therapy can be useful as the most important basic therapy during pregnancy. This also reduces the occurrence of increased pain and thrombophlebitis as well as thromboembolic events during pregnancy.
As a result of the hormonal changes, temporary progression of the disease can occur in all patients with vascular anomalies. However, this is only the case in some patients (up to 40%), especially patients with extensive vascular anomalies. For this reason, an individual consultation is necessary.
In some patients with previously untreated or insufficiently treated venous malformations, where there is a connection to the deep venous system (“communicating vein” or marginal vein) with a correspondingly increased risk of thrombosis or thromboembolism, the risk of this complication may be higher during pregnancy. Appropriate therapy before pregnancy with interventional or surgical closure of these communicating veins can significantly reduce or eliminate this risk. For some patients, longer-term anticoagulation with heparin may additionally be useful and necessary in consideration of the individual risk-benefit ratio during pregnancy. Data are available for patients with Klippel-Trénaunay syndrome, although the results were not entirely consistent. On the one hand, there was no difference in the frequency of complications between pregnant and non-pregnant women with Klippel-Trénaunay syndrome. Nevertheless, the risk of complications was increased compared to normal pregnant women, and the rate of postpartum bleeding was twice as high. In another study comparing Klippel-Trénaunay syndrome patients with non-pregnant women, 43% of KTS patients reported more symptoms during pregnancy. 6% experienced thrombosis during pregnancy and 2.3% experienced pulmonary embolism. Thus, the complication rate was significantly higher than for normal pregnant women.
In patients with arteriovenous malformations, but especially patients with HHT Osler’s disease and arteriovenous malformations of the lung or liver, the precise status of the findings must be evaluated before or at the start of pregnancy. These aneurysms sometimes expand rapidly and considerably, which can lead to dangerous bleeding. It is known from the literature that, especially in the second and third trimesters, this can cause a deterioration with increased bleeding in arteriovenous malformations of the lung and liver (high-output cardiac failure); paradoxical embolisms have also been described.
Patients with macrocystic lymphatic malformations of the spleen are a rare special case. These cysts can enlarge during pregnancy and rupture and bleed if in a subcapsular position.
Overall, however, the presence of a vascular anomaly is by no means a contraindication to pregnancy. An individual consultation at the onset of pregnancy or before a planned pregnancy at an interdisciplinary specialist center for vascular anomalies together with geneticists can help to clarify the situation, allay fears and, if necessary, help to initiate or optimize any necessary additional therapy.