Multidisciplinary management of giant genital tract venous malformations during pregnancy: case report and review of the literature

Introduction

Vascular anomalies affecting the genital tract are rare conditions in adults. They may involve arterial, lymphatic or venous vessels. Venous malformations are due to vascular dysmorphogenesis. These slow-flow vascular lesions can be congenital or acquired later on in life. They may be isolated or syndromic such as in Klippel-Trenaunay syndrome [1, 2].

In specific conditions such as trauma, infection and hormonal changes associated with pregnancy, these venous malformations could increase in size and develop localised intravascular coagulopathy (LIC). This condition is suspected in the presence of high D-dimers, low fibrinogen level and reduced platelets counts. It can be associated with thrombosis with palpable phleboliths and also can decompensate in disseminated intravascular coagulopathy (DIC) [2].

Pregnant women affected by genital venous malformations are specifically at risk of these complications, mainly during postpartum [3–8]. This paper aims to review therapeutic approaches of these patients knowing the underlying physiopathology of LIC and define a perinatal multidisciplinary management protocol.

Case report

A 25-year-old primigravida patient with a giant vulvar vascular anomaly extending to the posterior gluteal region and the left thigh was referred to our centre for antenatal care. No genetic abnormality has been identified in the patient. The vascular lesion partially obstructed the vagina with no extension to the side walls and the cervix (Figure 1).

Figure 1:

Giant vulvar and thigh venous malformation prior to caesarean section.

Before pregnancy, she experienced chronic thrombocytopenia and LIC, had serial vascular sclerosis courses and an unsuccessful vulvar resection. She also presented twice a leg deep venous thrombosis, treated by low-molecular-weight heparin (LMWH). She was chronically anaemic (haemoglobin concentrations below 10 g/dL) and thrombocytopenic (platelets count below 130,000×10³/μL).

From early in pregnancy, she received preventively daily injections of 0.6 units of LMWH at half therapeutic dose (Enoxaparin 1 mg/kg). Her antenatal care was uneventful. She had a normal first trimester scan. Biochemical screening for Down syndrome was negative. Morphological ultrasound and foetal growth were normal.

During pregnancy, blood tests were stable with a microcytic hypochromic anaemia (haemoglobin around 10 g/dL), mild thrombocytopenia (platelets count around 128,000×10³/μL), normal fibrinogen but elevated D-dimers (>10,000 ng/m).

She was Rhesus negative and had a previous history of blood transfusion. In the third trimester of pregnancy, she developed Rhesus alloimmunisation with anti-D levels reaching 1/128. Serial ultrasound MCA maximal velocity measurement remained normal.

A preoperative MRI was done at 36+1 weeks’ gestation to evaluate epidural spaces. Vascular lesions found at the level of the third and fourth lumbar vertebra contraindicated a loco regional anaesthesia. Antithrombotic treatment with Enoxaparin (1 mg/kg) was continued until the day before the delivery.

Perinatal care was conducted by a multidisciplinary team including obstetricians, vascular surgeon, haematologist and anaesthetist. An elective caesarean section was planned under general anaesthesia at 37 weeks with availability of blood and resuscitation facilities. A technically uncomplicated caesarean section was performed with a transverse lower uterine segment incision. A 15 mm diameter haemangioma was found on the parietal peritoneal layer (Figure 2). A healthy female infant weighting 2700 g was delivered with a normal APGAR score of 6/9/9 at 1, 5 and 10 min. The neonate was not anaemic nor found with venous malformation.

Figure 2:

Parietal peritoneum haemangioma at laparotomy.

After delivery, the patient received 100 μg of oxytocin agonist, carbetocin (Pabal®) (Ferring Pharmaceuticals, Aalst, Belgium). There was no immediate haemorrhage with an estimated bleeding volume less than 250 mL. LMWH was restarted 6 h after delivery. At day 3, the patient experienced active vaginal bleeding. Uterine atony was suspected at ultrasound. Blood clots were expelled by transcervical aspiration associated with intravenous oxytocin for 48 h. As blood tests evidenced severe anaemia (6.1 g/dL) and consumptive coagulopathy (thrombocytopenia at 82,000×10³/μL, hypofibrinogenemia at 106 mg/dL), LMWH was discontinued at day 4 and the patient received five packed red cells transfusion. Computerized topography scan showed diffuse active intra-abdominal bleeding sites and an elective embolisation was done using transfemoral arteriography in superficial and deep artery branches. The patient was transferred to intensive care unit for active management with LMWH and transfusions (5 units of packed red blood cells and 8 units of fresh platelets). Anemia and thrombocytopenia resolved at day 5. LMWH was stopped at day 9. The patient recovered with no further complications and was discharged home at day 22. Histological analysis of the placenta showed the presence of one single capillary haemangioma of 3 mm.

Discussion

Venous vascular malformations involving the female genital tract are rare, and their therapeutic approaches remain unclear [1, 2]. Large vascular anomalies are often associated with LIC [2].

During pregnancy, they can be associated with uterine and placental involvement responsible for intrauterine death and severe bleeding disorders [3]. An increased morbidity and mortality rate is evidenced according to the tumour location and size. The specific pregnancy hormonal environment and “procoagulant” effect may be responsible for intra-tumoral bleeding and DIC if anaemia, thrombocytopenia and hypofibrinogenemia [2–6]. Venous stasis and abnormal endothelial cells may induce platelets pooling and destruction with subsequent coagulation cascade activation. The maternal morbidity is associated either with a massive haemorrhage or is resulting from the coagulopathy decompensation in DIC [2–8]. It is clear that pregnancy in patients with large venous tumors is a high risk condition requiring specific management protocols.

In the literature, we identified only six cases of pregnancy in patients with large abdominal and genital vascular anomalies (Table 1). All of them had a normal antenatal course but 5/6 reported shortly after delivery life threatening bleeding followed by DIC (thrombocytopenia and hypofibrinogenemia). Only two of them received IV heparin. Three patients were treated with e-aminocaproid acid, a derivative of the amino acid Lysine known to inhibit plasminogen activators and used to reverse fibrinolytic activity [4–6]. Three of the pregnant patients had serial blood components transfusions. One case reported by Phillippe et al. [5] required repeated blood, clotting factors and platelets transfusions, heparin and e-aminocaproic acid administration. It is important to notice that this patient had another causal factor for bleeding (placenta accreta). It required repeated D & C, and the diagnosis was confirmed by histopathology. All maternal and fetal outcomes were favourable except in one case with placental haemangioma involvement responsible for an intrauterine death [3].

In all cases including ours, severe postpartal complications required a multidisciplinary involvement.

Our paper aims to present a treatment algorithm for these patients during pregnancy:

• LMWH should be administered preventively even in the absence of deep thrombosis previous medical history, to prevent decompensation of LIC to DIC until delivery [2].

• A planned delivery in a tertiary centre with all therapeutic facilities is strongly recommended.

• Anaesthetists should evaluate the opportunity of loco regional approach and a preoperative MRI should be performed in order to exclude vascular abnormalities from the lumbar spine and epidural space [6].

• Blood products transfusions including platelets and fresh frozen plasma should be available as well as a trained haematologist.

• Operative procedures such as caesarean sections are known to stimulate the underlying process of disseminated intravascular coagulation [5]. The mode of delivery should take in account only obstetrical reasons such as dystocia due to the tumour location and the bleeding risk associated with a vaginal delivery with tears or episiotomy.

• After delivery, LMWH should not be discontinued even in the presence of bleeding and DIC. Although it may seem counterintuitive for most physicians to treat bleeding complications with an anticoagulant agent such as LMWH, it effectively reduces localised coagulation and reverses the pathophysiological process underlying clotting factors consumption.

• Other therapeutic options including steroids, antiplatelet and antifibrinolytic drugs, pneumatic compression are not associated with a significant better outcome [7].

• Although transarterial embolisation is a standard procedure offered for postpartum haemorrhages, it is not clear whether it was helpful in our case, and then, its efficacy should be evaluated prospectively.

Conclusions

Pregnant patients with extended venous malformations, are at high risk of severe post-partum complications. Involvement of a multidisciplinary team is essential and facilities should be quickly available for medical therapy, haematological support, embolisation and vascular surgery. Provided the haemorrhage and the consumption coagulopathy are controlled by an appropriate use of LMWH, the maternal and foetal prognosis for these high risk pregnancies is good.