An unusual case of Behçet's disease presenting with postpartum ovarian iliac vein thrombosis and pulmonary embolism
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Thrombosis of the ovarian vein is a rare complication which arises classically in the postpartum. We report a case of 24-year-old woman with a history of Behçet's disease, who presented with pelvic and thoracic pain, tachycardia, dyspnea and fever occurring 2 weeks after delivery. Computed tomography revealed an ascending thrombosis of the iliac and right ovarian veins complicated by bilateral pulmonary embolism. The patient responded well to the combination of anticoagulants and immunosuppressive agents. Behçet's disease should also be considered as an etiologic factor for ovarian vein thrombosis.
KeywordsPulmonary Embolism Hyperhomocysteinemia Pulmonary Thromboembolism Ovarian Vein Vein Imaging
Postpartum ovarian vein thrombosis
Behçet's disease (BD) is a multisystemic recurrent inflammatory disorder. First described was the characteristic triad of oral ulcerations, genital ulcerations and, ocular lesions and later additional manifestations were described, including disorders of the skin, joints, large vessels, lungs, brain, and gastrointestinal and genitourinary tracts . BD strongly predisposes patients to venous and arterial thrombosis but pulmonary thromboembolism is very uncommon and it is occasionally observed in the form of deep vein thrombosis .
We present a patient with BD who developed thrombosis of the ovarian and iliac veins complicated by pulmonary thromboembolism during the postpartum period.
The influence of pregnancy on BD is variable and has not yet been clearly demonstrated. Pregnancy is associated with disease exacerbation in 27 % of patients . Our patient had active BD when she became pregnant for the second time (ulceration of the mouth and pseudofolliculitis), and simultaneously developed two thrombotic events at different sites during the puerperium. This suggests a possible interaction between BD and pregnancy.
Postpartum ovarian vein thrombosis (POVT) is an uncommon but potentially serious disorder. Melis et al reported that the risk of deep vein thrombosis is elevated 5 to 6 fold during pregnancy and 2 to 3 fold during the puerperium . This hypercoagulable state is present for 6 weeks postpartum, and can be explained by venous stasis or changes in the hemostatic system with increasing procoagulant factors and platelet adhesiveness. In addition, intrauterine infection and puerperal hypercoagulability, due to recent surgery, malignancy, thrombophilias, lupus, sickle cell disease, Crohn's disease, obesity, fluid and electrolyte imbalance, etc., might also be contributing factors [5, 6, 7]. Salomon et al reported that 50 % of patients have an inherited thrombophilia . Our patient did not have symptoms suggestive of any of these disorders. In addition, a diagnosis of intrauterine infection was not supported with complete clinical resolution after anticoagulant and immunosuppressive therapy. Vasculitic and thrombotic abnormalities of coagulation or fibrinolitic activity are both well known to predispose to thrombosis in BD . Additionally, in this case hyperhomocysteinemia might have contributed to the hypercoagulable state. Ates et al  reported that homocysteine levels are independently associated with thrombosis, but recent studies did not reveal such an association .
The main sign of typical clinically significant ovarian vein thrombosis is pain in the flanks or the iliac fossa, in association with fever. Thrombosis is most frequently identified in the right ovarian vein due to the physiologic dextrorotation of the uterus, which might compress the ovarian vein. When the thrombus is located on the right side, several conditions should be considered in the differential diagnosis, such as pyelonephritis and acute appendicitis. Besides the clinical signs, CT and magnetic resonance imaging are recommended as the procedures of choice for ovarian vein imaging . They are also recommended for evaluating wall thickening of the pulmonary artery.
POVT is a rare complication with potentially devastating consequences, including caval thrombus, pulmonary thromboembolism, and death . In BD, however, pulmonary embolism rarely occurs because the thrombi in the inflamed veins are strongly adherent. It is therefore possible that in active BD, pulmonary involvement is in part a result of insitu pulmonary pathology rather than embolization from a systemic vein. In Mogulkoc's review, 6 of 12 patients with pulmonary arterial thrombosis were free of peripheral thrombi . The incidence of pulmonary embolism in women with POVT has been reported to be from 13 % to 33 % especially when thrombus may extend into inferior vena cava . Kettaneh et al. report two patients with POVT complicated by pulmonary embolism in both cases . In our case pulmonary embolism may be a complication of the iliac vein thrombosis, there is no extension into vena cava of the ovarian vein thrombosis. Our patient responded well to the combination of anticoagulant, colchicine, corticosteroids and immunosuppressive agents. There is no agreement on the therapy of deep venous thrombosis and pulmonary embolism in BD. Thrombus is supposed to be due to the vasculitic process and the aim of our treatment being to eradicate the thrombus and prevent recurrence, for this reason immunosuppressant therapy can be added to anticoagulation in active Behçet's disease associated central venous thrombus especially when biological inflammation is present, On the other hand it is important to note that in the presence of pulmonary involvement, anticoagulant alone failed to prevent recurrence in some patient. Surgical treatment for this condition is generally reserved for patients for whom anticoagulation is contraindicated and who have recurrent pulmonary emboli despite medical management.
To the best of our knowledge, this is the first case of postpartum ovarian thrombosis in BD associated with hyperhomocysteinemia and pulmonary embolism. We conclude that BD should always be considered as an etiologic factor for ovarian thrombosis, especially during pregnancy and puerperium, for this reason prophylactic anticoagulant therapy might be considered depending on the risk factors.
Written consent was obtained from the patients and their relative for publication of study. We would like to thank Pr M. Hammami for his biochemistry assistance to homocysteinemia analysis. We thank Mahmoud Belkouja for her expert technical assistance
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