A rare case of idiopathic bilateral ovarian vein thrombosis

A rare case of idiopathic bilateral ovarian vein thrombosis

THE CASES OF THE MONTH A rare case of idiopathic bilateral ovarian vein thrombosis Raudel Garcia, MD, Antonios P. Gasparis, MD, Shang A. Loh, MD, and ...

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THE CASES OF THE MONTH A rare case of idiopathic bilateral ovarian vein thrombosis Raudel Garcia, MD, Antonios P. Gasparis, MD, Shang A. Loh, MD, and Nicos Labropoulos, PhD, Stony Brook, NY

ABSTRACT Ovarian vein thrombosis (OVT) is a rare medical disorder most often diagnosed in the peripartum period and maybe associated with other risk factors for thrombosis. Rarely, OVT is considered idiopathic. It occurs in the right ovarian vein alone in two-thirds of patients. In this report, we present a case of idiopathic and bilateral OVT in a 35-year-old woman who presented with 2-day history of left flank pain. Duplex ultrasound imaging and computed tomography confirmed the diagnosis. Oral anticoagulation achieved a favorable outcome. (J Vasc Surg: Venous and Lym Dis 2017;5:567-70.)

Ovarian vein thrombosis (OVT) is a rare medical condition almost exclusive of the peripartum period with an estimated incidence of 0.18%.1,2 OVT in nonpregnant women is usually associated with a diverse acquired or inherited hypercoagulable state conditions.3 Very rarely, OVT can be considered idiopathic.4,5 A high index of clinical suspicion combined with selective imaging modalities is required for a definite diagnosis. The right ovarian vein (ROV) is frequently the site of the thrombosis;4,6,7 bilateral or isolated left OVT is seldom reported. Anticoagulation is the main therapy used to alleviate symptoms, prevent thrombus extension into the inferior vena cava (IVC) and left renal vein (LRV), and prevent pulmonary embolism (PE). The patient provided written consent for the publication of this paper in a medical journal.

computed tomography (CT) angiograms of the chest performed

CASE REPORT

den and prothrombin gene G20210A mutation analysis, protein

because of chest pain with elevated D-dimer during different visits to the emergency department. Abdominal examination revealed tenderness to deep palpation on left lower quadrant with no guarding or rebound. The remainder of her physical examination was benign. The results of laboratory examinations were unremarkable. A D-dimer test was not ordered because she presented with abdominal pain. An abdominal and pelvis CT with intravenous contrast revealed dilated ovarian veins, thickened walls, and low attenuation consistent with bilateral OVT (Fig 1). The results of duplex ultrasound (DUS) imaging determined the acuity of the thrombus (Fig 2). Thrombus did not propagate into the IVC or LRV, and no evidence of compression of the left renal or iliac veins was detected. A repeat hypercoagulable study, which included factor V Lei-

A 35-year-old woman (gravida 4, para 4) presented to the emergency department with worsening left flank pain for 2 days. She had reported a similar but low intensity left flank pain consistently for w5 years. She had no other signs and symptoms. She had no recent travel, operations, trauma, infections, or hospitalizations, and she was not taking an oral contraceptive. Her medical history included a bilateral PE 1 week after giving birth to her last child 5 years ago. She stated having a negative

C, protein S, and antithrombin assays, lupus anticoagulant and anticardiolipin antibodies, and homocysteine level, revealed no abnormalities. The patient was discharged on therapeutic anticoagulation with rivaroxaban. At the 3 week follow-up appointment, the abdominal pain had resolved. Repeat ultrasound examinations at 6 and 12 weeks demonstrated recanalization of both ovarian veins with phasic antegrade flow and no reflux (Fig 3).

hypercoagulable workup at that time. Whether she had a concurrent deep venous thrombosis (DVT) is uncertain. Her surgical history was relevant for a laparoscopic cholecystectomy at age 25. She also reported several nondiagnostic

From the Division of Vascular and Endovascular Surgery, Department of Surgery, Stony Brook University Medical Center. Author conflict of interest: none. Correspondence: Nicos Labropoulos, PhD, Division of Vascular and Endovascular Surgery, Department of Surgery, Stony Brook University Medical Center, Health Sciences Tower, Level 19, Rm 090, Stony Brook, NY 11794 (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the Journal policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 2213-333X Copyright Ó 2017 by the Society for Vascular Surgery. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jvsv.2017.01.016

DISCUSSION OVT is a rare medical condition most often diagnosed in the peripartum period. Hypercoagulability caused by hormonal changes, ovarian vein stasis resulting from compression by an enlarged uterus, and a potential endothelial injury during delivery or from a puerperal infection are responsible for OVT developing in these patients.1 OVT outside of the peripartum period is commonly associated with hypercoagulable state conditions such as malignancy, surgery, trauma, infection, recent hospitalization, or hereditary thrombophilia.3,4,7 OVT with no underlying cause is extremely rare. Only five patients with truly idiopathic OVT have been reported.5,7-10 The patients in these reports had a negative thrombophilia workup and no identifiable risk factors for thrombosis. Two other patients are described in the 567

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Fig 1. Computed tomography (CT) scans of the abdomen and pelvis show enlarged ovarian veins (arrows), thickened walls, and low attenuation vein lumen consistent with ovarian vein thrombosis (OVT).

literature as idiopathic; however, their medical history revealed recent or active use of an oral contraceptive.11,12 Pregnancy-related OVT involves the ROV in w69% to 100% of cases.6,9,10,13,14 Several hypotheses have been proposed for this preponderance. The ROV may be more susceptible to compression during the physiologic dextrorotation by the gravid uterus, suggested first by Brown and Munsick1 in 1971 and supported by others subsequently.6,13 Other potential causes include the presence of numerous incompetent valves and a longer course of the right compared with the left ovarian vein resulting in greater venostasis.13,14 Interestingly, several publications show a similar incidence of left and right OVT in nonpregnant patients.4,14-16 A retrospective analysis of 26 patients with OVT by Gakhal et al14 in 2013 found a higher incidence of left OVT in nonpregnancy OVT (57%) compared with ROV thrombosis (35%), and

bilateral involvement (9%); whereas, isolated ROV thrombosis occurred in all cases with pregnancy-associated OVT. The results of Gakhal et al14 underscore the role of venous stasis caused by external compression as the main theory for the predominance of ROV thromboses in pregnancy-related OVT. A unilateral ROV thrombosis was found in all five patients with idiopathic OVT in previous publications.5,7-10 Our case report is unique: our patient had bilateral idiopathic OVT because after extensive workup, no cause was found. It also occurred 5 years after a remote episode of postpartum PE, thus considered recurrent venous thromboembolism. More importantly, our patient has rigorous clinical and imaging follow-up with detailed information. Fever and focal abdominal pain are the chief complaints for most patients with pregnancy-related OVT and are seen in 80% and 55% of cases, respectively.3,17,18

Fig 2. Ultrasound imaging demonstrates acute thrombosis of the ovarian veins. A, There is absence of color flow in the proximal left ovarian vein (LOV) until its union with the left renal vein (LRV). Normal color flow is seen in the LRV. B and C, Distal LOV diameter measured 0.91 cm and has absence of color flow (arrows). D, High-flow velocities were found in the left ovarian artery caused by the inflammation from the acute thrombosis. E, Normal color flow and no filling defect in the inferior vena cava (IVC). F, Absence of color flow in the proximal right ovarian vein (ROV) to its union with the IVC (arrow). G, ROV diameter is 0.96 cm and has absence of color flow. H, High-flow velocities were found in the right ovarian artery (ROA) caused by inflammation from the acute thrombosis.

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Fig 3. Follow-up ultrasound imaging of the ovarian veins at 6 weeks. A and B, Nearly complete recanalization of the left ovarian vein (LOV; arrow). C and D, Partial recanalization of right ovarian vein (ROV; arrow). Phasic antegrade flow was found in both veins.

Other symptoms include back pain, nausea, vomiting, shortness of breath, and tachycardia.14 A palpable mass is found in some cases.7 Abdominal pain of variable duration (hourse1 week) was the predominant symptom in all five patients reported as idiopathic. Three patients had nausea, and one patient also complained of dysuria. None of the patients were febrile.5,7-10 The differential diagnosis of OVT should include acute pyelonephritis, renal colic, endometritis, ovarian torsion, pelvic inflammatory disease, and acute appendicitis. A diagnosis of OVT can be obtained with the assistance of different imaging modalities. CT venogram is considered the preferred study in the emergency setting given its availability and high accuracy. DUS imaging is noninvasive, quickly available, and the least expensive; however, the ability of diagnosing OVT by DUS imaging is limited by patient habitus, bowel gas, overlying structures, and the experience of the examiner.14 Magnetic resonance imaging is also very good in OVT diagnosis but is time consuming, less available, and has a higher cost. Both DUS imaging and CT scanning provided a proper diagnosis in our patient (Figs 1 and 2).

None of the existing guidelines on DVT or PE tackle the optimal duration of anticoagulation therapy in patients with OVT, thus good clinical judgment is needed in all of these cases. Rottenstreich et al15 suggested 3 months of anticoagulation therapy in pregnancy-related OVT and at least 6 months in all other patients. Wysokinska et al16 proposed that the general treatment guidelines for lower extremity DVT be applied for such patients. These recommendations are based on a retrospective comparative analysis that showed an equal low recurrence rate among OVT (3% per patient-year) and DVT cases (2.2% per patient-year). Patients with concurrent puerperal infection require adjunctive antibiotic therapy until leukocytosis and neutrophilia resolve and they have been afebrile for a minimum of 48 hours.19 Other treatment options, such as direct catheter thrombolysis, IVC filter placement, and surgery, can apply under specific circumstances.17 Complications of OVT include thrombus extension into the IVC or LRV, PE, and death.14 Acute right ureteral obstruction has been reported as well.20 PE may complicate OVT in up to 13% of patients and has a mortality of w4%.2 A complete recanalization is expected to occur in w60% of the veins involved.4

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CONCLUSIONS OVT is a rare disorder commonly associated to the peripartum period. It occurs in the ROV in two-thirds of the patients. Idiopathic OVT and particularly the bilateral type is extremely rare. Acute abdominal pain is generally the initial presenting symptom. A high index of suspicion is essential to establish the diagnosis. Systemic anticoagulation for 3 to 6 months allows for symptom resolution and prevention of life-threatening complications, such as PE, in most cases.

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9. Chebl RB, Krupp S, Bourgi K, Dagher GA. Idiopathic ovarian vein thrombosis in the postmenopausal age. Int J Case Rep Imag 2013;4:611-4. 10. Khishfe BF, Sankovsky A, Nasr I. Idiopathic ovarian vein thrombosis: a rare cause of abdominal pain. Am J Emerg Med 2016;34:935.e1-2. 11. Murphy CS, Parsa T. Idiopathic ovarian vein thrombosis: a rare cause of abdominal pain. Am J Emerg Med 2006;24: 636-7. 12. Doherty K, New M. Idiopathic ovarian vein thrombosis in a nonperipartum patient. Obstet Gynecol 2015;125:1468-70. 13. Kominiarek MA, Hibbard JU. Postpartum ovarian vein thrombosis: an update. Obstet Gynecol Surv 2006;61:337-42. 14. Gakhal MS, Levy HM, Spina M, Wrigley C. Ovarian vein thrombosis: analysis of patient age, etiology, and side of involvement. Del Med J 2013;85:45-50. 15. Rottenstreich A, Da’as N, Kleinstern G, Spectre G, Amsalem H, Kalish Y. Pregnancy and non-pregnancy related ovarian vein thrombosis: clinical course and outcome. Thromb Res 2016;146:84-8. 16. Wysokinska EM, Hodge D, McBane RD 2nd. Ovarian vein thrombosis: incidence of recurrent venous thromboembolism and survival. Thromb Haemost 2006;96:126-31. 17. Prieto-Nieto MI, Perez-Robledo JP, Rodriguez-Montes JA, Garcia-Sancho-Martin L. Acute appendicitis-like symptoms as initial presentation of ovarian vein thrombosis. Ann Vasc Surg 2004;18:481-3. 18. Suleyman T, Gultekin H, Abdulkadir G, Abdulkerim UM, Ali A, Ismail K. Acute right lower quadrant abdominal pain as the presenting symptom of ovarian vein thrombosis in pregnancy. J Obstet Gynaecol Res 2008;34:680-2. 19. Gilbert DN, Chambers HF, Eliopoulis GM, Saag MS, Pavia AT, Black D, et al. The Sanford guide to antimicrobial therapy 2016. 46th ed. Sperryville, VA: Antimicrobial Therapy, Inc; 2016. 20. Toland KC, Pelander WM, Mohr SJ. Postpartum ovarian vein thrombosis presenting as ureteral obstruction: a case report and review of the literature. J Urol 1993;149:1538-40. Submitted Dec 12, 2016; accepted Jan 14, 2017.