Internal Jugular Vein Thrombosis in association with Ovarian

Internal Jugular Vein Thrombosis in association with Ovarian Hyperstimulation Syndrome 花蓮慈濟醫院 蔡祐任

Patient Profile • • • Ms. Tang, 33 y/o woman Hx: DM(-); HTN(-); Asthma(-) ; CVA(-) Denied other systemic disease and family history OBS history: received artificial reproduction technique 6 weeks ago, GA 6 weeks Alcohol(-) Tobacco(-) Good oral hygiene Chinese herbal medicine (+), ceased 3 months

Clinical Presentation Intrauterine insemination 6 -week gestation estradiol 1 mg + progesterone 100 mg twice a day Stimulation regimen 6 weeks ago FSH 50 iu+ LH 75 iu Pregnyl® 5000 iu Unremitting pain and swelling on right side of neck for 2 days Intermittent abdominal pain and mild shortness of breath for 2 weeks Outpatient department visit Numbness and soreness sensation extending from right shoulder to arm 1 day before visit

Clinical Presentation Physical examination: 1. Right lower neck swelling with tenderness 2. Tenderness over right sternocleidomastoid muscle and deltoid 3. No other lesion found in ENT field Neck ultrasonography with color doppler scan: 1. Swelling and thrombus formation at the right jugular vein with loss of flow and compressibility 2. Thrombus formation at the right subclavian vein with loss of flow

Ultrasonogram reveals noncompressable thrombus formation over right internal jugular vein

Color Doppler scan reveals thrombus formation with absent venous flow over right internal jugular vein

Color Doppler scan reveals thrombus formation with absent venous flow over right subclavian vein

Clinical Presentation Physical examination: 1. Right lower neck swelling with tenderness 2. Tenderness over right sternocleidomastoid muscle and deltoid 3. No other lesion found in ENT field Patient developed chest pain and mild dyspnea Admission Neck ultrasonography with color doppler scan: 1. Swelling and thrombus formation at the right jugular vein with loss of flow and compressibility 2. Thrombus formation at the right subclavian vein with loss of flow

Diagnosis • Thrombosis of right internal jugular vein and subclavian vein in association with ovarian hyperstimulation syndrome • Pregnancy, at 6 -week gestation, status post artificial reproduction technique

Clinical Presentation Physical examination: 1. Right lower neck swelling with tenderness 2. Tenderness over right sternocleidomastoid muscle and deltoid 3. No other lesion found in ENT field Patient developed chest pain and mild dyspnea Admission Lab data: 1. No leukocytosis 2. Slightly reduced protein S level, 42. 1% 3. Shortening prothrombin time, 9. 2 sec Neck ultrasonography with color doppler scan: 1. Swelling and thrombus formation at the right jugular vein with loss of flow and compressibility 2. Thrombus formation at the right subclavian vein with loss of flow

Lab data Blood Normal range PT 9. 2 sec 9. 9 -11. 6 sec INR 29. 6 sec 23. 9 -34. 9 sec Protein C 134. 9 % 70 -140 % Protein S 42. 1 % 60 -130 % AT III 95. 2 % 75 -125 % Cardio-Ig. G < 1. 6 GPL U/m. L 0 -20 Cardio-Ig. M 8. 8 MPL U/m. L 0 -12. 5 B 2 GP 1 -Ig. G < 1. 4 U/m. L 0 -20 B 2 GP 1 -Ig. G 2. 6 U/m. L 0 -20 ANA < 1: 40

Enoxaparin 5500 units given subcutaneously twice daily Patient continued pregnancy, and received follow-up at local hospital Discharged Chest pain relieved in 2 days Neck pain resolved over a 5 -day period Gave birth to a set of healthy twins at 37 weeks of gestation No further thromboembolic event or head and neck symptom

Introduction Thrombosis of the internal jugular vein (IJVT) • Rare but potential for serious consequences like pulmonary embolism • Mostly caused by central venous catheterization • May be secondary to malignancy • Other causes includes extensive head or neck sepsis and hypercoagulable state Ovarian hyperstimulation syndrome (OHSS) • A iatrogenic complication occurs in women undergoing ovulation induction • Increased vascular permeability in the region surrounding the ovaries and their vasculature • Symptoms include abdominal pain, dyspnea • Dyspnea, ascites, hypovolemia and hypotension in severe cases • Hypercoagulable state may cause thromboembolic complications • Pathophysiology may includes Factor V Leiden, protein C and concentration of estradiol

IJVT in association with OHSS • Previous study showed OHSS related thromboembolic event mostly occur in upper extremities and neck • 20 cases of IJVT in association with OHSS have been reported by 12 authors from year 2000 to 2018

IJVT in association with OHSS • Initial presentation is mostly neck pain, followed by neck swelling • Mostly occurred in the 1 st trimester of gestation • Ultrasonography tends to be more often used for diagnosing OHSS related IJVT • LMWH and dose-adjusted heparin have been used as treatment while LMWH seems to be more preferable

Conclusion • IJVT associated with OHSS is a rare but emergent condition • History taking and physical examination are the keys towards the diagnosis • Ultrasonography is an efficient diagnostic tool for this disease, especially for otolaryngologist • LMWH or heparin should be used once the diagnose is confirmed to avoid the possible lifethreatening sequalae

Reference • Chan WS. The 'ART' of thrombosis: a review of arterial and venous thrombosis in assisted reproductive technology. Curr Opin Obstet Gynecol. 2009; 21(3): 207 -18. • Schanzer A, Rockman CB, Jacobowitz GR, Riles TS. Internal jugular vein thrombosis in association with the ovarian hyperstimulation syndrome. Journal of Vascular Surgery. 2000; 31(4): 815 -8. • Arya R, Shehata HA, Patel RK, Sahu S, Rajasingam D, Harrington KF, et al. Internal jugular vein thrombosis after assisted conception therapy. Br J Haematol. 2001; 115(1): 153 -5. • Jesudason WV, Small M. Internal jugular vein thrombosis following ovarian hyperstimulation. J Laryngol Otol. 2003; 117(3): 222 -3. • Berker B, Demirel C, Satiroglu H. Internal jugular vein thrombosis as a late complication of ovarian hyperstimulation syndrome in an ICSI patient. Arch Gynecol Obstet. 2004; 270(3): 197 -8. • Ergas D, Elbirt D, Shelanger H, Sthoeger ZM, Levin D, Sokolovsky N. Internal Jugular Vein Thrombosis following Mild Ovarian Hyperstimulation Syndrome in Women with Factor V Leiden Mutation. The American Journal of the Medical Sciences. 2006; 332(3): 131 -3. • Raw DM, Collins MC. Internal jugular vein thrombosis and ovarian hyperstimulation syndrome. Journal of the Royal Society of Medicine. 2007; 100(7): 339 -40. • Alasiri SA, Case AM. Thrombosis of Subclavian and Internal Jugular Veins Following Severe Ovarian Hyperstimulation Syndrome: A Case Report. Journal of Obstetrics and Gynaecology Canada. 2008; 30(7): 590 -7. • Leibman Y, Ayalon M, Steiner IP. Internal Jugular Venous Thrombosis After in Vitro Fertilization. The Journal of Emergency Medicine. 2009; 37(1): 29 -31. • Gong F, Cai S, Lu G. [Jugular vein thrombosis, subclavian vein thrombosis and right brachiocephalic vein thrombosis after in vitro fertilization and embryo transfer: a case report]. Zhong Nan Da Xue Bao Yi Xue Ban. 2011; 36(5): 453 -6. • Fleming T, Sacks G, Nasser J. Internal jugular vein thrombosis following ovarian hyperstimulation syndrome. Australian and New Zealand Journal of Obstetrics and Gynaecology. 2012; 52(1): 87 -90. • Broek Rvd, Balen Mv, Blaauwgeers J, Wolde Mt. A 28 -year-old pregnant woman with a very rare cause of jugular vein thrombosis. The Netherlands Journal of Medicine. 2014; 72(4): 224 -6. • Lee SH, Park JW, Han M, Bae JW, Cho YJ. Internal jugular vein thrombosis with OHSS. J Clin Ultrasound. 2017; 45(7): 450 -2.

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