Management of Phlegmasia Cerulea Dolens Caused by a Giant Leiomyoma

This case is of a young female with a large uterine leiomyoma causing phlegmasia cerulean dolens with thrombosis of the left common and left external iliac veins. She underwent mechanical thrombectomy to temporize the condition until she could be evalu- ated by gynecology-oncologist to remove the cause of venous ob- struction. Prior to hysterectomy, suprarenal inferior vena cava filter was placed. Less than 12 hours post hysterectomy she developed recurrent thrombosis involving the left common and external iliac veins. She underwent repeat mechanical thrombectomy with wall stent placement in the left common iliac vein with resolution of her symptoms.

Phlegmasia; Cerulea dolens; Giant leiomyoma; Throm- bectomy

Uterine leiomyomas are the most common pelvic tumor, found in pre-menopausal women [1]. There have been reports of leiomyomas causing compression of the right common iliac vein and subsequent development of Deep Vein Thrombosis (DVT). We report a young female who presented with phlegmasia cerulean dolens secondary to compression of the left common iliac vein by a large uterine leio- myoma. She underwent mechanical thrombectomy to temporize her condition prior to total abdominal hysterectomy. Informed consent was obtained for all procedures and publication of this case report, including all accompanying images.

A 46 year old female presented to the Emergency Department with complaint of left lower extremity pain of two days duration. Swelling originated around the ankle with progression to the groin. Medical history was significant for oral contraceptive use and a known uterine leiomyoma, approximately 10cm at last evaluation. Physical exam re- vealed left lower extremity swelling extending from foot to mid-thigh with erythematous discoloration of skin (Figure 1). Biphasic doppler signals in left posterior tibial and dorsalis pedis arteries were present with triphasic signals on the right side. Motor and sensory function was intact in both lower extremities.

Duplex venous imaging showed extensive acute DVT of the left common femoral, femoral, popliteal, gastrocnemius, posterior tibi- al, peroneal, and great saphenous veins. The left External Iliac Vein (EIV) close to the junction with the Common Femoral Vein (CFV) showed evidence of thrombosis. However, the left Common Iliac Vein (CIV) could not be evaluated with duplex venous imaging. Computed Tomography (CT) pulmonary embolism protocol showed right lower lobe segmental and subsegmental emboli. CT venogram of abdomen and pelvis showed a large heterogenous mass which measured 18.6 x 15.5 x 9.9cm (Figure 2). Mass effect was noted on the Inferior Venacava (IVC) and left CIV with evidence of thrombus in the left EIV, Internal iliac vein, and CIV. Ankle-brachial indices were not obtained. She was started on intravenous heparin.

Figure 1: Left lower extremity swelling and skin color changes significant for phleg- masia cerulean dolens.

Figure 2: Left, CT coronal view, Right, CT axial view of 18.6v15.5x9.9cm pelvic mass causing mass effect on pelvic structures.

A left lower extremity venogram with access through the left pop- liteal vein, demonstrated thrombus within left femoral and common femoral veins and chronic near complete occlusion of the left EIV and the entire portion of the left CIV. A 30cm infusion length UniFuse catheter (Queensbury, N.Y.) was placed with the treatment area from proximal IVC to the distal femoral vein. Overnight, thrombolysis was initiated with Tissue Plasminogen Activator (tPa).

The Infusion catheter was removed after 12 hours. Left lower ex- tremity venogram demonstrated persistent thrombus within the left femoral vein. There was persistent lack of flow within the distal left external iliac vein and the left CIV.

Angioplasty, with a 4 x 40mm Armada balloon (Abbott Park, IL.) was performed within the IVC, left CIV and EIV for purposes of pre-dilatation prior to mechanical thrombectomy with the Inari, a 105cm x 16mm Clottriever (Irvine, CA.), in order to gain access into the lumen. Mechanical thrombectomy of the proximal IVC, left CIV, EIV, common femoral and femoral veins with retrieval of a large amount of acute, sub acute and chronic appearing clot (Figure 3).

A completion venogram demonstrated complete removal of the thrombus within the left femoral, common femoral and the visualized portions of the proximal EIV. Flow was present within the left CIV with contrast seen entering the IVC. There was chronic stenosis of the left CIV and EIV secondary to mass effect of the uterus.

Figure 3: Top left, imaging after tPa, prior to mechanical thrombectomy. Top right, Imaging after mechanical thrombectomy. Bottom, thrombus extracted using Inari device.

A suprarenal Option-Elite IVC filter (Athens, TX.) was placed pri- or to surgery for excision of pelvic mass. Systemic heparin was held 6 hours prior to surgery. She underwent total abdominal hysterectomy with bilateral salpingectomy, with preservation of normal ovaries. Pa- thology showed the mass to be a large benign leiomyoma measuring 18x 18cm.

Approximately eight hours postoperatively the patient started complaining of recurrent left calf tightness and swelling. Increased pitting edema with palpable pulses, compartments were soft and no pain with passive range of motion. Repeat US duplex showed acute DVT of left common femoral, femoral, posterior tibial, and peroneal veins.

Venogram showed diffuse thrombosis of the left femoral, common femoral, external and common iliac veins with high-grade stenosis involving the common iliac vein. Mechanical thrombectomy was repeated with the Inari Clottriever. The venogram did not show re- sidual thrombus. Intravascular ultrasound demonstrated an 80% ste- nosis involving the mid to distal left CIV that was likely missed on prior imaging due to overlying thrombus. Balloon angioplasty with a 14mm Atlas balloon was performed within the common iliac vein, followed by placement of a 16 x 120mm Boston Scientific Vici stent (Marlborough, MA.). Completion venogram demonstrated adequate inflow and outflow through the left common iliac vein stent with no significant residual thrombus or stenosis (Figure 4).

Figure 4: left common iliac vein stent.

Her post-operative course was complicated by adynamicileus that resolved with conservative management. She required blood transfu- sion due to steady drop in her hematocrit. CT arteriogram of abdo- men and pelvis showed large pelvic hematoma with extravasation. However, angiography failed to show active bleed, likely caused by missed bleed during hysterectomy. Anticoagulation was held until he- moglobin stabilized. She was discharged on hospital day 15 on oral Apixaban 5mg twice daily. Her left lower extremity edema had much improved, (Figure 5). She returned two months later and had the IVC filter retrieved. Follow up office visit three months later showed pa- tient left CIV and EIV stent.

Phlegmasia Cerulea Dolens (PCD) is a precursor of venous gan- grene. It is a limb and life threatening condition with a mortality of 20-40%, and 30% of those deaths caused by pulmonary embolism [2]. DVT is diagnosed in about two million Americans every year, but PCD is rare. PCD is also known as “milk leg” due to its association with third trimester pregnancy and postpartum state, even though it is more prevalent in men with a ratio of 1.5:1 [3]. Patients present with pain followed by significant swelling and cyanosis secondary to collateral vein involvement, causing massive fluid shift into interstitium, obstruction of arterial flow, and ultimately capillary involvement leads to venous gangrene [4].

Figure 5: Left lower extremity on hospital discharge.

May-Thurner Syndrome (MTS), is compression of the proximal segment of the left CIV by the right common iliac artery, it is rarely the differential for acute DVT, although can be associated with chron- ic thrombus. The most common variant found in about 20% of the population is the compression of the left iliac vein by the right iliac ar- tery against the lumbar spine causing collagen deposition in the vein known as venous spurs [5]. Consequential venous stasis increases the risk of DVT.

Risk factors for MTS overlap with the risk factors for DVT. These include female gender, especially those using oral contraceptives or in the postpartum period, scoliosis, and thrombophilias. This case did not involve this anatomic defect of compression of iliac vein by iliac artery, but instead was caused by the direct compression of left iliac vein by enlarged fibroid uterus.

Patients with DVTs are generally placed on systemic anticoagu- lation to prevent further thrombosis. In this case, catheter directed thrombolysis as well as mechanical thrombectomy was utilized until hysterectomy with bilateral salpingectomy was performed, address- ing the etiology of this extensive DVT. There have been several case reports of uterine mass causing occlusion to venous flow. However, it is difficult to effectively treat the phlegmasia and venous thrombosis without treating the underlying cause, such as pelvic mass.