Successful treatment of a spontaneous rupture of the left external iliac vein in a man

Introduction

Spontaneous iliac vein rupture is a rare cause of retroperitoneal hemorrhage that could be life-threatening. Only 39 cases have been reported so far.^1–6 Primary diagnosis is difficult because neither clinical presentation nor computed tomography (CT) scan images are specific to venous rupture. We report an additional case of left external iliac vein rupture with a successful surgical treatment in a man.

Case report

A previously healthy 73-year-old man presented with a swollen left leg. An initial CT scan revealed thrombosis of the left iliac vein (Figure 1a) with extrinsic compression of the left common iliac vein between the body of L5 and the right common iliac artery (May–Thurner syndrome). The patient was hospitalized and anticoagulant therapy was begun with low molecular weight heparin. The day after, he presented lower abdominal pain and a concomitant brutal syncope episode. His pulse was 115 beats per minute; his blood pressure was measured at 73/51 mmHg. Abdominal examination revealed tenderness and a large non-pulsatile mass in the left lower quadrant. Blood test revealed a hemoglobin level of 9.2 g/dL, hematocrit level of 31.8% and other chemistry parameters were within the normal range. Once the patient was stabilized with a prompt resuscitation, an urgent arterial phase CT scan showed a large hematoma of the left lower abdomen, without any evidence to suggest the location and nature of the bleeding (Figure 1b). Immediate exploratory laparotomy confirmed an extensive retroperitoneal hematoma with a large amount of dark blood coming from the external iliac vessels. At further dissection, a longitudinal 3 cm-rupture was found in the anterolateral wall of the left external iliac vein (Figure 2a). The damaged vein was isolated. After thrombectomy being performed, the vein's wall injury was repaired with continuous 5/0 Prolene suture (Figure 2b). Anticoagulation with low molecular weight heparin was maintained after the surgery, and substituted by fluindione, started at day 4 and stopped 12 months later. Patient's recovery was uneventful and he was discharged home eight days after admission. The left external iliac vein was still patent at two-year-follow-up; the left leg was free of swelling with the help of an elastic stocking. OPEN IN VIEWER

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Figure 2

(a) The ruptured left external iliac vein and (b) the left external iliac vein after completion of the surgical repair

Discussion

Spontaneous rupture of the iliac vein is a very rare entity. To date, 39 cases of spontaneous rupture have been reported in the literature.^1–6 Twenty reported cases concerned the common iliac vein.^1–6 Nineteen cases concerned external iliac vein,^1,5,6 from which only three were men. ¹ Mortality rates are high with 10 reported deaths (25.6%).

In the literature, predominant occurrence of spontaneous venous rupture in middle-aged or elderly women is in favor of unrevealed complications of pregnancy and widely admitted hormonal hypothesis. ¹ As thrombophlebitis weakens the vein wall and loss of estrogen decreases their distensibility, their association can actually be a contributing factor of spontaneous rupture. ¹ However, the pathogenesis of this disease is not fully established. Given left-side preponderance, the mechanical factor theory derives from the chronic anatomical obstruction of the left common iliac vein (Cockett syndrome) concomitant with deep vein thrombosis (May–Thurner syndrome) or thrombophlebitis (79%).^1–5 Additional venous hypertension caused by a Valsalva's manoevre (coughing, defecation, bending) is clear to be a contributing factor. ¹

As the main symptom of rupture is hypovolemic shock, preoperative diagnosis is very difficult. Our patient's hemodynamic status did not allow us further exploratory tests, like venous phase CT scan or venography. As a result, most of the reported cases have undergone surgical exploration, diagnosis, and repair (29/35) or vein ligation (6/35), with a mortality of 28.6% (10/35). Surgical repair of bleeding iliac veins is a treacherous operation, which requires particular skills of vascular surgery.

Only two cases underwent endovascular repair with stentgraft placement, and another one received combined surgical repair and endovascular stent placement.^1,3,5 Presently, these few reports may not be enough to make the endovascular treatment an effective approach in this diagnosis. Furthermore, to be considered, endovascular treatment needs an undoubted diagnosis in a stable patient and some technical issues to be solved, like precise sizing of the endograft and its placement within a thrombus, in a May–Thurner syndrome. However, endovascular approach might be considered as a treatment of the compression syndrome, secondarily.

Two authors have reported four cases of conservatively managed iliac vein ruptures. In one case, reversing anticoagulation, placing an inferior vena cava (IVC) filter and replacing blood products was successful. Three other cases were managed without placing IVC filter, starting blood thinners at 48 hours after the spontaneous retroperitoneal hemorrhage. These patients were hemodynamically stable.^1,5 This approach should only be considered for stable patients with an undoubted diagnosis, and under close observation, but there is no consensus on the management protocol.

In order to avoid systematic open surgery, and let the endovascular treatment show its abilities, patients presenting with lower abdominal pain and hypovolemic shock of sudden unset, in a context of deep vein thrombosis (particularly proximal vein thrombosis), should undergo an emergent venous phase CT scan. It has to be kept in mind that CT scan may not be contributory, only showing a leakage of contrast medium within the hematoma without localizing the source of hemorrhage.

Even if this entity remains extremely rare, it should be known and managed properly in men and women alike.