Intra-abdominal hemorrhage is one of the most common conditions that general surgeons must deal with in the emergency department. The most common causes are blunt or open abdominal trauma and gynecologic conditions (ruptured follicle or corpus luteum, peritoneal endometritis, complicated ectopic pregnancy)¹. Spontaneous rupture of the iliac vein is a rare cause of fatal retroperitoneal hemorrhage that is generally associated with trauma and iatrogenic injury during lower abdominal or pelvic surgery, endovascular interventions or placement of central venous accesses. It is more common in the left common and external iliac veins. The first case was described in 1961 and, at present, less than 60 cases have been published². Although they all share certain common characteristics, their incidence is so low that morbidity and mortality rates are still high.

We report the case of a 66-year-old female patient with a history of hypertension and type 2 diabetes mellitus who consulted for continuous abdominal pain vague and moderate in intensity in the left iliac fossa lasting two hours that started suddenly while she was having lunch. She denied history of trauma.

On physical examination, the patient was fully conscious, alert, and oriented (Glasgow Coma Scale 15/15); blood pressure was 120/80 mm Hg, heart rate was 90 beats per minute and ventilation was adequate. The abdomen was symmetrical, distended, soft, depressible, tender on deep palpation in the left iliac fossa, without guarding or rebound tenderness. The laboratory tests showed hemoglobin level 5.1 g/dL (reference value 11-13 g/dL), white blood count 15.6 × 103 (reference value 4-10 × 103, with neutrophilia), lactic acid 5.16 mmol/L (reference value 0.5-2.2 mmol/L) and prothrombin time 39% (reference value 70-120%); the acid-base balance revealed compensated metabolic acidosis. Three units of packed red blood cells were transfused. As the patient was hemodynamically stable and intra-abdominal hemorrhage was suspected, she underwent contrastenhanced multislice computed tomography scan of the chest. A large left retroperitoneal hematoma with extension to the ispsilateral pelvis was observed. The hematoma displaced left kidney anteriorly and ran between the iliac vessels (26 × 11 × 9 cm in diameter) (Fig. 1). There was no contrast media extravasation in the arterial phase.

Figure 1 Multislice computed tomography in the axial (A) and coronal (B) plane, arterial phase, showing a large pelvic hematoma running between the left iliac vessels (white arrow; the black arrow indicates the left external iliac artery). 

Immediately after the test, the patient presented pallor, sweating, somnolence, tachycardia, and hypotension unresponsive to aggressive resuscitation with blood products and crystalloid solutions. An emergency laparotomy was performed. A large, non-expandable, left retroperitoneal hematoma was visualized in zone III (iliac vessels). The left retroperitoneum was accessed using the Mattox maneuver. After the hematoma was completely evacuated, a 3-cm longitudinal lesion was found in the anterolateral aspect of the external iliac vein. The proximal and distal portions of the vein were checked and the defect was closed with nonabsorbable monofilament suture (Fig. 2) with adequate hemostasis.

Figure 2 Intraoperative image showing nonabsorbable monofilament suture (arrowhead) on the left anterolateral aspect of the left external iliac vein. The white arrow indicates the lateral displacement of the external iliac artery 

The patient was immediately admitted to the critical care unit; she was hemodynamically unstable with requirement of high doses of vasoactive drugs and inotropic agents, and mechanical ventilation. Despite the measures implemented, she persisted in refractory hemodynamic shock and permanent lactic acidosis and died 24 hours after surgery. Spontaneous rupture of the iliac vein is a rare condition. It usually occurs in women with a mean age of 63.4 years² and presents as hypovolemic shock lasting from 1 to 48 hours³. Different theories have been proposed to explain the etiopathogenesis of the disease, including mechanical, inflammatory and hormonal factors^4,5.

In our case, we think that mechanical factors (intimal injury caused by hypertension and diabetes mellitus) and hormonal factors are the main causes of spontaneous iliac vein rupture. None of the proposed theories can solely explain the phenomenon. In line with the publication by Cho et al.⁶, we believe that the combination of loss of vascular compliance with increased intraluminal pressure and wall weakening potentially leads to vein rupture. In addition, in patients with deep vein thrombosis some cases have been triggered by sudden increase in intra-abdominal pressure after defecation, vomiting or coughing³.

High level of suspicion is essential to detect this condition and perform early diagnosis and treatment to improve the associated morbidity and mortality. Tannous et al. described a postoperative mortality rate of 71%³. Multislice compute tomography angiography of the abdomen and pelvis is the gold standard imaging test for the diagnosis of this condition and to rule out other differential diagnoses⁶. Color-Doppler ultrasound may be useful in emergency cases and when iliofemoral deep vein thrombosis is clinically suspected or confirmed due to patients’ history (due to its common association). Abdominal cavography with phlebography of iliac vessels may also be useful and is also a therapeutic approach in selected cases. Adequate resuscitation is a priority for reverting shock, which is usually a common presentation. The type of treatment will depend on patient’s hemodynamic status and the characteristics of the venous injury (extent, vein wall disease or presence of associated thrombosis). In the series of 53 cases reviewed by McCready et al.², the following treatments were reported:

▪ Primary surgical repair in 36 cases: some patients underwent concomitant thrombectomy, iliac vein stenting, or inferior vena cava filter placement.

▪ Conservative therapy in 6 cases (observation, anticoagulants).

▪ Primary endovascular repair with inferior vena cava filter placement in 4 cases.

▪ Ligation of the iliac vein injured in 4 cases.

▪ Concomitant Palma-Dale bypass or lower extremity venous thrombectomy to treat associated phlegmasia in 2 cases.

▪ Evacuation of retroperitoneal hematoma only in 1 case.

Surgical treatment should be performed in unstable patients or when conservative treatment has failed. These measures include primary suture, ligation and exclusion of the vessel (according to damage control surgery strategy and depending on the severity of the vessel injured), postponing venous grafting (with prosthesis, total or partial autologous graft with saphenous vein patch in case of lateral lesion, or heterologous graft) according to patient’s clinical course. These procedures are associated with high rates of postoperative thrombosis².

In selected cases of hemodynamically stable patients or in the presence of non-expansive hematomas of up to 5 cm in diameter, an endovascular strategy with placement of covered stents is feasible⁵. As McReady et al. reported, laparotomy and primary repair is the most common treatment used for spontaneous iliac vein rupture². Spontaneous rupture of the iliac vein is a rare vascular emergency. It should be considered in the differential diagnosis of female patients in the 6th decade of life with syncope of unknown origin preceded by abdominal pain. Hemodynamic stabilization and emergency surgical or endovascular treatment are essential to achieve an effective resolution of this condition.