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Ninewells Hospital and Medical School, Dundee, Scotland, U.K.
J.R.Coll.Edinb., 47, April 2002, 510-511
Spontaneous rupture of the iliac vein is a rare cause of exsanguinating haemorrhage. A case is described of a 66 year old woman who required immediate resuscitation in Accident and Emergency following a 24 hour history of left leg and lower quadrant abdominal pain. Emergency laparotomy revealed a 2 cm tear in the left iliac vein. The previously reported cases are reviewed, together with discussion of the patterns of presentation and possible underlying causes.
Keywords: spontaneous rupture, iliac vein, thromboembolism
The spontaneous rupture of an iliac vein is a rare presentation, and was first reported in 1961.1 Certain similarities can be observed in the eighteen cases described in the literature to date.10 As patients often present in extremis, the initial diagnosis is frequently one of intra-abdominal arterial haemorrhage, necessitating exploratory laparotomy. Early diagnosis, prompt resuscitation and surgical exploration can lead to an excellent outcome.
A 66-year-old, previously healthy woman presented to the Accident and Emergency department with a 24 hour history of worsening pain in her left leg and lower abdomen. Two weeks previously she had suffered a fall, fracturing her right lateral malleolus. This had been treated with a below knee walking cast. Following an episode of vomiting and diarrhorea, symptoms had become more severe, with acute worsening of left lower quadrant abdominal pain.
On initial assessment, she was barely responsive, pale and sweaty. Her blood pressure was 60/30 mmHg and her pulse was regular at 118 per minute. Abdominal examination revealed generalised tenderness and a non-pulsatile mass in the left lower quadrant. Femoral pulses were palpable. The left leg appeared dusky and plethoric.
Owing to difficulties in achieving peripheral venous access, a wide bore rapid infusion cannula was inserted into the right femoral vein, and fluid resuscitation commenced. Her condition improved following the administration of 1 litre of crystalloid and 2 units of O negative blood.
At laparotomy, a large retroperitoneal haematoma was found extending along the left paracolic gutter. A 2 cm tear in the iliac vein was found to be the source of the haemorrhage. Established thrombus forming a cast of the iliac vein lay within the haematoma. Repair of the vein was attempted, but proved impossible due to the friable nature of the vessel wall. The common iliac vein was ligated and the haematoma evacuated. Persistent bleeding, however, necessitated packing of the area. Packs were removed on day two with no further bleeding. Histology revealed a normal thrombus, with no evidence of tumour infiltration of the thrombus.
The post-operative course was complicated by deterioration in respiratory function. A ventilation / perfusion scan suggested a low probability of thromboembolism and the symptoms, attributed to basal atelectasis, responded to supportive care. The patient was anticoagulated with low molecular weight heparin, continued until discharge from hospital. It was decided not to opt for long-term anticoagulation, as this was considered undesirable against a background of peptic ulcer disease. A 3 month follow-up Doppler ultra-sound scan failed to demonstrate any significant thrombus formation.
There appear to have been no significant long-term complications and the patient has resumed her normal activities.
Spontaneous rupture of the iliac vein is a rare, life-threatening event. Only 18 cases have previously been reported.10 There is a discernable pattern between these cases and the one described here. The condition is mainly reported in women (17 vs. 2 in males) with a mean age of 63.4 years (range 41 to 83 years). 9
Rupture is reported more often affecting the left iliac system than the right (17 vs. 2), and appears to be equally frequent in the common and external iliac veins.9 Minor trauma preceded rupture in only three of the cases described, 3,4 Onset after episodes of raised intra-abdominal pressure (such as bending, coughing, defecating or vomiting) is reported in ten cases.6,9 Established thrombus was identified within the venous system at laparotomy in twelve cases.10
Severe haemorrhagic shock was evident in all cases at presentation. Duration of symptoms ranged from 1 to 48 hours.9 In five cases, symptoms were mild initially, with a sudden, severe worsening associated with cardiovascular collapse.4, 5,7,8
While the aetiology of spontaneous iliac vein rupture remains ill-defined, several hypotheses have been proposed.1-10 The only other axis in which spontaneous rupture is described is the portal system, suggesting venous hypertension may play a part.10 In many cases, rupture would appear to have been precipitated by raised intra-abdominal pressure (coughing, bending, defecating etc). It has been suggested that the predominance of left sided rupture may be due to the relatively higher venous pressures caused by compression of the left common iliac vein by the right common iliac artery, a phenomenon known as Crocketts syndrome.10 Histopathological studies have demonstrated weakening of the vessel wall, suggesting rupture may be secondary to previous thrombosis in situ.4,5,8 The combination of hormonal factors and previous obstetric injury may account for the higher numbers of reported cases in women.2
In thirteen cases, vein repair was possible at the initial operation; in the remainder, ligation of the vein was performed. Thromboembolism was the most common reported complication, and long-term anticoagulation therapy was commenced in eleven cases. Anticoagulation may be more important where the surgeon elects to attempt repair rather than ligate the vein. Thirteen of eighteen cases of spontaneous rupture of the iliac vein documented in the literature survived.
Spontaneous iliac vein rupture is an uncommon vascular emergency. The condition should be considered in the differential diagnosis of intra-abdominal haemorrhage, especially in females with left-sided symptoms and with risk factors for venous thrombosis. Prompt resuscitation and early surgical management can lead to an excellent result.
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Copyright: 10 January 2002
Correspondence: D. Pedley, Specialist Registrar in Accident & Emergency, Ninewells Hospital and Medical School Dundee, Dundee DD1 9SY. E-mail: dpedley@doctors.org.uk