CASE REPORTS

A spontaneous rupture of the external iliac veinrevealed as a phlegmasia cerulea dolens with acutelower limb ischemia: Case report and review of theliteratureSaed Jazayeri, MD,a Etienne Tatou, MD, PhD,a Nicolas Cheynel, MD,b Francois Becker, MD, PhD,a

Roger Brenot, MD,a and Michel David, MD,a Dijon, France

We reported a case in a previously healthy 45-year-old woman of spontaneous rupture of the left external iliac vein, whichwas revealed as a phlegmasia cerulea dolens with acute lower limb ischemia. A tear on the anterior surface of the vein wasdiscovered during an emergency laparotomy, and the rent was repaired successfully. Twenty-four hours later, the legswelling increased, and at the reexploration, the common iliac vein was found to be occluded by an organized thrombusas the result of a well-endothelialized membranous band (Cockett or May-Turner syndrome). A Palma-Dale operation(crossover saphenous bypass grafting) was performed secondarily. The swelling in the leg diminished slowly, and thepatient was discharged 10 days later with an oral anticoagulant therapy and elastic stockings. (J Vasc Surg 2002;35:999-1002.)

The most frequent cause of lower limb ischemia isarterial occlusion. A true phlegmasia cerulea dolens withacute ischemia of a lower limb provoked by a spontaneousrupture of the iliac vein, without any abdominal sign, hasyet to be reported. An iliac vein rupture is rare and primarilyresults from major trauma or occurs during pelvic surgery.Spontaneous rupture of the iliac vein is even more unusual;only 20 cases have been reported worldwide. We reporthere a unique case of spontaneous rupture of an iliac veinwith an acute lower limb ischemia. Prodromal symptoms,etiology, and treatment are discussed. All previously re-ported cases are also reviewed.

CASE REPORT

A previously healthy 45-year-old female nurse was brought tothe cardiology department in the hospital in which she workedbecause of sudden onset of syncope and lower limb swelling withacute left lower limb pain and a suspected pulmonary embolism.She had no history of trauma and no other particular pathology.However, the day before she was admitted, she had walked in thewoods for several hours collecting mushrooms. She called her

physician in the morning because of a painful sensation in her leftleg. The first diagnosis made at that time was sciatic pain. Later thatmorning, while she was showering, she had a sharp pain in the leftlower limb and she collapsed. The mobile emergency unit wascalled.

The patient had low blood pressure and a swelling of the leftlower limb. A pulmonary embolism was suspected, and a throm-bolytic treatment should have been started but was not because theblood pressure rose to 85/45 mm Hg. The patient asked to betransported to the cardiology department in the hospital in whichshe worked for this treatment. On arrival at the cardiology depart-ment, she was conscious but pale and clammy. Blood pressure was80/40 mm Hg. The femoral pulses were present and equal, butthe left lower limb was cold and cyanic with a sensiomotor defi-ciency. A transthoracic echography was performed, and the resultsdid not show any sign of major pulmonary embolism. Therefore,the patient was transferred to the cardiovascular department withsuspicion of acute ischemia of the left lower limb, or a phlegmasiacerulea dolens.

The patient underwent peripheral arterial and venous colorduplex ultrasound scan study. The results revealed no flow in theleft iliac vein and normal arterial flow in the left femoral artery,except for a sharp Doppler scan signal characteristic of high distalresistance in arterial circulation. A hypoechoic and heterogeneousmass was also noted in the left iliac fossa, but it was thought to bea neoplasic mass at that time. The patient was taken to theoperating room with a diagnosis of phlegmasia cerulea dolens. Inthe operating room, after the general anesthesia was administered,the blood pressure fell to 60/30 mm Hg and the heart rateincreased to 120 beats per minute. The laboratory examination

From the Service de chirurgie Cardio-Vasculaire et d’angiologie CHU deDijona and the Service de chirurgie Viscerale, CHU de Dijon.b

Competition of interest: nil.Reprint requests: Dr Saed Jazayeri, Service de chirurgie Cardio-Vasculaire, 2

Bd Marechal de Lattre de Tassigny, BP 1542, 21034 Dijon, France(e-mail: saed.jazayeri@chu-dijon.fr).

Copyright © 2002 by The Society for Vascular Surgery and The AmericanAssociation for Vascular Surgery.

0741-5214/2002/$35.00 � 0 24/4/121569doi:10.1067/mva.2002.121569

999

revealed a hemoglobin level of 7 g/dL and a hematocrit level of22%. The abdominal examination results then revealed a huge,tender, nonpulsatile mass in the left iliac fossa. An internal hemor-rhage was suspected. The patient was resuscitated with a rapidintravenous infusion of colloid fluids, and an emergency laparot-omy was performed. An enormous retroperitoneal hematoma thatextended from the infrarenal aorta to the left iliac fossa was noted.Clamping the aorta just below the diaphragm stabilized the bloodpressure. The exploration of the hematoma showed a normalarterial system. Evacuation of the retroperitoneal hematoma,which contained large amounts of clotted venous blood, revealed alongitudinal 3-cm linear rent on the anteromedial side of the leftexternal iliac vein. The edges of the tear were smooth, and the veinwas macroscopically normal. There was no clot inside the venouslumen. The damaged vein was repaired without any clamp (afterapplication of a pressure on each side of the tear) with a continuous6/0 prolene suture (Ethicon, Inc, Somerville, NJ).

Twenty-four hours after surgery, the swelling of the left leghad extended up to the thigh. A venous color duplex ultrasoundscan study was performed, and results showed the occlusion of theleft femoral and the left iliac vein without collateral pathway.

The patient underwent operation, and a thrombectomy wasperformed. A Fogarty catheter was introduced into the left femoralvein, but it was blocked in the left common iliac vein. The explo-ration of the left common iliac vein showed that an intraluminalorganized thrombosis occluded the vein. The presence of theintraluminal web or spur was noted after the removal of thethrombus. Direct repair was not possible because of the structuralabnormalities of the vein. Because replacement of the vein with avascular prosthesis seemed to be difficult, we decided to perform acrossover saphenous vein bypass grafting procedure (Palma-Daleprocedure) with the right saphenous vein to deviate the venousflow of the left lower limb to the right femoral vein, with the

creation of an arteriovenous fistula between the saphenous veinand the left common femoral artery (Fig).

The postoperative course of these operations was uncompli-cated, and the swelling in the leg diminished slowly. The patientwas discharged 10 days later, with an oral anticoagulant therapy. Thepathologic examination of the common iliac vein showed the pres-ence of an organized thrombus and structural modification of thevein with the presence of fibrosis and inflammatory changes. Otherlaboratory explorations did not show a hypercoagulable profile.

DISCUSSION

Spontaneous rupture of the iliac vein is a rare event,with only 20 cases reported to date in the internationalliterature. All of the cases, including the one reported here,indicate a predominance of this condition among whitewomen between 41 and 83 years of age (average age, 63years). All patients except two were female with no historyof trauma. Sudden onset, hypotension, and abdominaldistension with a nonpulsatile mass in the iliac fossa are thesymptoms found in all the cases. The Table summarizeseach patient’s symptoms and the physician’s findings andmanagement that were reported in those 20 cases. Ourfindings are also summarized in the Table.

The rupture occurred on the left side in 19 cases, with11 cases in the left external and eight cases in the commoniliac vein. Only two cases on the right side have beenreported.1,2 Ischemia of the left lower limb was noted infour other cases,3-6 and a lower limb swelling occurred inthree cases.7-9 In our case, the patient had an acute ischemiaof the lower limb with a sensiomotor deficiency withoutabdominal pain. Because of these symptoms, the patientwas sent to the operating room with a diagnosis ofphlegmasia cerulea dolens.

The Palma Dale procedure with arteriovenous fistula.

JOURNAL OF VASCULAR SURGERYMay 20021000 Jazayeri et al

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JOURNAL OF VASCULAR SURGERYVolume 35, Number 5 Jazayeri et al 1001

The cause of these spontaneous venous ruptures re-mains obscure. A histopathology examination was per-formed only in seven cases. Inflammatory changes werenoted in five of these cases,1,10-12 recent phlebitis with nostructural changes was noted in one case,4 and no abnor-malities were noted in another.13 It is important to stressthat the origin of histopathology samples in these last twocases is not certain. In these cases, the samples couldpossibly have been obtained from the external iliac vein,thus not showing any inflammatory changes.

The presence of a fresh thrombus in the iliac vein wasnoted in 15 cases during surgery, and in all other cases butone,13 a thrombophlebitis was diagnosed after surgery. Inthis one case, the patient died 4 hours after the operation.In our opinion, because of the death of the patient so soonafter surgery, the thrombophlebitis could not be diag-nosed. Limb swelling increased during the first 48 hoursafter surgery in six cases,2,9,14-16 and in all these cases, theduplex ultrasound scan or venographic scan results showeda thrombophlebitis in the common iliac vein.

Proximal obstruction of the left common iliac vein wasidentified as a result of the overlying right common iliacartery in two cases5,7 or of an intraluminal endothelializedband in three cases.6,12 Because the rupture had occurredon the left in 19 cases (90%), proximal venous obstructionby the overlying right common iliac artery or the endolu-minal spur (Cockett or May-Turner syndrome) could be acontributing factor.

A predisposing factor like bending,2,5,17,18 defeca-tion,6,8,13,19 or postpartum10 could raise the intralumi-nal pressure in a segment of the vein between the ingui-nal ligament and the right common iliac artery. Thiscould cause a tear if only the venous wall had previouslybeen weakened (as we know that a vein can be used as anarterial substitute without problem). Such a weakeningcould be caused by thrombosis and inflammation as theresult of the structural abnormalities.

Because extensive preoperative investigation is not al-ways possible, the clinical picture of spontaneous ruptureusually mimics a ruptured abdominal aneurysm2,5,6,8,16,18,19

or an intraabdominal hemorrhage of unknown cause.17

Treatment depends on prompt resuscitation and early con-trol of bleeding. The vein can be tied1,4,12 (proximal anddistal to the tear), or, perhaps more favorably, the rent canbe repaired (all other cases). Four patients died after sur-gery: two after vein ligation and two after vein repair. In thepreviously reported cases, vein repair was associated withthrombectomy in five cases3,7-9,19 or with implantation of avena cava clip or filter in two cases.6,11 We believe that evenif a thrombectomy is performed, leg swelling could appearor increase after surgery because of obstruction of thecommon iliac vein.

In our case, the ultrasound scan results showed com-plete obstruction of the venous iliac axis without collateralpathway. As a result, we performed a Palma-Dale bypass

grafting procedure (crossover saphenous vein bypass graft-ing) with an arteriovenous fistula. We think that eventemporary patency of the venous circulation may preventchronic venous insufficiency by allowing time for collateralvenous channels to develop.

Postoperative anticoagulation treatment is recom-mended. Leg elevation, bandages, and mobilization arehelpful in postoperative care to decrease the limb swellingthat usually appears or increases after surgery. Oral antico-agulation therapy, elastic stockings, and physiotherapycould be necessary for several months thereafter.

CONCLUSION

Although rare, the diagnosis of spontaneous rupture ofthe iliac vein should be considered, particularly in femalepatients with sudden onset of lower abdominal pain andhypotension. Prompt resuscitation and repair of the veingive excellent results in an otherwise fatal condition.

REFERENCES

1. Hossne WS, Nahas PS, Vasconcelos E. Spontaneous rupture of the iliacvein: acute abdomen. Arq Circ Clin Exp 1961;24:27-30.

2. Elliot D, Ware CC. Spontaneous rupture of the external iliac vein. J RSoc Med 1982;75:477-8.

3. Herrin BJ, Osborne P, Diste A, et al. Spontaneous rupture of an iliacvein. Vasc Surg 1975;9:182-4.

4. Estevan Solano JM, Garcia-Pumarino JL, Pacho Rodriguez AJ, et al.Ruptura idiopatica de vena iliaca. Angiologia 1982;34:136-9.

5. Noszczyk W, Orzeszko W. Spontaneous rupture of the left iliac vein.Arch Surg 1983;118:1227.

6. Van Damme H, Hartstein G, Limet R. Spontaneous rupture of the iliacvein. J Vasc Surg 1993;17:757-8.

7. Stock SE, Gunn A. Spontaneous rupture of the iliac vein. Br J Surg1986;73:565.

8. Nishida S, Billings PJ, Walker RT, et al. Spontaneous rupture of the leftexternal iliac vein: a case report. J Jpn Surg Soc 1993;94:424-6.

9. Yamada M, Nonaka M, Murai N, et al. Spontaneous rupture of the iliacvein: report of a case. Surg Today 1995;25:465-7.

10. Herczeg B, Karpathy L, Kovecs G, et al. Spontaneous rupture of theiliac vein with lethal hemorrhage. Zentralbl Chir 1967;92:552-5.

11. Brown L, Sanchez F, Marrix H. Idiopathic rupture of the iliac vein. ArchSurg 1977;112:95.

12. McDonald RT, Vorpahl TE, Caskey J. Spontaneous rupture of the iliacvein. Vasc Surg 1980;14:330-3.

13. Hill S, Billings PJ, Walker RT, et al. True spontaneous rupture of thecommon iliac vein. J R Soc Med 1990;83:117.

14. Copeland GP, Saw Y, Monk D. Spontaneous rupture of the left com-mon iliac vein. J R Coll Surg Edinb 1989;34:165.

15. Majeed SMK, Phelis PJ. Spontaneous rupture of the left external iliacvein. Br J Clin Pract 1993;47:109-10.

16. Plate G, Qvarford P. Idiopathic rupture of the iliac vein. Eur J Surg1995;8:611-2.

17. Lin BC, Chen RJ, Fang JF, et al. Spontaneous rupture of the leftexternal iliac vein: case report and review of the literature. J Vasc Surg1996;24:284-7.

18. De Passe IE. Spontaneous common iliac vein rupture: a case report. CanJ Surg 1998;41:473-5.

19. Frosberg JO, Bark T, Lindholmer C. Nontraumatic rupture of the iliacvein. Eur J Vasc Surg 1988;2:267-8.

Submitted Apr 4, 2001; accepted Oct 30, 2001.

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