Coil embolization of multiple hepatic arteryaneurysms in a patient with undiagnosedpolyarteritis nodosaGlenn W. Stambo, MD,a Michael J. Guiney, MRCP, FF(RCSI), FRCR,b Xavier F. Cannella, MD,c andBernard F. Germain, MD,d Tampa, Fla; and London, United Kingdom

Hepatic aneurysms are a rare sequela of vascular abnormalities in the liver, including trauma, infection, necrotizingvasculitis such as polyarteritis nodosa (PAN), and iatrogenic and arterial mediolysis. Presentation with intra-abdominalhemorrhage is associated with a high mortality rate. We describe life-saving transcatheter coil embolization of multipleisolated ruptured hepatic pseudoaneurysms in a patient with no history or clinical findings of PAN. We presentangiographic findings and intra-arterial transcatheter embolization techniques in the treatment of ruptured large hepaticartery aneurysms. Endovascular specialists should recognize that PAN could present with classic angiographic findingsand, in some cases, as life-threatening ruptured isolated hepatic artery aneurysms as its first presentation. (J Vasc Surg2004;39:1122-4.)

Isolated pseudoaneurysms of the hepatic arteries in thesetting of polyarteritis nodosa (PAN) are extremely rare andtypically asymptomatic. The first presentation of PAN canbe life-threatening intra-abdominal hemorrhage from rup-tured visceral pseudoaneurysms, with a mortality of 20% to70%.1 Although endovascular treatment for hepatic arteryaneurysms in the setting of PAN has previously been de-scribed by Herskowitz et al,2 to our knowledge, we presentthe first description of life-saving coil embolization of mul-tiple acutely ruptured hepatic artery aneurysms in a patientpreviously undiagnosed with PAN. In this report, wepresent the angiographic findings of PAN along with theendovascular treatment of visceral pseudoaneurysms.

CASE PRESENTATION

A 64-year-old woman with a past medical history of uncon-trolled hypertension on four antihypertensive medications pre-sented emergently to the hospital with 24-hour history of epigas-tric and midabdominal pain, with a blood pressure of 180/100.The patient underwent a non-contrast computed tomography scanof the abdomen and pelvis, which demonstrated high-density fluidsurrounding the liver and spleen. The patient was brought directlyto the operating room, with high suspicion for a perforated viscus.Surgical exploration was performed and demonstrated hemoperi-toneum with hematoma in the hepatoduodenal ligament. Therewas no perforated viscus and the visceral organs were grossly

From the Division of Vascular and Interventional Radiology, Department ofRadiology, St. Joseph’s Hospital Medical Center,a Tampa, the Division ofVascular and Interventional Radiology, University College London Hos-pitals Trust,b and the Divisions of Vascular Surgeryc and Rheumatology,d

University Community Hospital, Tampa.Competition of interest: none.Reprint requests: Glenn W. Stambo, MD, Division of Vascular and Inter-

ventional Radiology, Department of Radiology, St. Joseph’s HospitalMedical Center, 4516 N. Armenia Avenue, Tampa, FL 33603 (e-mail:xraydoc2@yahoo.com).

0741-5214/$30.00Copyright © 2004 by The Society for Vascular Surgery.doi:10.1016/j.jvs.2004.01.018

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normal. An intraoperative ultrasound demonstrated grossly nor-mal hepatic, splenic, and gastroduodenal arteries. No source ofactive bleeding was identified during surgical exploration.

An emergent mesenteric arteriogram was obtained immedi-ately following the exploratory laparotomy to define the source ofthe hemorrhage. An abdominal aortogram was performed with a5F Pigtail catheter (Angiodynamics, Queensbury, NY). Selectivecannulation of the superior mesenteric artery (SMA) and commonhepatic arteries was performed with a 5F Cobra glide catheter(Terumo/Boston Scientific, Natick, Mass), which demonstratedtwo large hepatic artery aneurysms, measuring 2 � 1.5 cm and 1.5� 1.5 cm, arising from the left hepatic artery (Fig 1). There wasactive extravasation of contrast from the most proximal left hepaticartery aneurysm consistent with acute hemorrhage. A secondequal-sized aneurysm located distal to the first was also identified.A replaced right hepatic artery arising from the SMA demonstratedmultiple smaller aneurysms of variable size with associated multi-focal stenoses and ectasia within the distribution of the vessel (Fig2). No aneurysms arising from the replaced right hepatic arterywere actively extravasating at this time. The remaining visceralvessels demonstrated no aneurysms or vascular irregularities.

A 5F Cobra glide catheter was advanced into the left hepaticartery. A subselective microcatheter (Target Therapeutics/BostonScientific, Fremont, Calif) was advanced into the neck of theproximal hepatic artery aneurysm. Brisk extravasation of contrastwas identified from this aneurysm (Fig 3). Multiple helical coils(Target Therapeutics/Boston Scientific) were then packed withinthe neck of the proximal hepatic artery aneurysm. Immediateocclusion of the ruptured aneurysm was documented, with nofurther active extravasation or visualization of the aneurysm. Theadjacent hepatic artery aneurysm was then evaluated, and althoughit was not actively extravasating at this moment, its appearance wassimilar to the previously embolized aneurysm. Therefore, the neckof this aneurysm was also embolized with helical coils (TargetTherapeutics/Boston Scientific), with no further visualization ofthis aneurysm (Fig 4). A total of six 2 � 10-mm microcoils (TargetTherapeutics/Boston Scientific) were deployed during this proce-dure. Following embolization, there was no further evidence of

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bleeding. A rheumatology consult was called in light of the multi-ple hepatic artery aneurysms and irregularity of the hepatic arteryvascularity. An elevated C4 complement 74 (16-47mg/dL), Creactive protein 13.5 (0.0 –0.9), and erythrocyte sedimentationrate 30 (0-20) were documented on laboratory values, whichhelped reinforce our diagnosis of PAN. The patient was started onhigh-dose prednisone and cytoxan.

Fig 1. Selective left hepatic arteriogram shows two large hepaticartery aneurysms. Note the active extravasation from the mostproximal aneurysm (single arrow).

Fig 2. Selective superior mesenteric arteriogram shows a replacedright hepatic artery with multiple aneurysms of variable size arisingoff the intrahepatic branches containing luminal irregularities withmultifocal stenoses and vascular ectasia (double arrows).

The patient was discharged from the hospital on postoperativeday number 6 in good condition. She has done well clinically, withroutine visits with her rheumatologist as an outpatient for over twoyears and no further evidence of bleeding or other clinical sequelaeof PAN. During her acute presentation and over the last two years,no liver or muscle biopsies have been performed to evaluate forPAN activity. Also, there has been no further radiologic imaging

Fig 3. Subselective angiogram via a microcatheter in aneurysmneck shows brisk contrast extravasation from the most proximal lefthepatic artery aneurysm (single arrow).

Fig 4. Successful coil embolization of both left hepatic arteryaneurysms with no further active contrast extravasation or filling ofthe aneurysms with sparing of the native vessel (double arrows).

JOURNAL OF VASCULAR SURGERYMay 20041124 Stambo et al

for evaluation of aneurysm regression because her clinical condi-tion has been excellent. Currently, her blood pressure is wellcontrolled and is on a maintenance low-dose steroid.

DISCUSSION

There is a wide spectrum of clinical presentations ofPAN. Our case provides another example of the variousclinical and radiographic challenges in the management ofPAN. The variety in symptomatology can wax and wanefrom acute life-threatening abdominal hemorrhage second-ary to ruptured isolated hepatic aneurysms to an indolentsubclinical vasculitis. Furthermore, these isolated hepaticaneurysms are extremely rare in the setting of PAN. Ap-proximately seven cases have been described in the past 40years.3 PAN manifests as small aneurysms, vascular ectasia,and vascular occlusion of small and medium sized vessels.4,5

Visceral involvement approaches 50% in the gastrointesti-nal tract and 70% to 80% in the renal arteries. The incidenceof aneurysm formation is variable from 13-60%.3 Recently,Ryan et al6 described segmental arterial mediolysis of thehepatic artery that is radiographically similar to PAN with-out evidence of a vasculitic process. In their case, multipleaneurysms were identified without clinical and rheumato-logic findings of PAN. Our case demonstrates similar radio-graphic findings; however, rheumatologic and radiographicfindings helped confirm the diagnosis of PAN.

Although a tissue biopsy of the involved symptomaticnerve or muscle is ideal (65% sensitive), angiography isdiagnostic in some classic cases.4 The acute activity of thedisease is associated with the formation of aneurysms. Hy-pertension can be found in 75% of patients with renalinvolvement. The risk of aneurysm rupture increases oncehypertension develops.3 As in our case, the risk of aneurysmrupture increased significantly with the patient’s uncon-trolled hypertension; clinically, these patients do worse inlight of the acute nature of ruptured aneurysms. Further-more, we embolized the second nonruptured large hepaticaneurysm because its appearance angiographically was sim-ilar to the acutely ruptured aneurysm, and this aneurysmleft untreated could be a potential source of subsequentrupture in the future. Stanson et al4 state that prophylactictreatment of large aneurysms should be considered in an-ticipation of the risk of rupture. The other smaller aneu-rysms arising from the replaced right hepatic artery did nothave the appearance of impending rupture at the time ofthe procedure. Aneurysms of this caliber may resolve overtime as clinical symptoms resolve.4,7

Overall, transcatheter embolization of spontaneousperirenal hemorrhage secondary to PAN has been de-scribed with low incidence of procedure-related mortality(3.6%) and has the ability to spare adjacent non-involvedorgans.8 We used microcoil embolization during this pro-cedure for immediate and permanent thrombosis of theaneurysms. Excellent results have been achieved consis-tently over the years with catheter-directed embolic agents

such as microcoils, Gelfoam (Pharmacia and Upjohn,Kalamazoo, Mich), and particulate materials in the settingof acute gastrointestinal hemorrhage. Our patient has donewell following her procedure and has been symptom-freeover the last two years. She is normotensive on a regime ofsteroid and immunosuppressive therapies, with no furtherclinical sequelae of PAN. These aneurysms have beenshown to regress with aggressive medical management andtight control of blood pressure.3 Disappearing aneurysmsthat reappear in different location have also been describedon follow-up angiograms. Darras-Joly et al9 states thatfollow-up angiography is not indicated if clinical improve-ment is evident. However, recent data by Tarhan et al10

demonstrate that multidetector computed tomography isan excellent noninvasive modality for evaluation of aneu-rysmal involvement as small as 3 mm and for follow-upsurveillance after treatment.

In summary, we describe both the diagnostic and ther-apeutic advantages of transcatheter angiography and life-saving coil embolization in the setting of isolated acutelyruptured hepatic artery aneurysms in a previously undiag-nosed patient with PAN. Endovascular specialists should beaware that PAN could present with acutely life-threateningruptured hepatic artery aneurysms.

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3. Choy CWK, Smith PA, Frazer C, Jeffrey GP. Ruptured hepatic arteryaneurysm in polyarteritis nodosa: a case report and literature review.Aust NZ J Surg 1997;67:906-8.

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6. Ryan JM, Suhocki PV, Smith TP. Coil embolization of segmentalarterial mediolysis of the hepatic artery. J Vasc Interv Radiol 2000;11:865-8.

7. Guillevin L, Ruel M, Merrouche Y, Gayraud M, Royer I. Regressinganeurysms in polyarteritis nodosa related to hepatitis B virus. EurJ Intern Med 1990;1:267-72.

8. Allen AW, Waybill PN, Singh H, Brown DB. Polyarteritis nodosapresenting as spontaneous perirenal hemorrhage: angiographic diagno-sis and treatment with microcoil embolization. J Vasc Interv Radiol1999;10:1361-3.

9. Darras-Joly C, Lortholary O, Cohen P, Brauner M, Guillevin L. Re-gressing microaneurysms in 5 cases of hepatitis B virus related polyar-teritis nodosa. J Rheumatol 1995;22:876-80.

10. Tarhan NC, Coskun M, Kayahan EM, Yildirim E, Yucel E. Regressionof abdominal visceral aneurysms in polyarteritis nodosa: CT findings.AJR Am J Roentgenol 2003;180:1617-9.

Submitted Nov 20, 2003; accepted Jan 12, 2004.Available online Feb 23, 2004.