Hughes-Stovin syndrome is an extremely rare diseaseknown to cause multiple pulmonary artery aneurysms(PAAs) and venous thrombosis (1, 2). The patients candevelop massive hemoptysis and the disorder can resultin death. Although the use of systemic corticosteroidswith a combination of immunosuppressants has beenthe mainstay of treatment, interventional treatments arerequired for a life-threatening hemoptysis. However, athrombosis in the central veins can impede catheter pas-sage. In this report, we describe the use of percutaneoustranshepatic venous embolization through the hepaticvein due to occluded common vascular pathways to thepulmonary artery.

Case Report

The institutional review board approved the study re-ported here. A 42-year-old male patient was admittedwith acute abdominal pain. The patient had a history ofdeep vein thrombosis on the left lower extremity ninemonths prior to presentation and had been taking war-farin. The patient had had a recurrent oral ulcer for 10years, but a genital ulcer, eye lesion or skin lesion wasnot revealed. The blood pressure was 120/80 mmHgand the heart rate was regular at 96 beats per minute.On physical examination, the abdomen was rigid andflat and direct tenderness was felt in the right upperquadrant area. Laboratory investigations revealed a he-moglobin value of 14.3 g/dL that decreased to 9.3 g/dLthe next day, and a white blood cell count of 9,800/μL.The prothrombin time was prolonged 30.8 sec (normal,9.5-13.2 sec) due to the warfarin therapy. The amylaselevel was increased to 3263 IU/L (normal, 25-115 IU/L)from acute pancreatitis probably due to a mass effect ofa hematoma. A pathergy test of an intradermal injectionof 0.1 ml normal saline into a forearm and the develop-

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Percutaneous Transhepatic Venous Embolization ofPulmonary Artery Aneurysm in Hughes - Stovin Syndrome1

Kyung Ah Kim, M.D., Man Deuk Kim, M.D., Do Yun Oh, M.D.2, Pil Won Park, M.D.2

1Department of Diagnostic Radiology, Bundang CHA General Hospital,Pochon CHA University

2Department of Internal Medicine, Bundang CHA General Hospital,Pochon CHA UniversityReceived March 23, 2007 ; Accepted July 11, 2007Address reprint requests to : Man Deuk Kim, M.D., Department ofDiagnostic Radiology Bundang CHA General Hospital, Pochon CHAUniversity, 351 Yatap-dong, Bundang-gu, Sungnam-si, Kyonggi-do 463-712, Republic of Korea.Tel. 82-31-780-5382 Fax. 82-31-780-5381 E-mail: mdkim@cha.ac.kr

Hughes-Stovin syndrome is an extremely rare entity. We present a case of a 42-year-old man, who developed deep vein and inferior vena cava (IVC) thrombosis, repeatedinternal bleeding and pulmonary artery aneurysms (PAAs). The patient presented withmassive hemoptysis and with PAAs of a 2.5 cm maximum diameter. We describe thesuccessful percutaneous transhepatic venous embolization of the PAAs due to occlud-ed common vascular pathways to the pulmonary artery.

Index words : Pulmonary arteryEmbolizationAneurysmComputed tomography (CT)Cardiovascular

ment of a skin eruption (papule, nodule, pustule) was al-so negative.

Computed tomography (CT) of the abdomen revealeda hematoma and active bleeding in anterior pararenalspace around the duodenal second portion with a focal,localized eccentric thrombosis of the inferior vena cava(IVC) at the level of renal vein confluence. Arteriogra-phy demonstrated multiple microaneurysms at thetransverse pancreatic artery and the pancreaticoduode-nal artery; they were embolized with three 6-mmTornado platinum microcoils (Cook, Bloomington, INU.S.A.).

The patient had to stop anticoagulation therapy due tobleeding, and a Gunther Tulip IVC filter (Cook) wastemporarily placed via the right internal jugular vein.

Recovery was uneventful, and the patient was dis-charged in good condition after two weeks with antico-agulation therapy, again with warfarin for IVC thrombo-sis and the IVC filter was retrieved via the right internaljugular vein. However, after another 4 weeks, the pa-tient was readmitted to a local hospital because of mas-sive hemoptysis. When the patient was referred to ourhospital, a contrast-enhanced CT scan revealed well de-marcated, two ovoid shaped vascular masses on theright lower lobe compatible with PAAs (maximum di-ameter 1.5 cm and 2.5 cm, respectively). The diagnosisof Hughes-Stovin syndrome was made only after findingthe PAAs. The IVC was completely occluded andsuprarenal placement of a Gunther Tulip IVC filter(Cook) was performed. The left internal jugular veinwas used for placement of the IVC filter since the rightinternal jugular vein was occluded. The only medicaltreatment of immunosuppressive therapy was donewithout considering an interventional procedure sincethe patient had been stable when referred and had re-fused to undergo an intervention.

On a follow-up chest CT scan 4 months later, one oftwo PAAs in the superior segment of the right lowerlobe had spontaneously regressed. The secondaneurysm in the posterior basal segment of the rightlower lobe decreased in size, and was partially throm-bosed. The patient was readmitted with pain in the leftinguinal and left lower quadrant 4 months later. CT re-vealed a hematoma of the left psoas muscle area andcontrast extravasation. Arteriography revealed ananeurysm of the third left lumbar artery, and endovas-cular embolization with glue was performed successful-ly.

Three months later, the patient returned to the hospi-

tal for blood-tinged sputum during coughing. A chest CTrevealed that the PAA in the right lower lobe had in-creased in size measuring 2.5 × 2.0 cm (Fig. 1). Due tothe risk of aneurysm rupture and massive hemoptysis,embolization of the PAA was planned, but the commonvenous access routes (jugular, subclavian, IVC) were to-tally occluded.

Therefore, the right hepatic vein via a percutaneoustranshepatic venous approach was chosen for venousaccess. Fluoroscopic-guided puncture of the right hepat-ic vein was performed (Fig. 2A) by use of a NEFF percu-taneous access set (Cook). Right pulmonary arteriogra-phy demonstrated a PAA present on the right lower lobe(Fig. 2B). A 90 cm, 7-French introducer catheter(Vistabritetip, Cordis, Miami, FL U.S.A.) was advancedinto the right posteromedial branch of the pulmonaryartery, and the feeding artery was then subselected withuse of the inner 5-French catheter (DAV, Cook,) withthe coaxial system. Embolization of the feeding arteryand the aneurysmal sac was then performed usingtwelve platinum coils (12 mm× 14 cm, Nester, Cook)until there was complete cessation of blood flowthrough the aneurysm (Fig. 2C). Withdrawing the guid-ing catheter, gelfoam embolization was performed toprevent tract bleeding. The patient had no respiratorysymptoms after the procedure and the blood oxygena-tion level was within the normal range.

Post-procedural recovery was uneventful, and the pa-tient was discharged 7 days after embolization. No moreblood-tinged sputum was reported and a follow-up chestplain film shows no evidence of recurrence of a pul-monary aneurysm in 16 months.

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Fig. 1. A chest CT scan reveals a pulmonary artery aneurysmhas increased in size measuring 2.5 cm in the maximum diam-eter on the right lower lobe.

Discussion

Hughes and Stovin first described a syndrome consist-ing of multiple pulmonary aneurysms and peripheralvenous thrombosis in 1959 (1). Patients can show di-verse symptoms from mild symptoms such as cough,dyspnea, headache and intermittent fever to severesymptom such as papilledema or massive hemoptysis(3, 4). The etiology is still unknown. Hughes and Stovinpostulated that a congenital defect of the bronchial arter-ial wall results in inadequate nutrition to pulmonary ar-teries (1, 4, 5). In the setting of pulmonary embolic dis-ease, inflammation and vessel wall destruction occursand aneurysms are formed. Another theory is that in-

fected emboli with organisms of low-grade virulencewill cause mycotic aneurysms (1, 4, 5).

Hughes-Stovin Syndrome can be diagnosed in the caseof PAAs combined with deep vein thrombosis withoutevidence of Behcet’s disease.

Until now, fewer than 30 cases have been publishedin the English language literature.

Several cases revealed that massive hemoptysis due torupture of a PAA was the predisposing condition fol-lowed by death (6, 7).

In Hughes-Stovin syndrome, the pulmonary artery isusually involved with hemoptysis but there has been areport of hemoptysis caused by rupture of a bronchialartery aneurysm (8). In another case, deep vein throm-bosis and combined left hepatic artery aneurysms were

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A B

C

Fig. 2. A. Percutaneous transhepatic venousvenous ac-cess was attempted. An IVC filter is demonstrated onthe suprarenal portion for IVC thrombosis.B. A pulmonary artery angiogram reveals a 2.5 cmsized pulmonary artery aneurysm on the right lowerlobe.C. Angiography shows complete exclusion of the pul-monary artery aneurysm following embolization withtwelve Nester coils.

reported (5).This case showed pancreaticoduodenal and lumbar

artery aneurysms that were ruptured as well as PAAswith progrssive deep vein and IVC thrombosis. The fre-quency of pulmonary involvement by Behcet’s diseaseis about 1-8% (9). When a pulmonary artery aneurysmis revealed, usually Bechet’s disease is suspected.Nevertheless, if clinical symptoms such as a skin lesionor genital ulcer are not consistent with Behcet’s disease,the next step is to consider Hughes-Stovin syndrome.The use of a pathergy test that has been accepted as oneof the major criteria in Behcet’s disease shows a nega-tive finding for Hughes-Stovin syndrome.

Treatment for the PAAs is mainly the use of immuno-suppressive agents such as steroids, cyclophosphamideor azathioprine. Acican et al. (10) reported complete re-gression of a pulmonary aneurysm when azathioprine-steroid combination therapy was used in a patient withBechet’s disease. This case also demonstrated that med-ical treatment could induce regression of a PAA thoughthe partially regressed PAA later increased in size. Ifrapid resolution of a pulmonary aneurysm causing clini-cal symptoms is required, endovascular embolization orsurgery can be considered.

Lobectomy is major method for surgical treatment of apulmonary aneurysm. However, a lobectomy has signif-icant limitations. Pulmonary involvement in Hughes-Stovin syndrome shows a tendency of multiple pul-monary aneurysms in a few lobes, and close follow-upshould be mandatory. Endovascular embolization withcoils or other embolic agents is the ideal method forPAA treatment. This method can lead to rapid occlusionof an aneurysm and the procedures can be performedseveral times. The general condition of a patient will

more quickly recover than for surgery. However, athrombosis in central veins can impede catheter pas-sage. To the best of our knowledge, this is the first re-port of the use of transhepatic venous embolization ofPAA in Hughes-Stovin Syndrome.

We conclude that the percutaneous transhepatic ve-nous approach is a good alternative access route to treatPAAs in patients whose normal pathways are occludeddue to a venous thrombosis.

References

1. Hughes JP, Stovin PG. Segmental pulmonary artery aneurysmwith peripheral venous thrombosis. Br J Dis Chest 1959;53:19-27

2. Ammann ME, Karnel F, Olbert F, Mayer K. Radiologic findings inthe diagnosis of Hughes-Stovin syndrome. AJR Am J Roentgenol1991;157:1353-1354

3. Weintraub JL, DeMayo R, Haskal ZJ, Susman J. SCVIR annualmeeting film panel session: diagnosis and discussion of case 1:Hughes-Stovin syndrome. J Vasc Interv Radiol 2001;12:531-534

4. Fischer A, Korman DS, West SG. Radiologic vignette: Hughes-Stovin syndrome. Arthritis Rheum 2005;53:114-116

5. Mahlo HR, Elsner K, Rieber A, Brambs HJ. New approach in thediagnosis of and therapy for Hughes-Stovin syndrome. AJR Am JRoentgenol 1996;167:817-818

6. Kopp Wl, Green Ra. Pulmonary artery aneurysms with recurrentthrombophlebitis. The “Hughes-Stovin syndrome”. Ann Intern Med1962;56:105-114

7. Durieux P, Bletry O, Huchon G, Wechsler B, Chretien J, GodeauP. Multiple pulmonary arterial aneurysms in Behcet’s disease andHughes-Stovin syndrome. Am J Med 1981;71:736-741

8. Herb S, Hetzel M, Hetzel J, Friedrich J, Weber J. An unusual caseof Hughes-Stovin syndrome. Eur Respir J 1998; 11:1191-1193

9. Hiller N, Lieberman S, Chajek-Shaul T, Bar-Ziv J, Shaham D.Thoracic manifestations of Behcet disease at CT. Radiographics2004;24:801-808

10. Acican T, Gurkan OU. Azathiopine-steroid combination therapyfor pulmonary arterial aneurysms in Behcet’s disease. RheumatolInt 2001;20:171-174

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대한영상의학회지 2007;57:141-144

Hughes-Stovin Syndrome 환자에서경피경간정맥 폐동맥류의 색전: 증례 보고1

1포천중문 의과대학 분당차병원 영상의학과

김경아·김만득·오도연2·박필원2

Hughes-Stovin syndrome은 매우 드문 질환으로 심부 정맥 혈전과 폐동맥류를 동반한다. 저자들은 객혈을 동반

한 Hughes-Stovin syndrome으로 진단 받은 42세 남자 환자에서 심부 정맥혈전으로 인해 일반적인 방법으로는

폐동맥 접근이 어려워 경피경간 색전술로 치료한 1예를 경험 하였기에 이를 문헌 고찰과 함께 보고하고자 한다.