Damar Cer Derg 2014;23(1) 47
ehcet’s syndrome is a multisystemic vasculitis characterized by muco-cutaneous, ocular, arthritic, vascular, and central nervous system ma-nifestations. The prevalence is 80 to 370 cases per 100,000 population
in Turkey.1The male to female ratio is 7:1 in symptomatic forms.2It
invol-ves all size and type of invol-vessels including, both arterial and venous system. Ve-nous disease is more common than arterial involvement, and prevalence may account for 14-39% and 3-5% respectively in patients with Behcet’s disease
respectively.1-3Thrombosis and/or aneurysms are observed; being
predomi-nantly false aneurysms.4Surgical treatment is controversial due to frequent
occurrence of graft occlusion and anastomotic pseudoaneurysms.
Surgical Therapy for
Disseminated Arterial Involvement in
Behcet’s Disease: Case Report
AABBSS TTRRAACCTT Surgical treatment of arterial Behcet’s Disease has a higher incidence of anastomotic pseudoaneurysm or graft occlusion. A 59-year-old male patient presented with arm pain and short-ness of breath. Medical history revealed Behcet’s disease. Magnetic resonance imaging and com-puterized tomography showed aneurysms of main pulmonary, right innominate and left subclavian arteries and occlusion of bilateral subclavian arteries. Main pulmonary artery was plicated. Right innominate artery was bypassed to right subclavian artery, and left carotid artery was bypassed to left subclavian artery with 8 mm biological grafts. Scans showed progressive pulmonary artery aneurysm and patent grafts 12 months postoperatively. Surgery for aneurysm of main pulmonary artery in Behcet’s disease should be planned carefully because Behçet’s vascular disease can be pro-gressive even after surgery.
KKeeyy WWoorrddss:: Behçet’s disease; arterial involvement; surgical therapy Ö
ÖZZEETT Behçet Hastalığı’nın arteriyel tutulumunda, cerrahi tedavide anastomotik psödoanevrizma ve greft oklüzyonu insidansı yüksektir. Elli dokuz yaşında erkek hasta kol ağrısı ve nefes darlığı şika-yeti ile başvurdu. Özgeçmişinde Behçet Hastalığı mevcuttu. Manşika-yetik rezonans görüntüleme ve bilgisayarlı tomografi ile ana pulmoner arter, sağ innominate arter ve sol subklavian arter proksi-malinde anevrizma, bilateral subklavian arterlerde oklüzyon tespit edildi. Ana pulmoner artere pli-kasyon uygulandı. Sağ innominate arter, sağ subklavian artere ve sol karotis arter de sol subklavian artere 8 mm biyolojik greft ile bypass edildi. Postoperatif 12. ay takibindeki görüntülemede pul-moner arter anevrizmasında ilerleme görüldü ve greftler açık olarak izlendi. Vasküler tutulum gös-teren Behçet hastalarında ana pulmoner arter anevrizma cerrahisi dikkatlice düşünülmelidir, çünkü operasyon sonrasında da tutulum progresyon gösterebilir.
AAnnaahh ttaarr KKee llii mmee lleerr:: Behçet hastalığı; arteriyel tutulum; cerrahi tedavi
DDaa mmaarr CCeerr DDeerrgg 22001144;;2233((11))::4477--99
Mehmet KALENDER,a
Ayşe Gül KUNT,a
Okay Güven KARACA,a
Mehmet TAŞARa
aClinic of Cardiovascular Surgery,
Konya Education and Research Hospital, Konya
Ge liş Ta ri hi/Re ce i ved: 27.09.2012 Ka bul Ta ri hi/Ac cep ted: 15.04.2013 Ya zış ma Ad re si/Cor res pon den ce: Mehmet KALENDER
Konya Education and Research Hospital, Clinic of Cardiovascular Surgery, Konya, TÜRKİYE/TURKEY
ka97084@yahoo.com
doi: 10.9739/uvcd.2012-32206
Cop yright © 2014 by
Ulusal Vasküler Cerrahi Derneği
OLGU SUNUMU
Arteriyo-Venöz Malformasyonlar,
Damar Cer Derg 2014;23(1)
48
Mehmet KALENDER et al. SURGICAL THERAPY FOR DISSEMINATED ARTERIAL INVOLVEMENT IN BEHCET’S DISEASE: CASE REPORT
CA SE RE PORT
A 59-ye ars-old ma le pa ti ent had suf fe red from right arm pa in and short ness of bre ath for 3 ye ars. Me di cal his tory re ve a led Beh cet’s di se a se for 14 ye -ars. His vi tal signs we re stab le on ad mis si on. Pul ses of both up per ex tre mi ti es we re not pal pab le. The -re was no ot her system in vol ve ment. Mag ne tic -re s-o nan ce (MR) an gi s-og raphy s-of the ex tre mi ti es and com pu te ri zed to mog raphy of tho rax sho wed that ma in pul mo nary ar tery was 50 mm; right and left pul mo nary ar te ri es we re 31 mm and 29 mm, res pec ti vely in di a me ter; right in no mi na te ar tery ane -urysm was 26 mm; left subc la vi an ar tery was 20 mm. Right subc la vi an ar tery was occ lu ded star ting from 1 cm dis tal to the bi fur ca ti on of in no mi na te ar tery as in the se scans (Fi gu res 1A and 1B).
The pa ti ent was ope ra ted un der ge ne ral anest -he si a with me di an ster no tomy to re ach t-he ma in pul mo nary ar tery, right and left subc la vi an ar te ri es. Ma in pul mo nary ar tery was pli ca ted pri ma rily. Anas to mo ses of left ca ro ti co-subc la vi an and right in no mi na te ar ter i es to the axil lary ar tery we re per-for med u sing 8 mm bi o lo gi cal grafts. Ane ury sma tic seg ment of left subc la vi an ar tery was ti ed up. One me di as ti nal dra i na ge tu be was pla ced af ter he mos -ta sis. Ster num was clo sed with ste el wi res, as usu al.
Pos to pe ra ti ve co ur se was une vent ful. The pa-ti ent was disc har ged on pos to pe ra pa-ti ve fo urth day. MR an gi og rap hi es of both ex tre mi ti es and com pu -te ri zed to mog raphy of tho rax we re ob ta i ned 12-months pos to pe ra ti vely. Scans sho wed that all grafts we re pa tent and prog res si ve na ti ve ma in pulmo nary ar tery ane urysm re ac he d 52 mm in di a me -ter (Fi gu res 2A and 2B).
DIS CUS SI ON
Beh cet’s Di se a se is a mul tisy ste mic chro nic au to im mu ne inf lam ma tory di se a se cha rac te ri zed by re cur -rent oral and ge ni tal aph tho us ul cers and ocu lar le si ons. Ar te ri al in vol ve ment in Beh cet’s di se a se is ra re, throm bo sis and/or ane urysms are ob ser ved, be ing ma inly fal se ane urysms.4The se “ar te ri al aphta e” are lo ca li zed on pul mo nary ar te ri es, aor aphta, re -nal and pe rip he ral ar te ri es. Vas cu lar sur gery is ob li ga tory, but graft throm bo sis and re lap se of
ane urysm at the si te of bypass are fre qu ent. The pulmo nary ane urysms ha ve a se ve red re ad ful prog no -sis.5Pre vi o us pa pers re por ted that graft occ lu si on or anas to mo tic pse u do a ne urysm tend to oc cur ear li er com pa red to ot her di se a ses.3,6The se fin dings ca u se dif fi cul ti es in se lec ting t he sur gi cal pro ce du re and graft ma te ri al in pa ti ents with Beh cet’s di se a se. Ba -sed on this in for ma ti on, we avo i ded use of a graft for tre at ment of pul mo nary ar tery ane urysm. Whi le per for ming pul mo nary ar tery ane urysm pli ca ti -on, we pre fer red off-pump clamp and sew tech ni qu e. The ma in re a son for cho o sing this tech-ni qu e was to ma in ta in mi tech-ni mal in va si on es pe ci ally to ma jor vas cu lar struc tu res. Alt ho ugh the re is not eno ugh evi den ce to avo id cardiopulmonary bypass, high comp li ca ti on ra tes of comp li ca ti ons and ca tas -trop hic risks mo ti va ted us to cho se off-pump tech-ni que. En do vas cu lar stent graf ting for re cur rent ane urysms is no te worthy for Beh cet’s di se a se.7Ane -urysms of the dis tal ves sels in Beh çet’s di se a se are very in fre qu ent and each ca se sho uld be eva lu a ted FI GU RE 1A: Mag ne tic re so nan ce ima ging: Ane urysm of right in no mi na te
ar-tery and occ lu si on of left subc la vi an ar ar-tery.
FI GU RE 1B: Computerized tomography of thorax: Aneurysm of the main
Damar Cer Derg 2014;23(1) 49 SURGICAL THERAPY FOR DISSEMINATED ARTERIAL INVOLVEMENT IN BEHCET’S DISEASE: CASE REPORT Mehmet KALENDER et al.
on an in di vi du al ba sis. En do vas cu lar tre at ment can be a go od al ter na ti ve the ra pe u tic tech ni qu e.8
This par ti cu lar ca se he re in ful fil led the di ag -nos tic cri te ri a of Beh cet’s di se a se and the ane urysm of the pul mo nary ar tery has be en inc lu ded in the cha rac te ris tics of the pre sen ta ti on of vas cu lar Beh -cet’s di se a se. The re fo re, we se lec ted pul mo nary
ar-tery pli ca ti on and bi o lo gi cal grafts for pe rip he ral by-pas ses. The scans in the fol low-up sho wed pa tent grafts and no pse u do a ne urysms at the si te of the anas to mo sis. On the ot her si de, pul mo nary ar te ri al ane urysm re lap sed at pos to pe ra ti ve 12 months. De-s pi te the in cre a De-se in the di a me ter, we de ci ded to fol-low up the pa ti ent, be ca u se he did not ha ve he mody na mic prob lems and the re was no ac com -pan yin g he mopt ysis. In ad di ti on, pul mo nary ar tery ane urysm was se en only in the ma in pul mo nary ar-tery. Se ya hi et al. re por ted pul mo nary ar te ri al in-vol ve ment as a po or prog nos tic fac tor.5Ad di ti o nally, Ha mur yu dan et al. and Tu zun et al. re por ted se ri es with pul mo nary ar tery ane urysms and conc lu ded that prog no sis was po or with high mor ta lity ra tes in sur gi cally tre a ted pa ti ents, es pe ci ally w hen ac com -pan ying he mopt ysis was se en.9,10Sa ba et al. re por ted 25% sur vi val ra te in 8 pa ti ents with a pul mo nary ar-tery ane urysm. In this pa per, the aut hors inc lu ded 23 pa ti ents with ar te ri al ane urysms ac com pan ying Beh cet’s Di se a se.11On the ot her hand, so me pa pers cla im that pro per im mu no sup pres sant the rapy may reg ress pul mo nary ar tery ane urysm, but the se pa-pers ha ve smal ler pa ti ent num bers.13,14
Sur gery for ane urysm of ma in pul mo nary ar tery in Beh cet’s di se a se sho uld be eva lu a ted ca refully be ca u se Behcet’s vas cu lar di se a se can prog -res si ve even af ter sur gery.
C
Coonnfflliicctt ooff IInntteerreesstt
Authors declared no conflict of interest or financial sup-port.
FI GU RE 2A: Magnetic resonance imaging: Biological graft of left
carotico-sub-clavian bypass and right innominate artery to axillary artery bypass are patent.
FI GU RE 2B: Computerized tomography: Relapsed main pulmonary artery
aneurysm.
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