비디오흉강경을이용하여폐절제술로치료한편측미만성폐동정맥루 1 례 연세대학교의과대학내과학교실 1, 흉부외과학교실 2, 영상진단학과학교실 3, 폐질환연구소 4 정종열 1, 임종근 1, 전성완 1, 서원나 1, 김대준 2, 이광훈 3, 박무석 1, 김세규 1,4, 장준 1,4, 김성규 1,4, 김영삼 1,4 A Case of Video-Assisted Thoracoscopic Pneumonectomy for Unilateral Diffuse Pulmonary Arteriovenous Malformation Jong Yul Jung, M.D. 1, Jong Keun Lim, M.D. 1, Sung Wan Chun, M.D. 1, Won Na Suh, M.D. 1, Dae Jun Kim, M.D. 2, Kwang Hun Lee, M.D. 3, Moo Suk Park, M.D. 1, Se Kyu Kim, M.D. 1,4, Joon Chang, M.D. 1,4, Sung Kyu Kim, M.D. 1,4, Young Sam Kim, M.D. 1,4 1 Department of Internal Medicine, 2 Thoracic and Cardiovascular Surgery, and 3 Diagnostic Radiology, 4 The Institute of Chest Diseases, Yonsei University College of Medicine, Seoul, Korea Pulmonary arteriovenous malformations (PAVMs) are abnormal direct communications between the pulmonary arteries and veins. PAVMs may occur as either an isolated abnormality or in association with hereditary hemorrhagic telangiectasia, also called Osler-Weber-Rendu disease. The topic of PAVM has recently been extensively reviewed, but little is known about the clinical characteristics and course of patients having a diffuse pattern of the disease. Herein, is reported a case of unilateral diffuse PAVM in an 18 year old female patient, who underwent a right pneumonectomy, under a video-assisted thoracic surgery (VATS) approach, as the diffuse small pulmonary arteriovenous malformation involved the whole right lung. (Tuberc Respir Dis 2006; 61: 585-590) Key Words: Diffuse, Pulmonary, Arteriovenous malformation. 서 폐동정맥루는비정상적으로폐동맥과폐정맥사이에직접적인연결이있는것을말한다. 폐동정맥루는단독으로생길수도있고 Olser-Weber-Rendu disease 즉, 유전출혈모세혈관확장증 (hereditary hemorrhagic telangiectasia, HHT) 과연관되어발생하기도한다. 전혀증상이없을수있지만심각한임상양상을보일수도있다. 폐동정맥루는좌우단락의결과로호흡곤란이나청색증등의증상을나타내게되고대량객혈이나혈흉의원인이되기도한다. 특히좌우단락때문에일과성허혈발작이나뇌졸중, 그리고뇌농양등의심각한신경학적합병증을일으키는경우가있다 1. 저자들은작은크기의폐동정맥루가편측의폐전체에광범위하게분포하여동정맥루에색전술을시행 론 Address for correspondence: Young Sam Kim, M.D. Department of Internal Medicine, Yonsei University College of Medicine, 134 Shinchon-Dong, Seodaemun-Ku, Seoul 120-752, South Korea. Phone: 82-2-2228-1965, Fax: 82-2-393-6884, E-mail: ysamkim@yumc.yonsei.ac.kr Received: Oct. 16. 2006 Accepted: Nov. 6. 2006 할수없어, 비디오흉강경 (VATS) 을이용하여폐절제술로치료한증례가있어문헌고찰과함께보고하는바이다. 증례환자 : 고O지, 18세, 여자주소 : 객혈, 운동시호흡곤란현병력 : 약 3년전부터점차심해지는운동시호흡곤란이있었고객혈이있어내원하였다. 과거력 : 약 3년전부터운동시호흡곤란을느꼈고친구들보다운동능력이떨어짐을느꼈으며, 장출혈, 비출혈이나신경학적이상증상등의특이사항은없었다. 가족력 : 특이사항없었다. 문진소견 : 내원시문진결과운동시호흡곤란이점차심해진다고하였고간헐적두통, 기침, 쉽게피로해짐을호소하였다. 신체검사소견 : 신장은 165 cm, 체중은 45 kg 이었고내원당시혈압은 100/70 mmhg 맥박은분당 90 회, 호흡수분당 18회, 체온은 36.5도이었다. 입술, 손과발의청색증과곤봉지소견이관찰되었 585
JY Jung et al: Video-assisted thoracoscopic pneumonectomy for unilateral diffuse PAVM Figure 1. Chest roentgenogram showed minimal parenchymal infiltration on right parahilar area at admission. 다. 피부와구강점막, 비점막등에모세혈관확장소견은없었다. 청진상호흡음은깨끗하였으며, 심음은규칙적이었고심잡음은청진되지않았다. 복부는편평하고장음은정상이었으며, 압통이나반발통은없 었고, 종물은촉지되지않았다. 사지운동제한은없었고, 양측다리의함요부종이관찰되지않았다. 검사실소견 : 말초혈액검사상혈색소 14.8 mg/dl, 적혈구용적률 45.6%, 백혈구수 7,410 /mm 3 ( 정상감별혈구범위내 ) 였고대기호흡중시행한동맥혈검사에서 PaO 2 48.4 mmhg, PaCO 2 30.9 mmhg, 동맥혈산소포화도는 87.3% 였다. 방사선학적검사 : 단순흉부 X-선검사결과우측폐문부에결절음영을보였고 (Figure 1), 흔들어작은공기방울을일으킨생리식염수 (agitated saline) 를말초정맥에주입한후에조영심장초음파검사를시행한결과우심방에공기방울이나타나고 4주기의심장박동후에좌심방에공기방울이나타났다. 흉부전산화단층촬영결과우측폐의 1번, 2번, 3번, 6번, 7번, 8번, 9번, 10번기관지폐구역에서확장되고구불구불한폐동맥과폐정맥이관찰되었다 (Figure 2). 폐동맥혈관조영술에서는조영제가폐동맥에서폐정맥으로빠르게이행되는것을볼수있었고다수의작은주머 Figure 2. Chest CT scan showed slightly dilated tortuous pulmonary arteries and pulmonary veins (long arrow) in right segment 1, 2, 3, 6, 7, 8, 9, 10. Hypertrophied bronchial arteries and mediastinal collaterals through right pulmonary ligament. 586
Tuberculosis and Respiratory Diseases Vol. 61. No.6, Dec. 2006 Figure 3. Main pulmonary arteriography showed innumerable tiny saccular aneurysms on the whole right pulmonary artery peripheral portion and early venous drainage (A). Selective right interlobar pulmonary artery and upper lobar pulmonary artery angiography demonstrated innumerable tiny saccular aneurysms and early venous drainage (B). Figure 4. 3-Dimentionally reconstructed CT scan showed diffuse pulmonary arteriovenous malformations on the right Lung.(A) Magnified Image of the right Lung (B) 니모양동맥류가말초부위를포함한우측폐전체범위에서관찰되었다. 선택적우상엽폐동맥혈관조영술시에도다수의작은주머니모양동맥류가보였고조기폐정맥배류를보였다 (Figure 3, 4). 폐동맥혈관조영술의소견은우측폐전체를침범하고있는미만성폐동정맥루의소견이었다. 경과및치료 : 동정맥루에대한색전술은크기가작고수가많아시술이불가능하였다. 객혈이있었고환자의증상이점차심해지고있었으며발생가능한합병증의심각성을고려하여비디오흉강경하폐절제술을시행하였다. 폐절제술전에기관내삽관상태에서 100% 산소공급시 PaO 2 는 97.6 mmhg, PaCO 2 는 587
JY Jung et al: Video-assisted thoracoscopic pneumonectomy for unilateral diffuse PAVM Figure 5. The specimen showed geographic hemorrhage in the superior segment of lower lobe. On section, multiple dilated blood vessels and multifocal hemorrhage are noted 31.9 mmhg 산소포화도는 98.2% 였고계산된단락분율은 29% 였다. 폐절제술시행직후 PaO 2 383.9 mmhg, PaCO 2 31.1 mmhg 산소포화도는 99.9% 를보였다. 환자는수술후부작용없이퇴원하였으며현재특별한증상없이건강한상태로외래추적관찰중이다. 병리학적검사 : 조직병리검사결과폐동정맥루의소견인, 확장되고구불구불한정맥과기관혈관초밖에비정상적으로위치한동맥을볼수있었고이러한폐동정맥루는주로우하엽에있었으나중엽과상엽에도분포하고있었다 (Figure 5). 고 폐동정맥루 (pulmonary arteriovenous malformation) 는폐동맥과폐정맥사이에비정상적인연결이형성된것으로학자들에따라 pulmonary arteriovenous fistula, hemangioma of lung, congenital telangiectasis of lung 등의다른용어로불린다. 폐동정맥루의국내발생빈도는알려져있지않지만외국의경우 15,000 례의부검시 3 명에게서발견되었다는보고가있는등드문질환이다. 병인이명확히밝혀져있지는않으나선천성으로는단독으로생기거나유전출혈모세혈관확장증과연관되어발생하는데폐동정맥루환자의 70% 가유전출혈모세혈관확 찰 장증과연관이있고반대로유전출혈모세혈관확장증환자의 15-35% 에서폐동정맥루가발생하는것으로보고되었다 2,3. 후천적으로는외상, 주혈흡충증, 간경변, 전이성폐암, 방선균증등에의해발생하는것으로알려져있다 4. 폐동정맥루는얇은벽으로이루어진비정상혈관으로모세혈관을거치지않고폐순환과체순환간을직접연결한다. 우좌단락을통과하는폐동맥은산소공급이되지않으므로저산소혈증을유발한다. 폐동정맥루는모세혈관계의여과기능이없으므로입자등이체순환에도달하는것을막지못하고임상적으로는특히뇌혈관순환에문제를일으키는데뇌졸중이나뇌내농양등을초래하게된다 5. 비정상적이고약한혈관은기관내나흉강내에치명적인출혈을일으킬수도있다. 폐동정맥루가있는환자의약 30% 에서뇌졸중이발생하며 10% 정도에서뇌농양, 폐출혈은약 10% 정도에서발생하는것으로보고되고있다 1,4. 치료로는수술적절제, 색전술등이주로이용되는데수술적인방법에는결찰, 부분절제, 구역절제술, 엽절제술, 폐절제술 1 등이있고색전술은폐동맥조영술을시행하면서선택적으로동정맥루부분을풍선이나코일을이용해서폐쇄하는데일반적으로직경이 3 mm 이상되는경우시행하게된다 7. 최근에는폐혈류재분포술이치료에이용되기도한다 8. 1940년에 Shestones 이폐동정맥루를폐절제술로 9 성공적으로치료한후로부분절제술이나구역절제술등, 가능하면폐실질을보존하는방식의수술이주류를이루어왔으며최근에는중재적인방법으로색전술을시행하는것이우선적으로시행되어왔다. 그러나색전술만으로해결이되지않는경우에는수술과색전술을병행하기도하며단계적인시술을하기도한다 10. 비디오흉강경을이용한폐절제술은 1998년에 Temes RT 11 등이단일폐동정맥루에대한쐐기절제술을보고한이후, Thung KH 12 등이비디오흉강경을이용한폐엽절제술을보고하였고, 두증례에서수술후특이한합병증및증상을보이지않았다. 저자들은기존의수술및색전술의장점을지니고있는, 최소침습적인방법인비디오흉강경을이용한수술을또하나의치료방법으로고려될수있을것으로 588
Tuberculosis and Respiratory Diseases Vol. 61. No.6, Dec. 2006 제시하고있다. 폐동정맥루의치료는색전술의도입으로진전을이루었으나미만성인경우치료법이나임상양상, 진행경과등에대해알려진것이별로없고특히미만성폐동정맥루가양측폐를침범한경우는예후가매우불량하여폐이식을주장하는학자들도있다. 그러나이또한이식자체의합병증때문에예후가불량하다. Frughnan 등은 16명의미만성폐동정맥루환자를치료하고추적관찰한연구에서편측성미만성폐동정맥루환자에게폐혈류재분포술 (pulmonary flow redistribution) 을시행하여좋은결과를보고하였는데이들환자는폐동정맥루가하나의폐엽에국한되어있어이러한시술이가능하였다. 이들중한환자는시술후임상적으로호흡곤란에서회복되었으며다른환자는시술전 4번의자연유산이반복되었으나시술후임신에성공하여정상분만후직장생활이가능하였다 13 (Table 1). 국내의경우 3 례의미만성폐동정맥루가보고되었다. 그중한환자는좌하엽에국한된미만성폐동정맥루를엽절제술로치료하였고 14 두번째환자는의식장애로내원하여뇌혈관기형과양측폐의미만성폐동정맥루를진단받고색전술을시행하였으며세번째환자는치료없이경과관찰하였다 15 (Table 1). 본증례에서환자는우측에만다수의작은폐동정맥루가광범위하게분포하고있었고유전출혈모세혈관확장증등다른동반질환을의심할만한소견은없었다. 크기가작은다수의폐동정맥루에색전술을시행할수없었고상 중 하엽을모두침범하여폐혈류재분포술을시도할수없었다. 유전출혈모세혈관확장증소견이없는단독폐정맥루에서도심각한폐출혈이나신경계부작용이보고되었으며임신시에는폐동정맥루의크기가커지는것으로알려져있다. 본증례의환자는가임기로객혈이있었고환자의증상이점차심해지고있었으며색전술을시행할수없었다. 심각한합병증을고려 Table 1. Characteristics of patients with diffuse pulmonary AVMs* Patient, Reference 1 st 2 nd Frughnan et al. Frughnan et al. (13) (13) 3 rd Jeong et al. (14) 4 th Jeon et al. (15) 5 th Jeon et al. (15) Age 33 31 26 28 58 18 Gender M F M F F F Distribution of AVM Unilateral in 1 lobe Unilateral in 1 lobe Unilateral in 1 lobe Bilateral Bilateral current Unilateral in a whole lung Hb mg/dl 18.2 NA 19.0 14.6 18.1 14.8 PaO 2, mmhg 50 / 65 53 / 99 52 / NA 42 / NA 48.2 / NA 48.4 / 99 TIA - / - - / + - / NA + / NA - / NA - / - Brain abscess +/- - / - - / NA - / NA - / NA - / - Hemoptysis - / - - / - - / NA - / NA - / NA + / - Other malformation - - - - Spider angioma on face, trunk Treatment PFR PFR Lobectomy Embolization Observation Outcome Significantly improved dyspnea *AVMs : arteriovenous malformations NA : Not available TIA : Transient ischemic attack PFR: Pulmonary flow redistribution Miscarriages 4 times pregnant and normal delivary NA No neurologic symptoms NA - Pneumonectomy(VATS) Symptom free 589
JY Jung et al: Video-assisted thoracoscopic pneumonectomy for unilateral diffuse PAVM 하여비디오흉강경을이용해서폐절제술을시행하였고현재환자는외래추적관찰중으로건강한상태이다. 요 저자들은편측성으로우측폐전체를침범한미만성폐동정맥루에대해색전술등의중재적시술이불가능하여비디오흉강경을이용해서폐절제술로치료한 1예를경험하였기에, 문헌고찰과함께보고하는바이다. 약 참고문헌 1. Gossage JR, Kanj G. Pulmonary arteriovenous malformations: a state of the art review. Am J Respir Crit Care Med1998;158:643-61. 2. Vase, P, Holm M, Arendrup H. Pulmonary arteriovenous fistulas in hereditary hemorrhagic telangiectasia. Acta Med Scand 1985218:105-9. 3. Hodgson CH, Burchell HB, Good CA, Claggett OT. Hereditary hemorrhagic telangiectasis and pulmonary arteriovenous fistula. N Engl J Med 1959261:625-36. 4. Ahn JB, Kim IS, Jung SC, Kim WS, Shin YC, Yoo HK, et al. Pulmonary arteriovenous fistula with hemothorax. Korean J Thorac Cardiovasc Surg 2004; 37:702-6. 5. Cottin V, Plauchu H, Bayle JY, Barthelet M, Revel D, Cordier JF. Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. Am J Respir Crit Care Med 2004;169:994-1000. 6. Dines DE, Arms RA, Bernatz PE, Games MR. Pulmonaryarteriovenous fistulas. Mayo Clin Proc 1974;49:460 5. 7. White RI Jr, Lynch-Nylan A, Terry P, Buescher PC, Farmlett EJ, Charnas L, et al. Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology 1988;169:663 9. 8. Shannon T, Pollak J, White RI. Redistribution of pulmonary blood flow by embolotherapy: a new method for improving oxygenation in patients with diffuse pulmonary arteriovenous malformations [Abstract]. Am Rev Respir Dis 1992;145:600A. 9. Hepburn J, Dauphinee JA. Successful removal of hemangioma of lung followed by disappearance of polycythemia. Am J Med Sci 1942;204:681-7. 10. Wallenhaupt SL, D'Souza V. Combined radiological and surgical management of arteriovenous malformation of the lung. Ann Thorac Surg 1988;45:213-5. 11. Temes RT, Paramsothy P, Endara SA, Wernly JA. Resection of a solitary pulmonary arteriovenous malformation by video-assisted thoracic surgery. J Thorac Cardiovasc Surg 1998;116:878-9. 12. Thung KH, Sihoe AD, Wan IY, Lee TW, Wong R, Yim AP. Hemoptysis from an unusual pulmonary arteriovenous malformation. Ann Thorac Surg 2003;76: 1730-3. 13. Frughnan ME, Lui YW, Wirth JA, Pugash RA, Redelmeier DA, Hyland RH, et al. Diffuse pulmonary arteriovenous malformations: charecteristics and prognosis. Chest 2000;117:31-8. 14. Jeong WK, Jeon SC, Choi YW, Park CK, Hong EK, Yoon HJ, et al. Telangiectatic pulmonary arteriovenous malformation. J Thorac Imaging 200318:113-5. 15. Jeon YB, Lee JK, Kim JH, Lee DC, Kim DS. Two cases of diffuse type of pulmonary arteriovenous fistula. Korean J Med 1986;30:555-61. 590