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1 154 소아외과 : 제 16 권제 2 호 2010 년 Vol. 16, No. 2, December 2010 폐분리증의치료와흉강경적절제술의경험 울산대학교의과대학서울아산병원소아청소년병원소아외과 조민정 김태훈 김대연 김성철 김인구 서 론 절제술에대해보고하고자한다. 폐분리증은폐동맥이아닌전신순환계에서혈액공급을받는비기능성폐조직이다. 무증상에서출생직후의호흡부전, 이후소아에서감염으로인한폐렴까지다양한임상양상을나타내며최근에는산전검사의발달로증상이없는신생아에서의진단율이높아지고있다 1-2. 치료는고식적으로외과적절제라고알려져있으나실제임상에서는관찰및동맥색전술, 그리고수술적치료가명확한기준없이행해지고있다 3-6. 수술을시행한예들은이전대부분흉부외과에서개흉으로폐엽절제술을시행하였으나특히무증상의소아에서개흉폐엽절제술은아직도수술을많이꺼려하는이유이다. 저자들은폐분리증을진단받은환자들의임상양상및경과, 치료방법을분석하고특히저자들이시행한 3예의흉강경을이용한폐분리증 본논문의요지는 2010 년 6 월 11 일대구에서개최된제 26 회대한소아외과학회춘계학술대회에서구연되었음. 접수일 : 10/7/15 게재승인일 : 10/9/13 교신저자 : 김대연, 서울송파구풍납동 서울아산병원소아외과 Tel : 02) , Fax : 02) kimdy@amc.seoul.kr 대상및방법 1999년 4월부터 2010년 2월까지서울아산병원소아청소년병원에서폐분리증을진단받은만 15세미만 96명의환자를대상으로하였다. 의무기록을바탕으로후향적연구를시행하였고, 경과관찰및동맥색전술을시행한환자들은 CT에서, 수술을시행한환자들은수술후조직검사에서폐분리증을진단받았다. 이전경과관찰이나색전술을받았더라도이후수술이나다시색전술을받은경우최종치료를기준으로분류하였다. 폐분리증의유형은장측흉막 (visceral pleura) 을기준으로내측에위치하는엽내폐분리증 (intralobular type) 과외측에위치하는엽외폐분리증 (extralobular type) 으로분류하였다. 동맥색전술은생후 5일이내의환자는제대동맥을이용하였고, 5일이상의환자는대퇴동맥을통하여시행하였다. 개흉폐엽절제술은본원흉부외과에서시행하였고흉강경적절제술 3예는본과에서시행하였다.

2 조민정외 : 폐분리증의치료와흉강경적절제술의경험 155 결과 96명의환자중남자는 59명여자는 37명이었고평균추적기간은 24.5개월 (1개월 - 96 개월 ) 이었다. 81명은산전검사에서진단받았고평균진단주수는 24주였다, 이들의출생시재태연령과체중의중간값은각각 38주 (14주 - 36주 ), 3780 gm (1044 gm gm) 으로정상신생아들과차이는없었다. 11명은출생이후폐렴으로, 4명은흉부방사선이상소견으로검사중진단받았다. 27명은경과관찰중 1명에서폐분리증이자연소실되었고 10명은추적소실 (follow up loss) 되었다. 39명은동맥색전술을시행하였고이후 3명에서완전소실을보였으며 14 명은도중추적소실되었다 ( 그림 1). 수술당시나이는개흉절제술이가장많았고 (14.5개월, 2일-13세 ) 색전술은대개생후 5 일이내시행되었다 ( 표 1). 경과관찰환자들과흉강경적절제술을받은환자들은모두무증상이었고색전술과개흉수술전각각 3예, 4예에서출생시호흡부전을보였다. 폐분리증의크기는경과관찰군에서 32 mm 로가장작았고폐분리증의유형은의미있는차이는없었으나개흉수술을받은환자에서엽내폐분리증 (intralobular type) 이 2배이상많았다. 개흉수술을받은환자에서수술후상처감염을보인것이외합병증은없었으나, 색전술후 2예에서패혈증소견을보였으며 1예에서신동맥색전증으로신농양이발생하였다. 1예에서는시술중 coil이장골동맥에위치하여제거하지못한상태로대퇴동맥맥박이감소하였고 urokinase사용후증상및혈류가호전되었다. 30예에서수술을시행하였고이중 4명은이전색전술을하였으나경과관찰중낭성 Fig. 1. Course of Each Therapeutic Group

3 156 소아외과제 16 권제 2 호 2010 년 부분이커지거나동맥혈류의재유입이관찰되어수술을시행하였다. 27예는흉부외과에서개흉으로폐엽절제술 16예, 분리증절제술 6예, 폐분엽절제술 5예를시행하였고, 3 예는본소아외과에서수술하였고엽내폐분리증 1예는흉강경으로좌하엽절제술을시행하였고 1예는엽외폐분리증과동측의횡격막탈장이동반되어있어흉강경으로폐분 리증절제술, 횡격막탈장복원술을시행하였다 ( 표 2). 1예는좌측횡격막직하방에위치한엽외폐분리증으로흉강경으로흉곽내를확인한후복강경으로전환해폐분리증을절제하였다. 개흉수술과흉강경수술에서각각평균수술시간 133분과 167분, 흉관제거일은 4.5일과 2.3일, 입원기간은 8일, 7일을보였다. Table 1. Demographics Observation (n=27) Average Age at Tx Respiratory distress (n) Type (n) (intralobular/ extralobular) Average size of sequestration (mm) Combined anomaly (n) Embolization (n=39) 5d (1d 15yr) Thoracotomy (n=27) 14.5m (2d 13yr) Thoracoscopic (n=3) d 11/16 20/19 20/8* 1/ Ebstein s anomaly (1), ASD (1) 0 CDH (2) Coarctation (1) Imperforate anus (1) CDH (1) Complication (n) Abbreviations: CDH, congenital diaphragmatic hernia; ASD, artrial septal defect. *: one patient had both intra and extralobular type Table 2. Review of Thoracoscopic Resection; 3 cases Case 1 Case 2 Case 3 Type Intralobular Extralobular with Extralobular diaphragmatic hernia (infradiaphragmatic) Op name Left lower lobectomy Excision & diaphragmatic hernia repair Excision Age at Tx 15m (7d, embolization) 23d 11d Feeding artery Aorta Celiac trunk Celiac trunk Op time (min) Chest tube remove (d) 3 2 2

4 조민정외 : 폐분리증의치료와흉강경적절제술의경험 157 고찰폐분리증은발생빈도가 0.15 %-1.7 % 라고알려져있는드문선천성폐질환이다 7. 원인은아직도명확히밝혀진바가없으며비정상적인혈관기형, 기관지의변형, 혹은기관지의폐쇄에서기인한분리등의가설이제기되고있다 가장흔한합병증은감염및반복적인폐렴이며, 본연구에서는없었지만태아유미흉, 심부전이나태아흉수, 객혈을유발하기도한다 전장과의교통 (foregut communications), 기관지원성낭종 (bronchogenic cysts), 낭성선종양기형 (cystic adenomatoid malformations, CCAM) 과만도증후군 (scimitar syndrome) 등의동반기형이 50 % 까지보고되고있고 13,14, 선천성횡격막탈장이가장흔한동반기형이다 15. 최근낭성폐병변의치료로태아수술, 태아흉수에대해흉강양막강단락형성 (thoracoamniotic shunting), 동맥색전술, 수술등다양한방법들이시행되고있지만 11,16,17, 정립된치료적지침은없는상태이다. 특히산전검사에서진단되는비율이높아지고무증상의신생아환자들이증가하면서이들을어떻게치료할것인가에대한관심이늘고있다. 본연구에서도 81명 (84.4 %) 환자들이산전에진단되었고출생시호흡부전을보인 7명, 출생후폐렴으로진단된 11명을제외한 78명 (81.3 %) 의환자가무증상이었다. 몇몇연구에서는폐분리증을포함해태아폐병변이출생전혹은후에자연소실되는예들을보고하고있으나 드물며, 본연구에서도경과관찰중단한명에서영상학 적진단상자연소실을보이고있다. 본연구에서는경과관찰 27명중 10명, 색전술후병변이남아있는환자 35명중 14명이추적소실을보여보호자의순응도가좋지않았다. 1/3이상의환자들이병변이남아있는상태로외래내원을중단하였지만대부분무증상의신생아여서보호자들의순응도가낮은것으로생각된다. 색전술의가장큰문제는이러한낭성병변에서병리학적결과를확인할수없다는점이다. 낭성선종양기형은악성종양과관련이있다는보고가있고 21,22, 낭성선종양기형과폐분리증은구분도어려울뿐아니라동반되어있는경우가많기때문에 23 정확한병리학적결과를확인하지않는것은위험한일이다. 본연구에서도 7예는낭성선종양기형이동반되었고 2예는경과관찰중, 1예는색전술후낭성선종양기형이의심되고있다. 색전술후완전소실되는예는 63 % 라고보고한문헌도있지만 3 본연구에서는 3예만관찰되었다. 흔하지는않지만색전술후심각한합병증이발생하기도한다 5. 본연구에서도수술보다색전술후합병증이더많았으며전신혈관계를접근하는방법이므로색전술자체가신생아들에게색전증혹은패혈증을유발할수있어비교적침습적인시술로여겨진다. 따라서저자들은본연구와여러문헌들을고찰해볼때수술로서폐분리증을절제하는것이가장효율적이며안전한치료라고생각한다. 다만수술시기에대해서는아직논란이많다. 생후 3개월이내흉강경적절제술을제안하는연구도있고 24, 평균증상발현시기가 7개월정도로생각되어 10

5 158 소아외과제 16 권제 2 호 2010 년 개월-1년이내수술을선호하는저자들도있다 1,23,25. 또다른연구에서는크기와증상에따라수술시기를권유하고있다 6. 조기수술을주장하는근거는앞서논한합병증이외에도생후 5년이내까지폐분리증이차지하고있는공간만큼새로운폐포형성과성장이지속되는것을방해하기때문이다 5. 저자들역시출생시호흡부전증상이있는경우와크기를정확히정의하기어렵지만 3 cm이상의폐분리증, feeding혈관이굵은경우는합병증발생을고려해신생아시기혹은진단즉시수술하는것이좋다고생각된다. 3 cm이하의무증상환자들은 1세경수술시기를고려하고있으나이부분에대해서는앞으로좀더많은연구가필요하다. 저자들이경험한흉강경을이용한폐분리증절제술은적은수이나신생아와영아에서도안전하게시행하였다. 특히반흔을줄일수있다는점에서보호자들의만족도가매우높고무증상환자들의수술을결정할때보호자들이쉽게수긍하며순응도를높이는요인이될수있다. 개흉수술의단점인반흔과통증이외에도익상형견갑골 (winged scapula) 이나흉곽의불균형등흉곽의근골격계발달과관련된문제, 척추측만증과유방모양의비정상발달을피할수있다 21,26. 개흉과흉강경을비교한연구에서흉관제거일이나입원기간은개흉과흉강경군에서비슷하거나흉강경군에서더짧다고보고되고있고 본과에서시행한흉강경절제예에서도큰차이는없었다. 하지만저자들이경험한 3예는저자들의초기경험이어서수술시간은개흉보다더길었고향후에는개흉보다수술시간과재원일수를줄 일수있을것으로기대된다. 결 론 폐분리증의치료로서경과관찰이나색전술은병소의완전소실을기대하기어렵고병리학적진단을확인할수없으며드물지만색전술후중증의합병증이발생할수있다. 폐분리증의치료는외과적절제가일차적으로고려되어야하며특히많은수의환자가신생아인것을고려할때흉강경적절제술의확대가예상된다. 참고문헌 1. Stanton M, Njere I, Ade-Ajayi N, Patel S, Davenport M: Systematic review and meta-analysis of the postnatal management of congenital cystic lung lesions. J Pediatr Surg 44(5): , Davenport M, Warne SA, Cacciaguerra S, Patel S, Greenough A, Nicolaides K: Current outcome of antenally diagnosed cystic lung disease. J Pediatr Surg 39(4): , Curros F, Chigot V, Emond S, Sayegh N, Revillon Y, Scheinman P, Lebourgeois M, Brunelle F: Role of embolisation in the treatment of bronchopulmonary sequestration. Pediatr Radiol 30(11): , Chowdhury M, Samuel M, Ramsay A, Constantinou J, McHugh K, Pierro A: Spontaneous postnatal involution of intraabdominal pulmonary sequestration. J Pediatr Surg 39(8): , Lee BS, Kim JT, Kim EA, Kim KS, Pi SY, Sung KB, Yoon CH, Goo HW: Neonatal pulmonary sequestration: clinical

6 조민정외 : 폐분리증의치료와흉강경적절제술의경험 159 experience with transumbilical arterial embolization. Pediatr Pulmonol 43(4): , Becmeur F, Horta-Geraud P, Donato L, Sauvage P: Pulmonary sequestrations: prenatal ultrasound diagnosis, treatment, and outcome. J Pediatr Surg 33(3): , Weinbaum PJ, Bors-Koefoed R, Green KW, Prenatt L: Antenatal sonographic findings in a case of intra-abdominal pulmonary sequestration. Obstet Gynecol 73(5 Pt 2): , Becmeur F, Horta P, Donato L, Christmann D, Sauvage P: Accessory diaphragm--review of 31 cases in the literature. Eur J Pediatr Surg 5(1):43-47, Clements BS, Warner JO: Pulmonary sequestration and related congenital bronchopulmonary-vascular malformations: nomenclature and classification based on anatomical and embryological considerations. Thorax 42(6): , Morin L, Crombleholme TM, Louis F, D'Alton ME: Bronchopulmonary sequestration: prenatal diagnosis with clinicopathologic correlation. Curr Opin Obstet Gynecol 6(5): , Park ST, Yoon CH, Sung KB, Yoon HK, Goo DE, Kim KS, Pi SY, Auh YH: Pulmonary sequestration in a newborn infant: treatment with arterial embolization. J Vasc Interv Radiol 9(4): , Rubin EM, Garcia H, Horowitz MD, Guerra JJ, Jr.: Fatal massive hemoptysis secondary to intralobar sequestration. Chest 106(3): , Bratu I, Flageole H, Chen MF, Di Lorenzo M, Yazbeck S, Laberge JM: The multiple facets of pulmonary sequestration. J Pediatr Surg 36(5): , Felker RE, Tonkin IL: Imaging of pulmonary sequestration. AJR Am J Roentgenol 154(2): , Rammos KS, Foroulis CN, Rammos CK, Andreou A: Prenatal interventional and postnatal surgical therapy of extralobar pulmonary sequestration. Interact Cardiovasc Thorac Surg 10(4): , Tsao K, Albanese CT, Harrison MR: Prenatal therapy for thoracic and mediastinal lesions. World J Surg 27(1):77-83, Nicolini U, Cerri V, Groli C, Poblete A, Mauro F: A new approach to prenatal treatment of extralobar pulmonary sequestration. Prenat Diagn 20(9): , Laberge JM, Bratu I, Flageole H: The management of asymptomatic congenital lung malformations. Paediatr Respir Rev 5 Suppl A:S , Garcia-Pena P, Lucaya J, Hendry GM, McAndrew PT, Duran C: Spontaneous involution of pulmonary sequestration in children: a report of two cases and review of the literature. Pediatr Radiol 28(4): , Winters WD, Effmann EL, Nghiem HV, Nyberg DA: Disappearing fetal lung masses: importance of postnatal imaging studies. Pediatr Radiol 27(6): , Bianchi A, Sowande O, Alizai NK, Rampersad B: Aesthetics and lateral thoracotomy in the neonate. J Pediatr Surg 33(12): , d'agostino S, Bonoldi E, Dante S, Meli S, Cappellari F, Musi L: Embryonal rhabdomyosarcoma of the lung arising in cystic adenomatoid malformation: case report and review of the literature. J Pediatr Surg 32(9): , 1997

7 160 소아외과제 16 권제 2 호 2010 년 23. Samuel M, Burge DM: Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation. Thorax 54(8): , Jesch NK, Leonhardt J, Sumpelmann R, Gluer S, Nustede R, Ure BM: Thoracoscopic resection of intra- and extralobar pulmonary sequestration in the first 3 months of life. J Pediatr Surg 40(9): , Cano I, Anton-Pacheco JL, Garcia A, Rothenberg S: Video-assisted thoracoscopic lobectomy in infants. Eur J Cardiothorac Surg 29(6): , Chetcuti P, Myers NA, Phelan PD, Beasley SW, Dickens DR: Chest wall deformity in patients with repaired esophageal atresia. J Pediatr Surg 24(3): , Diamond IR, Herrera P, Langer JC, Kim PC: Thoracoscopic versus open resection of congenital lung lesions: a casematched study. J Pediatr Surg 42(6): , Albanese CT, Sydorak RM, Tsao K, Lee H: Thoracoscopic lobectomy for prenatally diagnosed lung lesions. J Pediatr Surg 38(4): , Koontz CS, Oliva V, Gow KW, Wulkan ML: Video-assisted thoracoscopic surgical excision of cystic lung disease in children. J Pediatr Surg 40(5): , 2005

8 조민정외 : 폐분리증의치료와흉강경적절제술의경험 161 Treatment of Pulmonary Sequestration with Thoracoscopic Approach Min Jeng Cho, M.D., Tae Hoon Kim, M.D., Dae-Yeon Kim, M.D., Seong-Chul Kim, M.D., In-Koo Kim, M.D. Division of Pediatric Surgery, University of Ulsan College of Medicine & Asan Medical Center, Seoul, Korea Pulmonary sequestration (PS) is a rare congenital malformation of the lower respiratory tract. The anomaly is characterized by absence of communication with the tracheobronchial tree and isolated blood supply from an anomalous systemic vessels. With the utilization of antenatal ultrasound, the diagnosis of asymptomatic neonatal PS has increased. Treatment options include observation, arterial embolization and surgical resection. The aim of the present study is to review the clinical course of PS and to share our experience with thoracoscopic resection. A total of 96 patients with PS were treated at Asan Children s Hospital between 1999 and The diagnosis of PS was established by CT in the cases managed by observation or embolization, and by tissue pathology in the surgical cases. Medical records and radiographic images were retrospectively reviewed. Thirty-nine patients were managed by embolization and 30 patients by surgery. The remaining 27 patients have been under observation without any procedures. Among 27 observation patients, 1 patient regressed completely and 10 patients were lost to follow up. Of the 39 embolizations patients, 2 had their lesion regress and sepsis was suspected after embolization. In 1 patient, the microcoil migrated to the iliac artery during the embolization procedure, and another patient developed renal abscess caused by renal artery embolization. Among 30 surgical cases, resection by thoracotomy was performed in 27 at the Department of Thoracic Surgery, and thoracoscopic resection in 3 at the Division of Pediatric Sugery. Only one wound complication ocurred. We conclud that surgical excision should be recommended for pulmonary sequestration, whether the sequestration is symptomatic or not because of the risk of infection, the low rate of natural regress, poor compliance, severe complications after embolization, and to exclude other pathology. In summary, thoracoscopic resection of the pulmonary sequestration is feasible, efficacious, safe and cosmetically superior even in neonatal period. (J Kor Assoc Pediatr Surg 16(2):154~161), Index Words:Pulmonary sequestration, Asymptomatic, Thoracoscopic resection Correspondence:Dae-Yeon Kim, M.D., Division of Pediatric Surgery, University of Ulsan College of Medicine & Asan Medical Center, 388-1, Poongnap-Dong, Songpa-Ku, Seoul , Korea Tel : 02) , Fax : 02) kimdy@amc.seoul.kr

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<30382EC1F5B7CA2DB1E8BFB5C7D12E687770> Korean Journal of Obstetrics and Gynecology Vol. 53 No. 1 January 2010 산전초음파에서종격동종괴소견을보인폐분리증 1 예 연세대학교의과대학산부인과학교실 황유임 손가현 김영한 권자영 박용원 A case of pulmonary sequestration mimicking mediastinal mass detected