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1 ORIGINAL ARTICLE Korean J Obstet Gynecol 2011;54(7): doi: /KJOG pissn eissn COMPARISON OF PRENATALLY DIAGNOSED FETAL CONGENITAL CYSTIC ADENOMATOID MALFORMATION AND BRONCHOPULMONARY SEQUESTRATION Ju Hyun Ryu, MD, Hyun-Hwa Cha, MD, Suk-Joo Choi, MD, Soo-Young Oh, MD, Cheong-Rae Roh, MD, Jong-Hwa Kim, MD Department of Obstetrics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Objective This study is to investigate and compare the pregnancy outcomes and prognostic factors of congenital cystic adenomatoid malformation (CCAM) and bronchopulmonary sequestration (BPS). Methods From May 2005 to September 2010, fifty-five medical records of fetuses with prenatally diagnosed CCAM (42 cases) and BPS (13 cases) were reviewed retrospectively in Samsung Medical Center (SMC). We compared the demographic characteristics, sonographic findings, postnatal diagnoses and pregnancy outcomes between CCAM and BPS. The sonographic findings included the locations of lesion, changes of size, existences of mediastinal shift, associated anomalies and existences of hydrops. Postnatal diagnoses were confirmed by pathologic findings and image examinations. Results A total of fifty-five cases were prenatally diagnosed as CCAM (42 cases) and BPS (13 cases). Thirty cases were followed up in SMC. Three of the thirty cases underwent termination and one case was intrauterine fetal death. Twenty-six cases were delivered alive in SMC. The masses were found out to be disappeared by prenatal sonography in four cases (15.4%) who were antenatally diagnosed as CCAM. The sizes of the mass were decreased more than 50% in CCAM seven cases and BPS three cases. The absence of mediastinal shift (P=0.019) was significantly correlated with the decrease of mass size. Conclusion The concordance rate between antenatal and postnatal diagnosis was 63.0%. There was a tendency of decreasing in lesion with most of the cases. Without mediastinal shift, the mass size decreased significantly. So, existence of mediastinal shift is the most important prognostic factor of CCAM and BPS. Keywords: Congenital cystic adenomatoid malformation of lung; Bronchopulmonary sequestration; Prenatal diagnosis; Pregnancy outcome 산전초음파의발달로태아폐종양의진단율이높아지고있다. 산전초음파에서태아의폐종양으로진단될수있는질환으로는선천성고기관지폐쇄증후군 (congenital high airway obstruction syndrome, CHAOS), 기관지폐분리증 (bronchopulmonary sequestration, BPS), 선천성낭성샘모양기형 (congenital cystic adenomatoid malformation, CCAM) 등이있으며, 가장흔한질환은선천성낭성샘모양기형과기관지폐분리증이다. 선천성낭성샘모양기형은폐의발달과정이상에의해생성되는선천성기형으로, 말단세기관지 (terminal bronchiole) 의과성장을특징으로한다. 발생빈도는 35,000명당 1명에서 11,000명당 1명까지다양하게보고되며 [1-6] 삼성서울병원에서의최근 5년간발생률은 0.15% 로조사 Received: Accepted: Corresponding author: Jong-Hwa Kim, MD Department of Obstetrics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-dong, Gangnam-gu, Seoul , Korea Tel: Fax: jh369.kim@samsung.com This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Copyright Korean Society of Obstetrics and Gynecology 335

Transverse sonogram of the fetal chest shows a multicystic lung mass of congenital cystic adenomatoid malformation (white arrow). H, heart. 2.

2 KJOG Vol. 54, No. 7, 2011 다고하였다 [7]. 저자들이조사한결과에의하면전세계적으로선천성폐종양의산전연구대부분에서, 증례의수는 30예미만이었고, 30예이상의증례를보고한연구는 8개에불과하다 [8]. 본연구에서는산전초음파를통하여선천성낭성샘모양기형또는기관지폐분리증으로진단된 55예중 30 예를추적관찰하여임신결과를비교하였고병변의크기감소에영향을미치는예후인자를제시하고자하였다. Fig. 1. Transverse sonogram of the fetal chest shows a multicystic lung mass of congenital cystic adenomatoid malformation (white arrow). H, heart cm 2.73 cm Fig. 2. Transverse sonogram of the fetal chest shows an echogenic lung mass of bronchopulmonary sequestration (white dotted line). 되었다. 주로임신이삼분기에초음파검사에서다수의낭종소견으로발견되며 (Fig. 1) 생존율이 95% 까지보고되어비교적예후가좋은질환이다. 기관지폐분리증은정상적인폐와분리되어있는비기능성폐조직이, 호흡기계와교통하지않으면서체순환계로부터혈액공급을받는선천성기형이며초음파에서흉강내고에코성덩어리의소견으로관찰된다 (Fig. 2). 발생빈도는 50,000명당 1명이하로보고되고 [1-6] 삼성서울병원의최근 5년간발생률은 0.07% 였다. 최근보고된연구에의하면선천성낭성샘모양기형과기관지폐분리증은자궁내자연소실률이 43-86% 이며, 자연소실된경우에서예후는좋 년 5월부터 2010년 9월까지삼성서울병원에서산전초음파를통해선천성낭성샘모양기형또는기관지폐분리증으로진단된 55예를대상으로임신부및신생아의임상기록을조사하여후향적연구를시행하였다. 55예중 30예는본원에서추적관찰이이루어졌으며, 27예는임신을유지하였고, 3예는임신중절을시행하였다. 임신부의특성은나이, 산과력, 진단당시의임신주수에대해기술하였고산전초음파소견은추정진단, 종격동이동여부, 연관기형, 태아수종동반여부, 병변의크기, 위치등을조사하였다. 선천성낭성샘모양기형은낭종의크기를 Stocker 등 [2] 의분류에따라 2 cm 이상의거대낭종이나여러개낭종으로구성되어있는 type I, 2 cm 이하의여러개소낭으로구성되어있는 type II, mm의미세낭종이나낭성병변이없는균질한고에코성병변으로구성된경우 type III로분류하였다 [9]. 분만후병변의소실이나최대장축값의감소를조사하였고, 병변크기의감소는진단당시크기에비해 50% 이상감소한것을기준으로하였다. 30% 감소를기준으로해서조사한결과는통계학적으로의미있게나온결과가없어 50% 를기준으로하여연구를진행하였다. 산전초음파검사에의한추정진단과산후신생아의영상의학적검사나수술후조직학적소견으로확인된최종진단을비교하여진단의일치율을얻었다. 연구결과의통계분석은 Student t-test, Mann-Whitney U-test, Chisquare test, Fisher s exact test를사용하였다. P 값이 0.05 미만인경우를통계학적유의성이있는것으로간주하였다. CCAM과 BPS군간에임신부연령, 산과력, 처음진단된임신주수, 분만임신주수의유의한차이는없었다 (Table 1). Fig. 3은산전초음파검사를통해선천성낭성샘모양기형또는기관지폐분리증으로진단된 55예의임신결과에대한모식도이다. 55예중 30예에서추적관찰이가능하였으며임신중절을한 3예를제외하고 27예중 18예는산전초음파검사에서선천성낭성샘모양기형으로진단되었고, 9예는기관지폐분리증으로진단되었다. 기관지폐분리증으로진단된 9예중 1예는 27주에자궁내사망하였다. 산후의영상의학적검사와수술시병리소견을통해산전진단과비교한결과본연구에서의산전진단의민감도는 CCAM 336

3 Ju Hyun Ryu, et al. Comparison of congenital cystic adenomatoid malformation and bronchopulmonary sequestration Table 1. Demographic characteristics between prenatally diagnosed CCAM and BPS CCAM (n=42) BPS (n=13) Mean age (yr) 31.83± ±5.96 Parity Primipara (n) 26 (61.9%) 7 (53.8%) Multipara (n) 16 (38.1%) 6 (46.2%) GA at diagnosis (wk) 23.06± ±1.52 GA at birth (wk) 36.84± ±2.55 Birth weight (g) 2,270±1,000 3,310±710 CCAM, cystic adenomatoid malformation; BPS, bronchopulmonary sequestration; GA, gestational age. TOP = 3 n = 55 Birth = 27 (Live birth = 26; FDIU = 1) F/U loss = 25 Fig. 3. Overall of pregnancy outcome of 55 cases prenatally diagnosed congenital cystic adenomatoid malformation and bronchopulmonary sequestration. F/U, follow-up; TOP, termination of pregnancy; FDIU, fetal death in uterus. Table 2. Diagnostic sensitivity and specificity and predictive value of CCAM and BPS Prenatally diagnosed CCAM (n) BPS (n) Postnatally CCAM (n) 14 4 Diagnosed BPS (n) 6 3 Sensitivity value: CCAM 0.7 (70.0%); BPS 0.42 (42.9%). Speciticity value: CCAM 0.42 (42.9%); BPS 0.7 (70.0%). Positive predictive value: CCAM 0.78 (77.8%); BPS 0.67 (66.7%). Pegative predictive value: CCAM 0.33 (33.3%); BPS 0.78 (77.8%). CCAM, congenital cystic adenomatoid malformation; BPS, bronchopulmonary sequestration. 70%, BPS 42.9% 였고특이도는 CCAM 42.9%, BPS 70.0% 였다. 양성예측도는 CCAM 77.8%, BPS 66.7%, 음성예측도는 CCAM 33.3%, BPS 77.8% 였다 (Table 2). 산전초음파검사에서선천성낭성샘모양기형이의심된 42예중, 태아수종은 5예에서발견되었으며기관지폐분리증이의심된 13예중에서는 1예에서발견되었다. 기관지폐분리증과태아수종이동반되었던 1예는임신 27주에자궁내태아사망하였다. Table 3. Clinical characteristics between prenatally diagnosed CCAM and BPS CCAM n (%) BPS n (%) Total 42 (76.4) 13 (23.6) Mass Location Left 15 (35.7) 8 (61.5) Right 27 (64.3) 5 (38.5) Type of CCAM I 9 (21.4) II 17 (40.4) III 13 (30.9) NA 3 (7.1) Size at diagnosis (cm) 3.31± ±0.93 Size at birth (cm) 1.63± ±0.64 Decreasing size (cm) Spontaneous regression 6 (14.3) 0 F/U loss 22 (52.4) 3 (23.1) Mediastinal shift Yes 25 (59.5) 6 (46.2) No 17 (40.5) 7 (53.8) Hydrops Yes 5 (11.9) 1 (7.7) No 37 (88.1) 12 (92.3) Associated anomalies Yes 5 (11.9) 0 (0) No 37 (88.1) 13 (100) FDIU 0 (0) 1 (7.7) CCAM, congenital cystic adenomatoid malformation; BPD, bronchopulmonary sequestration; NA, not available; F/U: follow-up; FDIU, fetal death in uterus. 진단시종격동이동은선천성낭성샘모양기형의 59.5% (25/42), 기관지폐분리증의 46.2% (6/13) 에서발견되었다. 병변의위치는 CCAM의경우오른쪽폐에병변이있는경우가 27예 (64.3%) 로왼쪽보다많았고, BPS는왼쪽이 8예 (61.5%) 로오른쪽보다많았다. CCAM과 BPS 모두양쪽폐에병변이있는경우는없었다. CCAM을 Stocker의분류에따라기술하였으며 type II가 40.4% 로가장많았다 (Table 3). 병변의크기는 CCAM의경우처음진단시 3.31 ± 1.38 cm에서분만시 1.63 ± 0.56 cm으로 1.68 cm 감소하였고, BPS는 3.28 ± 0.98 cm에서 2.86 ± 0.64 cm으로 0.42 cm 감소해서 CCAM의크기가더많이감소한것으로나타났다 (Table 3). CCAM 이나 BPS 로진단된후 25 예에서추적관찰이소실되었으며, 추 337

4 KJOG Vol. 54, No. 7, 2011 적관찰이이루어진 30예중 3예는임신중절을시행하였으며, 1예는 27 주에자궁내태아사망하였고나머지 26예는정상분만하였다. 정상분만한 26예를분석한결과 4예에서분만전에병변이완전소실되었다. 10 예에서 50% 이상의병변크기감소가있었고이중 CCAM이 7예, BPS 가 3예있었다. CCAM 7예중 2예에서출생이후병변의완전소실이있었다. 나머지 CCAM 7예, BPS 5예에서는병변크기의변화가 50% 이내이거나약간증가한것으로나타났다. CCAM 18예중종격동이동이동반되지않은 10예중 5예가 50% 이상의크기감소가있었고 BPS 9예중종격동이동이동반되지않은 3예에서 1예가 50% 이상의크기감소가나타났다. 이에 50% 이상병변크기의감소에영향을미치는인자를분석한결과종격동이동이동반되지않은경우에서통계학적으로유의하게병변크기가감소하는것으로나타났다 (P=0.019). 진단시평균주수는 CCAM ± 2.79주, BPS ± 1.52주였으며평균분만주수는 CCAM ± 5.72주, BPS ± 2.55주였다. 분만시신생아의평균체중은 CCAM 2,770 ± 1,000 g, BPS 3,310 ± 710 g 였고이러한차이는 CCAM이 BPS보다이른주수에분만이이루어졌기때문으로생각된다. 분만시병변의평균크기는 CCAM 1.63 ± 0.56 cm, BPS 2.86 ± 0.64 cm였으며 CCAM 5예에서동반된기형이있었고이중 1예에서태변마개증후군 (meconium plug syndrome) 이동반되어장절제술을시행하였고기관지폐분리증은동반기형이없었다 (Table 3). 출생후신생아의경과를분석한결과분만전까지완전소실된경우는 4예, 출생후완전소실된경우가 2예있었고, 6예모두산전에선천성낭성샘모양기형으로진단되었다. 출생후수술을시행한경우는선천성낭성샘모양기형 6예, 기관지폐분리증 2예있었고 4예의선천성낭성샘모양기형, 2예의기관지폐분리증에서흉부단층촬영등을이용한추적관찰중이며추적관찰기간동안양호한예후를보였다 (Table 4). 본연구에서산전진단과산후진단의일치율은 63.0% 였으며 3 예의임 Table 4. Neonatal outcomes (n=26) CCAM (n) BPS (n) Regression of mass at birth 4 0 Regression of mass during postnatal period 2 0 Postnatal operation 6 2 Ongoing F/U 4 2 F/U loss after birth 3 3 CCAM, congenital cystic adenomatoid malformation; BPS, bronchopulmonary sequestration; F/U, follow-up. 신중절을제외하고임신을지속한 27예중기관지폐분리증으로진단된 1예는자궁내태아사망하였고 26예는모두생존하여생존율은 96.3% 였다. 외국의문헌에따르면 CCAM의사망률은 9-49% 이고, BPS의사망률은다양하게보고되고있으나태아수종이동반되지않는경우에서는예후가좋은것으로알려져있다 [10]. 본연구에서는산전에태아수종이나복수가동반된경우가 CCAM 5예, BPS 1예있었고 BPS 1예는자궁내태아사망하였다. CCAM 5예중 1예는분만하여현재추적관찰중이며 4예는추적관찰되지않았다. Roberts 등 [11] 의보고에따르면선천성낭성샘모양기형과 recombinant chromosome 18이연관성이있다고하였고, 동반되는소견으로기관지폐분리증, 양수과다증, 태아수종, 거대태반등이있다고하였다 [11]. 연관기형으로는신장기형, 복벽이상, 중추신경계이상, 척추이상, 소화기계이상등이있을수있다 [11]. 본연구에서는 CCAM 5예에서 mega cisterna magna, bowel obstruction, adrenal gland cyst 등의동반기형이있었으나염색체이상은발견되지않았다. 문헌에따르면 CCAM에서 bowel obstruction이동반되는이유는병변의 mass effect 때문일것으로추측하고있다 [11]. 선천성낭성샘모양기형의크기는보통 28주에최대성장에이르고, 29주이후에는약 20-40% 에서크기가감소하는것으로알려져있다 [12-16]. 추적관찰중병변이소실된경우에도출생후반드시 computerized tomography (CT) 와같은영상검사를시행할것이권고되는데 Illanes 등 [17] 은병변이소실된 21예에서도흉부 CT에서이상이발견된경우가 14예 (67%) 였다고보고하였다. 본연구에서는병변이소실된경우에서생후에 CT를시행하였고이상이발견된예는없었다. Stocker 등 [2] 은 CCAM을낭종의크기에따라 3가지유형으로분류하였고 type I에서예후가좋다고하였는데본연구에서는 type과예후와의연관성은발견하지못하였다. 이러한결과는대상이된수가적어서통계학적으로의미있는결과가나오지않은것으로생각된다. 본연구결과 CCAM은오른쪽, BPS는왼쪽에병변이있었던경우가더많았으나 Cavoretto 등 [8] 의연구에서는 CCAM이왼쪽에서더많이발견되었고, BPS의경우도왼쪽이더많았다. 그러나양쪽에병변이있었던경우는없었고다른문헌들에서도병변이한쪽폐엽에국한되는경향을보였다 [9]. 병변의위치가예후에미치는영향에대해서는아직정확히밝혀진기전이없어추후연구를지속한다면의미있는결과를얻을수있을것으로생각된다. 전세계적선천성폐덩어리의산전연구대부분에서증례의수가 30 예미만이었던것에비해본연구에서는증례의수가 55예였고추적관찰이가능했던경우도 30예에달했다는점에서의의가있다 [8]. 최근의선천성폐덩어리에관한연구에서종격동이동과예후와의의미있는연관성을보고한연구는없었으나 [17,18], 본연구에서는종격동이동이동반되지않은 CCAM이나 BPS에서의미있게병변크기가감소하거나소실되는결과를보였다. 이러한결과는산전에 CCAM 혹은 BPS로진단된산모들의산전상담에유용한지표로사용될수있을것으로생각된다. 그러나본연구에서는태아수종등의다른요인들과병변크기감소 338

5 Ju Hyun Ryu, et al. Comparison of congenital cystic adenomatoid malformation and bronchopulmonary sequestration 와의연관성을찾지못하였고, 병변의크기변화를장축의길이로만비교하였다는제한점이있다. 향후더많은 CCAM, BPS 환자군을대상으로임신결과와예후인자들에대한연구가필요할것으로생각된다. References 1. Ahn HK, Han JY, Choi JS, Lim HJ, Moon MJ, Jung JH, et al. Pregnancy outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation. Korean J Obstet Gynecol 2003;46: Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Classifi cation and morphologic spectrum. Hum Pathol 1977;8: Wilson RD, Hedrick HL, Liechty KW, Flake AW, Johnson MP, Bebbington M, et al. Cystic adenomatoid malformation of the lung: review of genetics, prenatal diagnosis, and in utero treatment. Am J Med Genet A 2006;140: Yildirim G, Güngördük K, Aslan H, Ceylan Y. Prenatal diagnosis of an extralobar pulmonary sequestration. Arch Gynecol Obstet 2008;278: Plattner V, Haustein B, Llanas B, Allos N, Vergnes P, Héloury Y. Extra-lobar pulmonary sequestration with prenatal diagnosis. A report of 5 cases and review of the literature. Eur J Pediatr Surg 1995;5: Dolkart LA, Reimers FT, Helmuth WV, Porte MA, Eisinger G. Antenatal diagnosis of pulmonary sequestration: a review. Obstet Gynecol Surv 1992;47: Lee HJ, Song MJ, Cho JY, Lee YH. Echogenic fetal lung masses: comparison of prenatal sonographic and postnatal CT findings. J Clin Ultrasound 2003;31: Cavoretto P, Molina F, Poggi S, Davenport M, Nicolaides KH. Prenatal diagnosis and outcome of echogenic fetal lung lesions. Ultrasound Obstet Gynecol 2008;32: Thorpe-Beeston JG, Nicolaides KH. Cystic adenomatoid malformation of the lung: prenatal diagnosis and outcome. Prenat Diagn 1994;14: Davenport M, Warne SA, Cacciaguerra S, Patel S, Greenough A, Nicolaides K. Current outcome of antenally diagnosed cystic lung disease. J Pediatr Surg 2004;39: Roberts D, Sweeney E, Walkinshaw S. Congenital cystic adenomatoid malformation of the lung coexisting with recombinant chromosome 18. A case report. Fetal Diagn Ther 2001;16: Mann S, Wilson RD, Bebbington MW, Adzick NS, Johnson MP. Antenatal diagnosis and management of congenital cystic adenomatoid malformation. Semin Fetal Neonatal Med 2007;12: Adzick NS. Management of fetal lung lesions. Clin Perinatol 2003;30: MacGillivray TE, Harrison MR, Goldstein RB, Adzick NS. Disappearing fetal lung lesions. J Pediatr Surg 1993;28: Miller JA, Corteville JE, Langer JC. Congenital cystic adenomatoid malformation in the fetus: natural history and predictors of outcome. J Pediatr Surg 1996;31: Kunisaki SM, Barnewolt CE, Estroff JA, Ward VL, Nemes LP, Fauza DO, et al. Large fetal congenital cystic adenomatoid malformations: growth trends and patient survival. J Pediatr Surg 2007;42: Illanes S, Hunter A, Evans M, Cusick E, Soothill P. Prenatal diagnosis of echogenic lung: evolution and outcome. Ultrasound Obstet Gynecol 2005;26: Oh KJ, Yang HJ, Lee KA, Lee SM, Lee JH, Park CW, et al. Pregnancy outcomes of antenatally diagnosed congenital cystic adenomatoid malformation of lung. Korean J Obstet Gynecol 2009;52:

6 KJOG Vol. 54, No. 7, 2011 산전진단된태아의선천성낭성샘모양기형과기관지폐분리증의비교 성균관대학교의과대학산부인과학교실류주현, 차현화, 최석주, 오수영, 노정래, 김종화 목적산전에선천성낭성샘모양기형과기관지폐분리증으로진단된증례들의임신결과를비교하고예후인자를알아보고자하였다. 연구방법 2005년 5월부터 2010년 9월까지성균관대학교삼성서울병원에서산전에선천성낭성샘모양기형과기관지폐분리증으로진단된 55예를대상으로임신부및신생아의의무기록을후향적으로조사하여산모의특성, 산전초음파소견, 분만후임신결과와신생아결과를비교하였다. 출생후진단은조직학적검사및영상의학검사를기준으로하였다. 결과산전에선천성낭성샘모양기형과기관지폐분리증으로총 55예가진단되었고, 선천성낭성샘모양기형이 42예 (76.4%), 기관지폐분리증이 13 예 (23.6%) 였다. 종격동이동은선천성낭성샘모양기형 25예, 기관지폐분리증 6예에서관찰되었다. 본원에서분만이이루어진총 27예중자궁내태아사망 1예를제외한 26예에서병변이완전소실된경우가 4예 (15.4%) 였고모두산전에선천성낭성샘모양기형으로진단되었다. 50% 이상병변크기의감소가있었던경우는선천성낭성샘모양기형 7예, 기관지폐분리증 3예였고, 증가한경우는선천성낭성샘모양기형에서만 3예, 크기의변화가 50% 이내였던경우는선천성낭성샘모양기형 4예, 기관지폐분리증 5예로나타났다. 병변의소실이나 50% 이상크기가감소한경우에영향을미치는예후인자를조사한결과종격동이동 (P=0.019) 유무가병변크기와유의한상관관계가있었다. 결론본연구에서산전과출생후진단의일치율은 63.0% 였다. 산전초음파에서종격동이동이없었던경우는선천성낭성샘모양기형 59.5%, 기관지폐분리증 46.2% 였고, 이경우병변의크기가의미있게감소하였다. 중심단어 : 선천성낭성샘모양기형, 기관지폐분리증, 산전진단, 임신결과, 예후인자 340

012임수진

012임수진 Received : 2012. 11. 27 Reviewed : 2012. 12. 10 Accepted : 2012. 12. 12 A Clinical Study on Effect of Electro-acupuncture Treatment for Low Back Pain and Radicular Pain in Patients Diagnosed with Lumbar