150 소아외과 : 제 11 권제 2 호 2005 년 Vol. 11, No, 2, December 2005 소아요막관기형 성균관대학교의과대학삼성서울병원소아외과, 비뇨기과 * 강은영 이철구 박관현 * 서정민 이석구 서 론 요막관은태생기에방광이골반내로이동할때배꼽과연결되는구조물로태생 4 개월에서 5개월사이에폐쇄된다. 요막관폐쇄는요막관, 전복벽성장속도및방광하강속도에따라 4가지해부학적변형이유발된다 1,2. 요막관기형은출생후에도요막관이남아있는것으로개방성요막관 (patent urachus), 요막동 (urachal sinus), 요막관낭종 (urachal cyst), 방광게실 (bladder diverticula) 로분류되며, 증상이없는경우가대부분이나임상증상은다양한형태로발현된다. 소아요막관기형환자를분석하여요막관기형에대한임상양상및적절한진단방법의평가, 치료에대해고찰해보고자한다. 대상및방법 1995 년 3 월부터 2005 년 2 월까지성균관대 본논문의요지는 2005 년도 6 월 9 일서울에서개최된제 21 차소아외과학회춘계학술대회에서구연되었음. 교신저자 : 서정민, 135-710, 서울강남구일원동 50 번지성균관대학교의과대학삼성서울병원 Tel : 02)3410-0282, Fax : 02)3410-0040 E-mail: seojm@smc.samsung.co.kr 학교의과대학삼성서울병원에서요막관기형으로입원한환자 32명 ( 소아외과 : 25명, 비뇨기과 : 6명, 소아과 : 1명 ) 을대상으로성별, 연령별분포, 임상증상, 진단방법, 치료방법등을의무기록과조직병리기록, 검사결과를후향적분석하였다. 요막관기형은방광과배꼽사이에완전히교통되어있는개방성요막관, 배꼽에만교통이남아있는요막동, 방광과교통이있는방광게실, 양측으로교통되지않고탈락된상피세포로이루어진요막관낭종으로분류하였다 3. 결과요막관기형으로진단된 32명의성별및연령분포는남자 20명, 여자 12명으로남자가여자보다 1.67배많았다. 대상연령은출생직후부터 14세까지며, 1개월미만이 16 명 (50.0 %) 으로가장많은분포를차지하였다 ( 표 1). 요막관기형에따른분포는요막동 13예 (40.6 %), 요막관낭종 10예 (31.3 %), 개방성요막관 9예 (28.1 %) 의순으로조사되었다. 요막관기형은다양한임상증상으로발현되었으며, 배꼽분비물이 14예 (43.8 %), 배꼽
강은영외 : 소아요막관기형 151 Table 1. Age and Sex Distribution of Urachal Anomalies Age Male Female Total (%) <1m 11 5 16 (50.0) 1m~1yr 4 1 5 (15.6) 1yr~6yr 4 1 5 (15.6) 6yr~11yr 0 4 4 (12.5) 11yr~14yr 1 1 2 (6.3) Total 20 12 32 육아종이 8예 (25.0 %) 로가장많은비중을차지하였다. 그밖에복통과발열을동반한경우가 3예 (9.4 %), 발열을주소로입원한경우가 3예 (9.4 %), 복통으로발현된경우가 2예 (6.2 %), 복부종괴가 2예 (6.2 %) 로관찰되었다. 각각의유형별증상을살펴보면, 요막동 13예중 11예가배꼽분비물, 2예가배꼽육아종으로발현되었으며 ( 표 2), 개방성요 Table 2. Clinical Charateristics of Urachal Sinus Case Sex/Age Presentation Diagnosis Treatment Combined disorder 1 M/1d Umbilical discharge US, VCUG Excision None 2 M/1d Umbilical discharge US Excision None 3 M/1d Umbilical discharge US Excision None 4 F/9d Umbilical discharge US, Fistulography Excision None 5 M/10d Umbilical discharge US Excision None 6 M/13d Umbilical discharge US, VCUG Excision None 7 F/14d Umbilical discharge US, Fistulography Excision None 8 M/16d Umbilical discharge US Excision None 9 F/22d Umbilical granuloma US, VCUG Excision OMD 10 M/30d Umbilical granuloma US Excision None 11 F/2m Umbilical discharge US Excision None 12 F/9yr Umbilical discharge US Excision None 13 F/14yr Umbilical discharge US, Fistulography, CT Conservative None US;ultrasonography, CT;computed tomography, VCUG;voiding cystourethrography OMD;omphalomesenteric duct Table 3. Clinical Charateristics of Patent Urachus Case Sex/Age Presentation Diagnosis Treatment Combined disorder 1 F/prenatal Umbilical discharge US, VCUG Excision None 2 M/1d Umbilical granuloma intraoperative Excision None 3 M/1d Umbilical granuloma US Excision None 4 M/10d Umbilical granuloma US Excision None 5 F/30d Umbilical granuloma US Excision None 6 M/2m Umbilical discharge US, VCUG,Indigo Excision None 7 M/3m Umbilical granuloma US Excision None 8 M/4m Umbilical discharge US, VCUG Excision None 9 M/2yr Umbilical granuloma intraoperative Excision None US;ultrasonography, VCUG;voiding cystourethrography, Indigo;indigocarmine test
152 소아외과제 11 권제 2 호 2005 년 Table 4. Clinical Charateristics of Urachal Cyst Case Sex/Age Presentation Diagnosis Treatment Combined disorder 1 M/18d Fever US Excision None 2 M/2m Fever US, CT,VCUG Conservative None 3 F/2yr Abdominal pain, fever US, CT Excision None 4 M/3yr Fever US Excision None 5 M/4yr Abdominal pain US Excision None 6 M/4yr Abdominal mass US Excision None 7 F/7yr Abdominal pain, fever US, CT Conservative None 8 F/8yr Abdominal pain US, CT, VCUG Excision,App appendicitis 9 F/10yr Abdominal mass US, MRI, VCUG Excision None 10 M/13yr Abdominal pain, fever US, CT Excision None US;ultrasonography, CT;computed tomography, MRI;magnetic resonance imaging, VCUG;voiding cystourethrography, App;appendectomy 막관 9예중 6예가배꼽육아종, 3예가배꼽분비물로발현되었다 ( 표 3). 요막관낭종 10 예중복통과발열이동반된경우가 3예, 복통 2예, 발열 3예, 복부종괴 2예로다양한형태의증상이발현되었다 ( 표 4). 요막동환자에서는모두초음파가시행되었고, 그외에누관조영술이 3예, 배뇨방광조영술이 3예, 전산화단층촬영이 1예에서시행되었다 ( 표 2). 개방성요막관환자에서는초음파 7예, 배뇨방광조영술 3예, indigocarmine test 1예, 시험적개복술 (exploration) 2 예가시행되었다 ( 표 3). 요막관낭종환자에서는초음파 10예, 전산화단층촬영 5예, 자기공명영상 1예, 배뇨방광조영술 3예가시행되었다 ( 표 4). 총 32예중초음파가 30예에서시행되었으며, 초음파만으로요막관기형을확진한경우는 23예 (71.9% ) 였으며, 확진방법으로누관조영술 3예, 전산화단층촬영 3예, 자기공명영상 1예가시행되었다. 수술전소변배양검사를 13예에서시행하여 2예에서그람양성균주가배양되었고, 2예에서 Escherichia coli가배양되었다. 제대분비물에대한균배양검사를 7예에서시행하여 4 예에서 Staphylococcus aureus, 1예에서 Escherichia coli, 1예에서 β-hemolytic streptococcus Group A가배양되었고, 1예에서는자라나는균주가없었다. 요막관기형의치료는요막동 12예 ( 표 2), 개방성요막관 9예 ( 표 3), 요막관낭종 8예 ( 표 4) 에서근치적절제술이시행되었으며, 요막동 1예, 요막관낭종 2예에서보존적치료가시행되었다. 근치적절제술을받은 29 예모두조직검사결과요막관기형이확인되었고, 만성염증이동반된경우가 7예, 화농성염증이동반된경우가 4예에서관찰되었다. 대부분동반된기형이나질환이관찰되지않았으며, 요막동 1예에서배꼽창자간막관 (omphalomesenteric duct) 이동반되었고 ( 표 2), 요막관낭종 1예에서급성충수돌기염이동반되어충수돌기절제술을함께시행하였다 ( 표 4).
강은영외 : 소아요막관기형 153 고찰요막관은태생기방광이골반내로이동할때배꼽과연결되는구조물로정상적으로태생기 4개월에서 5개월사이에폐쇄되어섬유성띠로남게된다. 요막관의길이는 3-10 cm이고직경은 8-10 mm로, 구조적으로제방광근막 (umbilicovesical fascial sheath) 으로둘러싸여있으며외측으로배꼽동맥, 아래로방광이위치한다. 이공간은복막으로부터완전히분리되어감염또는종양이확산되는것을제한하게된다. 요막관은조직학적으로 3개의층으로바깥쪽은근육, 중간은결체조직및혈관, 안쪽으로는변형이형또는입방상피세포로구성되어있다 4. 요막관기형은출생후에도요막관이완전히폐쇄되지않고남아있는것으로폐쇄정도및양상에따라개방성요막관, 요막동, 요막관낭종, 방광게실등의네가지유형으로분류된다. 개방성요막관은방광과배꼽사이가완전히교통되어있으며, 요막동은배꼽에만교통이남아있는구조를보인다. 방광게실은방광과교통되어있고, 요막관낭종은양측으로폐쇄되어탈락된상피세포로이루어진낭을형성하게된다 3. 요막관기형의성별발생율은남자가여자보다 2배정도높은것으로보고되어있다 5. 본연구에서도총 32예중남자가 20예로여자에비해 1.67배높게관찰되었다. 개방성요막관은매우드물며 Nix등 5 (1958) 은 200,000명의입원환자중단지 3예만을보고했으며, 소아부검결과개방성요막관은 1:7610, 요막관낭종은 1:5000의비율로발견되었다 6. 유형별로는요막관낭종 (43-59 %) 과요막동 (29-43 %) 이가장흔하며, 개방성요막관 (10-15 %), 방광게실 (4-6 %) 순으로보고되었다 7. 본연구에서도요막동과요막관낭종이각각 13예 (40.6 %), 10예 (31.3 %) 로가장많은빈도로조사되었다. 요막관기형의증상은주로배꼽분비물 (42 %), 배꼽육아종 (33 %), 복통 (22 %) 으로발현되었으며, 배뇨통이 2% 로보고되고있다 7. 요막관낭종은주로배꼽아래복부종괴로만져지며염증이동반된경우복통을동반한고열이나배뇨시통증으로발현된다 8. 요막동은배꼽분비물로발현되며배꼽통증및육아종을동반하기도한다. 개방성요막관은복와위또는울거나배뇨시에배꼽에서소변이관찰되며, 배꼽탈장과연관되어배꼽부종이나종괴로나타날수도있다 9. 방광게실은대부분증상을유발하지않으나반복되는요로감염이있을경우의심해볼수있다 10. 본연구에서도배꼽분비물이 43.8 %, 배꼽육아종이 25.0 % 로가장많은빈도로관찰되었으며, 요막관낭종의경우주로복통과발열증상이나타났으며, 요막동과개방성요막관의경우주로배꼽분비물과배꼽육아종으로발현되었다. 요막관기형의진단은병력청취, 이학적검사, 적합한영상학적검사에의해이루어진다. 요막관낭종은주로초음파로진단되며방광과복막관계를파악할수있다 7. 전산화단층촬영은낭종의범위와인접구조물의침범정도를파악하는데종종유용하게이용되며, 경정맥신우조영술, 배뇨방광조영술, 방광경이방광벽의침범정도를파악하는데이용된다 3. 요막동은초음파검사및누관조영술로진단할수있다 7,11. 개방성요막관은배꼽염, 배꼽창
154 소아외과제 11 권제 2 호 2005 년 자간막관과의감별진단이필요하며, 배꼽분비물에서크레아티닌분석및누관조영술이감별진단에유용하다. 또한배뇨방광조영술은누관확인및방광출구폐쇄, 방광요관역류확인을위해필요하다 7. 방광게실진단에는배뇨방광조영술이최선의진단방법으로이용될수있다 12. 본연구에서도총 32예중 30예에서초음파가시행되었으며 23예 (71.9 %) 에서요막관기형을진단할수있었다. 요막관기형의합병증으로는감염이가장흔하며 9, 감염성요막관낭종에서분비물을배양검사한결과포도상구균이가장흔하다고보고되어있다 13. 본연구에서도제대분비물에대한배양검사가 7예에서시행되었고 4예에서 Staphylococcus aureus가동정되었다. 또한소아에서요막관악성종양이약 0.01 % 보고되어있다 14. 이는전체방광암 (0.34 %) 에비하여적은빈도이지만악성종양이요막관기형에서발생할수있다는 15,16 사실을간과해서는안될것이다. 요막관기형의치료로외과적절제및항생제치료, 경피적배액술이이용될수있으나, 근치적치료를위해외과적절제술이권고된다. 또한최근에는요막관기형의외과적절제술에복강경이시도되어성공한예가보고되고있다 17,18. 결론 1995년 3월부터 2005년 2월까지본원에서요막관기형으로진단된 32명의환자를분석한결과대부분 1개월미만에서발견되었으며, 증상은주로배꼽분비물또는배꼽육아종으로발현되었다. 요막관기형의진단은 정확한이학적검사와영상학적검사로이루어지며, 초음파가가장유용한검사방법이다. 요막관기형은드물게악성화가능성이있고, 감염으로인한합병증및재발방지를위해근치적절제술이권고된다. 또한요막관기형의치료에앞서동반기형에대한정확한평가가선행되어야할것이다. 참고문헌 1. Blichert-Toft M, Koch F, Nielsen OV: Anatomic variants of the urachus related to clinical appearance and surgical treatment of urachal lesions. Surg Gynecol Obstet 137:51-54, 1973 2. Bauer SB, Retik AB: Urachal anomalies and related umbilical disorders. Urol Clin North Am 5:195-211, 1978 3. Nagasaki A, Handa N, Kawanami T: Diagnosis of urachal anomalies in infancy and childhood by contrast fistulography, ultrasound and CT. Pediatr Radiol 21: 321-323, 1991 4. Hammond G, Yglesias L, Gavid JE: The urachus, its anatomy and associated fasciae. Anat Rec 80:271, 1941. 5. NIX JT, MENVILLE JG, ALBERT M, WENDT DL: Congenital patent urachus. J Urol 79:264-273, 1958 6. Rubin A: Handbook of congenital malformations. Philadelphia, WB Saunders, 1967, Pp334 7. Cilento BG, Jr., Bauer SB, Retik AB, Peters CA, Atala A: Urachal anomalies: defining the best diagnostic modality. Urology 52:120-122, 1998 8. 염규영, 박정양, 최한용 : 감염된요막관낭종 1 례, 대한비뇨회지 27:945-948, 1986 9. Mesrobian HG, Zacharias A, Balcom AH, Cohen RD: Ten years of experience with
강은영외 : 소아요막관기형 155 isolated urachal anomalies in children. J Urol 158:1316-1318, 1997 10. Ward TT, Saltzman E, Chiang S: Infected urachal remnants in the adult: case report and review. Clin Infect Dis 16:26-29, 1993 11. 서형석, 안종훈, 박대준, 신민식, 장대수 : 감염된요막관동치험 1례, 대한비뇨회지 27:752-756, 1986 12. Hernanz-Schulman M, Lebowitz RL: The elusiveness and importance of bladder diverticula in children. Pediatr Radiol 15: 399,1985. 13. MacMillan RW, Schullinger JN, Santulli TV: Pyourachus: an unusual surgical problem. J Pediatr Surg 8:387-9, 1973 14. Clapuyt P, Saint-Martin C, De Batselier P, Brichard B, Wese FX, Gosseye S: Urachal neuroblastoma: first case report. Pediatr Radiol 29:320-321, 1999 15. Henly DR, Farrow GM, Zincke H: Urachal cancer: role of conservative surgery. Urology 42:635-639, 1993 16. Sheldon CA, Clayman RV, Gonzalez R, Williams RD, Fraley EE: Malignant urachal lesions. J Urol 131:1-8, 1984 17. Khurana S, Borzi PA: Laparoscopic management of complicated urachal disease in children. J Urol 168:1526-1528, 2002 18. Groot-Wassink T, Deo H, Charfare H, Foley R: Laparoscopic excision of the urachus. Surg Endosc 14:680-681, 2000
156 소아외과제 11 권제 2 호 2005 년 Urachal Anomalies in Children Eun-Young Kang, M.D., Cheol-Koo Lee, M.D., Kwan-Hyeon Park*, M.D., Jeong-Meen Seo, M.D., Suk-Koo Lee, M.D. Devision of Pediatric Surgery, Department of Surgery and Department of Urology*, Samsung medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Failure of the urachus to regress completely results in anomalies that may be classified as patent urachus, urachal sinus, urachal cyst and bladder diverticula. The presenting symptoms of children with urachal anomalies are variable and uniform guidelines for diagnosis and treatment are lacking. The purpose of this study was to analyze our experience and develop conclusions regarding the presentation, diagnosis and treatment of urachal anomalies. We retrospectively analyzed the records of 32 patients who were admitted for urachal anomalies from March 1995 to February 2005. The age distribution of these patients at presentation ranged from 1 day to 14 years old (median age 1 month). There were 20 boys and 12 girls. The 32 cases comprised 13 cases of urachal sinus (40.6 %), 10 urachal cyst (31.3%), and 9 patent urchus (28.1%). In 30 patients ultrasonography was used for diagnosis and 2 patients with patent urachus were explored without using a diagnostic method. Twenty-three patients were confirmed by ultrasonography alone and 7 patients were examined using additional modalities, namely, computed tomography for 2 patients with an urachal cyst, magnetic resonance imaging for 1 patient with an urachal cyst, and fistulography for 3 patients with an urachal sinus. The presenting symptoms were umbilical discharge (14 patients), umbilical granuloma (8), abdominal pain and fever (3), fever (3), abdominal pain (2), and a low abdominal mass (2). Excision was performed in 29 patients, and 3 patients were conservatively managed. Urachal anomalies in children most frequently presented in neonates, and the most common complaint was umbilical discharge with infection. Urachal anomalies can be diagnosed by a physical examination and an appropriate radiographic test. Ultrasound was the most useful diagnostic method. Complete surgical excision of an urachal anomaly is recommended to avoid recurrence, and the rare development of carcinoma. (J Kor Assoc Pediatr Surg 11(2):150~156), 2005. Index Words:Congenital anomaly, Urachus, Sinus, Cyst, Patent Correspondence:Jeong-Meen Seo, M.D., Devision of Pediatric Surgery, Sungkyunkwan University School of Medicine, Samsung Medical Center, 50 Ilwon-Dong, Kangnam-Gu. Seoul 135-710, Korea Tel : 02)3410-0282, Fax : 02)3410-0040 E-mail: seojm@smc.samsung.co.kr