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Korean J Gastroenterol Vol. 65 No. 3, 177-181 http://dx.doi.org/10.4166/kjg.2015.65.3.177 pissn 1598-9992 eissn 2233-6869 CASE REPORT 간문맥내가스와장관포상기종으로발현한괴사성대장염 1 예 송종규, 구자설, 강효성, 박진용, 김승영, 현종진, 정성우, 이상우 고려대학교의과대학내과학교실 A Case of Necrotizing Colitis Presenting with Hepatic Portal Venous Gas and Pneumatosis Intestinalis Jong Gyu Song, Ja Seol Koo, Hyo Sung Kang, Jin Yong Park, Seoung Young Kim, Jong Jin Hyun, Sung Woo Jung and Sang Woo Lee Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea Hepatic portal venous gas is a very rare radiologic sign which is characterized by gas accumulation in the portal venous circulation. Pneumatosis intestinalis is also very rare and is characterized by multiple air cysts in the serosal or submucosal layers of the gastrointestinal tract walls. These two findings are caused by various pathological conditions and can develop individually or simultaneously. The latter is clinically more significant because it is frequently related to bowel ischemia or necrosis, and represents a poor prognosis. However, prognosis is more influenced by the severity of underlying disease rather than hepatic portal venous gas or pneumatosis intestinalis itself. If bowel ischemia or necrosis is the primary cause, emergency operation is very important to improve patient s prognosis. Herein, we report a case of necrotizing colitis presenting as hepatic portal venous gas and pneumatosis intestinalis which was successfully managed by early surgery. (Korean J Gastroenterol 2015;65:177-181) Key Words: Hepatic portal venous gas; Pneumatosis intestinalis 서론 간문맥내가스와장관포상기종은매운드문영상학적소견으로, 장허혈, 장괴사, 장폐쇄, 위장관팽창, 게실염, 바륨관장등다양한질환에의해발생할수있다. 1,2 두소견모두특별한치료가필요없는양성경과부터패혈증및사망에이를수있는치명적인경과까지다양한임상양상을보일수있다. 3-5 이러한임상경과는간문맥내가스또는장관포상기종자체보다는, 오히려원인질환의중증도에더영향을받는다. 3-5 두소견은단독으로나타나기도하며때로는함께발견되기도하는데, 특히두가지소견이함께나타날때는장허혈또는장괴사와관련된경우가많다. 3,6 이런경우원 인질환에대한평가가늦어진다면치명적인경과를보일수있기때문에조기진단과적극적인치료가중요하다. 3,6 저자들은간문맥내가스와장관포상기종이함께관찰되었던환자에서, 괴사성장염의심하에적극적으로시험적개복술을시도하여호전된증례를경험하였기에문헌고찰과함께보고하는바이다. 증례 52세남자가내원 1시간전수차례의오심, 구토후발생한토혈을주소로응급실에내원하였다. 과거력에서 30년간하루소주 2-3병가량의음주력외에특이소견은없었다. 내원 Received July 29, 2014. Revised September 11, 2014. Accepted September 16, 2014. CC This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/ by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Copyright 2015. Korean Society of Gastroenterology. 교신저자 : 구자설, 425-707, 안산시단원구적금로 123, 고려대학교안산병원소화기내과 Correspondence to: Ja Seol Koo, Division of Gastroenterology, Department of Internal Medicine, Korea University Ansan Hospital, 123 Jeokgeum-ro, Danwon-gu, Ansan 425-707, Korea. Tel: +82-31-412-5580, Fax: +82-31-412-5582, E-mail: jskoo@korea.ac.kr Financial support: None. Conflict of interest: None. Korean J Gastroenterol, Vol. 65 No. 3, March 2015 www.kjg.or.kr

178 송종규 등. 간문맥 내 가스와 장관 포상 기종으로 발현한 괴사성 대장염 Fig. 1. Plain abdomen radiography. (A) Initially, no remarkable findings are present. (B) After two days, numerous air bubbles are noted in the ascending and descending colon (arrows). Fig. 2. Abdominal computed tomography scan. (A) Numerous air densities are noted in the distal portal vein branches of the liver (short arrows), and gas is also seen in the extrahepatic portal vein (long arrow). (B) Gas is present within the inferior mesenteric vein (arrow). (C) Numerous air bubbles in the bowel wall are noted on descending and sigmoid colon (arrows). (D) Mild fluid collection and minimal amount of free air are observed in the left pericolic gutter (arrow). The Korean Journal of Gastroenterology

Song JG, et al. Necrotizing Colitis Presenting with Hepatic Portal Venous Gas and Pneumatosis Intestinalis 179 당시활력징후는혈압 105/72 mmhg, 맥박수 110회 / 분, 체온 36.0 o C, 호흡수 24회 / 분으로빈맥을보였다. 결막은창백하지않았고, 공막황달소견도없었다. 복부진찰소견에서장음은감소되어있었으며, 심와부와우상복부의압통이있었으나반발통은관찰되지않았다. 항문수지검사는특이소견없었다. 말초혈액검사에서백혈구 11,930/mm 3 ( 호중구 78.4%, 림프구 14.3%), 혈색소 14.7 g/dl, 혈소판 353,000/mm 3, 적혈구침강속도는 2 mm/hr였다. 혈청생화학검사에서 Na 137.7 mmol/l, K 4.15 mmol/l, Cl 93.7 mmol/l, BUN 19.0 mg/dl, creatinine 1.2 mg/dl였으며, 총빌리루빈 2.3 mg/dl, 직접빌리루빈 0.6 mg/dl, 알부민 3.3 g/dl, AST 1,048 IU/L, ALT 413 IU/L, amylase 231 IU/L, PT는 79.1% 였다. 소변검사와흉부 X선검사는정상이었다. 복부 X선검사에서도특이소견은없었다. 토혈에대해시행한상부위장관내시경검사에서, 위식도접합부에약 1 cm 가량의열상과함께활동성출혈이관찰되어 hemoclip 지혈술을시행하였다. 또한위각부에서출혈이없는활동성위궤양이관찰되었다. 내시경지혈술시행후환자는약간의심와부통증외에특이소견은없었다. 재출혈여부에대한경과관찰과, 간수치의상승, 급성췌장염의증에대한보존적치료를위해입원하였다. 다음날오전에간수치의상승에대해 B형간염표면항원항체검사를시행한결과모두음성이었으며, ELISA법으로시행한 C형간염항체검사는 weakly reactive (1.52) 로측정되었다. 복부초음파에서는중등도의지방간소견이관찰되었다. 환자의음주력과혈액검사, 초음파소견을통해알코올에의한급성간염과급성췌장염으로판단하여보존적치료를계획하였다. 저녁부터환자의복통이급격히악화되면서 39.2 o C의발열이발생하였다. 이외의활력징후는혈압 150/80 mmhg, 맥박수 140회 / 분, 27회 / 분으로빈맥을보였다. 신체진찰에서복부강직과함께복부전체에서심한압통과반발통이관찰되어, 다시복부 X선검사를시행한결과상행결장과하행결장에서다수의공기방울음영이관찰되었다 (Fig. 1B). 이에복부전산화단층촬영을시행하였고, 간피막 2 cm 이내에특징적으로관찰되는간주변부공기음영과간외문맥내의공기음영이관찰되어간문맥내가스를확인할수있었다 (Fig. 2A). 이러한공기음영은하장간막정맥으로부터이어지고있었다 (Fig. 2B). 또한, 상행결장, 하행결장, S자결장의장관벽공기음영이관찰되어장관포상기종도확인할수있었다 (Fig. 2C). 이밖에하행결장근위부주변에서복수와함께소량의자유공기가관찰되어미세천공이의심되었다 (Fig. 2D). 또한, 맹장과상행결장의장벽비후가관찰되었고, 대장벽의조영증강은뚜렷하지않았다 (Fig. 2B, C). 괴사성대장염을의심하여시험적개복술을시행하였고, 전대장에걸친출혈성장괴사가관찰되어전대장절제술및회장루형성술을 Fig. 3. Gross finding. Extensive necrosis and hemorrhagic infarction are seen on the entire colon. 시행하였다 (Fig. 3). 이후수술 43일째추적한복부전산화단층촬영결과간문맥내의공기음영은완전히소실되었고, 임상적호전을보여퇴원하였다. 고 찰 간문맥내가스는매우드문영상학적소견으로, 1955년 Wolfe와 Evans 7 의괴사성장염영아 6명에대한증례를통해처음보고되었다. 대개소아에서더흔히발생하나, 성인에서도발생할수있다. 8 성인에서는 1960년 Susman과 Senturia 에의해처음보고된후다양한증례가보고되면서, 간문맥내가스는장괴사, 복강내농양, 염증성장질환, 소화성궤양, 천공, 외상, 의인성손상등다양한병적상황에의해발생할수있다고알려졌다. 8 발병기전은아직불분명하며, 장관내압력증가, 장관점막의병적변화, 가스를형성하는세균감염등이원인으로제시되고있다. 1,9 장관포상기종은위장관벽의장막또는점막하층에다발성의공기로찬낭종이형성되는드문영상학적소견으로 1730년 Du Vernoi가부검을통해처음발견하였다. 10 장관포상기종은원인미상의, 대체로양성경과를보이는특발성장관포상기종과, 기저질환에속발하여나타나는이차성장관포상기종으로나뉘며, 각각의비율은대략특발성이 15%, 이차성이 85% 로보고하였다. 2,10 이차성장관포상기종을일으키는기저질환으로는항암치료나의인성원인 ( 바륨관장, 공장루관삽입등 ), 소화성궤양같은양성질환부터장괴사, 장허혈, 장폐쇄같은치명적인질환까지다양한원인들이보고되고있다. 2 기종형성기전으로는, 장점막손상으로발생한점막결손이있는상태에서질병이나의인성원인에의해장관내압력이증가하여장관내의공기가장관벽으로들어가기종을형성한다는물리적가설, 가스를생성하는장내세균이손상된장관점막을통해장관벽을침투하여기종을 Vol. 65 No. 3, March 2015

180 송종규등. 간문맥내가스와장관포상기종으로발현한괴사성대장염 형성한다는세균가설, 폐질환환자에서폐포파열에의한공기가혈관을따라장막하층에낭종을형성한다는폐가설이제시되고있으나정확한기전은불분명하다. 11 간문맥내가스의확인에가장유용한검사는복부전산화단층촬영이다. 9 간문맥내가스는복부전산화단층촬영에서간피막으로부터 2 cm 이내에서말초방향으로가지를치는듯한음영으로나타난다. 12 이는가스가간문맥혈관내에존재하면서, 혈관흐름이간문부에서말초방향으로원심성으로 (centrifugal) 흐르기때문이다. 12 이러한소견은기담관 (pneumobilia) 과의감별에도도움이되는데, 기담관의경우는담도내에존재하는가스이며, 담즙은말초에서간문부를향해구심성 (centripetal) 흐름을보이기때문에가스가간문부주변에형성된다. 12 장관포상기종은, 대장내시경에서장관내로돌출되어있는다발성종괴가관찰되며겸자로눌렀을때스펀지처럼들어가고, 낭종천자시공기가새면서찌그러지는것이특징이다. 13 그러나이증례와같이환자의전반적인상태가나쁘고급성경과를보여대장내시경확인이불가능한경우가많다. 장관포상기종의진단에가장빈번히이용되는검사는복부 X선검사와복부전산화단층촬영이다. 2 단순복부촬영이나복부전산화단층촬영에서장벽을따라나타나는선상또는거품형태의기체음영이장관벽에평행하게관찰되는것이특징이며, 대장조영촬영을할경우장관벽내의공기로인한충만결손등이관찰된다. 2 특히복부전산화단층촬영의경우장관포상기종의원인질환에대한부가적인정보를줄수있다는점에서유용하다. 2 간문맥내가스와장관포상기종모두질병이아닌영상학적소견으로, 이들의존재여부나침범범위의정도가환자의예후를반영하는것이아니라기저질환의중증도에의해환자의예후가결정된다. 2,12 원인질환에따라보존적치료만으로충분한경우에서패혈증과사망에이르는치명적인상황까지, 다양한임상경과를보일수있다. 2,12 특히두소견이동시에발견되는경우원인질환이장허혈이나괴사성장염인경우가많아임상적으로중요하다. 1,6 과거에는간문맥내가스의사망률이 75% 정도로매우높게보고되었으나최근복부전산화단층촬영에의한조기진단과조기치료가시행되고, 괴사성장염외에많은비치명적인원인들이밝혀지면서사망률은 29-39% 정도로보고된다. 9 그러나여전히괴사성장염이간문맥내가스의가장흔한원인이며, 특히장관포상기종이동반될경우괴사성장염이원인일가능성이높고사망률이 85% 로높기때문에두가지소견이모두관찰되면적극적인대처가필요하다. 12 복부전산화단층촬영만으로는간문맥내가스나장관포상기종이양성질환에의한것인지, 장허혈이나괴사성장염에의한것인지구별할수없으며임상증상과혈청생화학검사를종합하여판단해야한다. 12 지금까지국내에서보고된, 간문맥내가스와장관포상기종이동시에발현된증례는 7예가있었다 (Table 1). 14-19 7예모두복부전산화단층촬영을통해진단되었으며, 이중 4명이사망하여높은사망률을보였다 (57%). 원인은다양하였으며십이지장궤양천공, 괴사성장염, 급성췌장염, 혐기균감염, 기종성신우신염, 그리고임상경과가급격히진행되어원인을규명하지못했던 2예의증례가있었다. 괴사성장염에의한증례는 1예에불과하였으며, 이번증례와마찬가지로복부전산화단층촬영소견과신체검사를통해초기부터적극적으로괴사성장염을의심하여개복술을시행하여회복되었다. 이번증례의경우토혈을주소로내원하여식도열상증후군을내시경지혈술로치료한후관찰하던중환자의증상이발생하였다. 그렇기때문에즉각적인복부 X선촬영추적검사와복부전산화단층촬영을통해간문맥내가스와장관포상기종을비교적빠르게진단할수있었다. 비록복부전산화단층촬영에서장간막동맥색전이나폐쇄가보이지는않았으나 Table 1. Clinical Data of Seven Patients with Hepatic Portal Venous Gas and Pneumatosis Intestinalis Reported in Korea Report Sex/ age (yr) Clinical features Etiology Comorbidity Diagnostic modality Treatment Outcome Kim et al. 14 M/47 Abdominal pain, vomiting, Unknown DM, alcoholics CT Supportive Expired altered mentality Kim et al. 14 M/54 Abdominal pain Peptic ulcer None CT Surgery Expired perforation Jang and Lee 15 F/55 Diarrhea, vomiting, Necrotizing colitis DM, HTN, ESRD CT Surgery Recovered abdominal pain Park et al. 16 M/53 Fever, abdominal pain Acute pancreatitis Alcoholics CT Supportive Expired Yang et al. 17 F/83 Abdominal pain, vomiting Unknown HTN CT Supportive Expired Lee at al. 18 M/53 Abdominal pain Anaerobe infection DM, HTN, ESRD CT Supportive Recovered Yu et al. 19 F/87 Altered mentality Emphysematous pyelonephritis DM CT Supportive Recovered DM, diabetes mellitus; HTN, hypertension; ESRD, end stage renal disease. The Korean Journal of Gastroenterology

Song JG, et al. Necrotizing Colitis Presenting with Hepatic Portal Venous Gas and Pneumatosis Intestinalis 181 환자가임상적으로급속히악화되는점과신체진찰소견, 그리고간문맥내가스와장관포상기종이동시에나타난점, 그리고미세천공과복수가관찰된점을통해괴사성장염의가능성을의심하였고, 즉시시험적개복술을시도하여양호한경과를보였다. 결론으로간문맥내가스와장관포상기종은여러가지기저질환에의해나타날수있지만특히장괴사가많은빈도를차지하며, 사망률이매우높기때문에주의가필요하다. 12 복부전산화단층촬영과환자의증상, 기저질환에대한세심한병력청취와신체검사, 혈청생화학검사등을통하여장괴사의가능성에대해주의를기울여야하며, 특히장관포상기종이동시에보인다면더욱주의가필요하다. 12 만약임상적으로장괴사가의심된다면치명적인결과를초래할수있으므로초기에시험적개복술을포함한적극적인치료가시도되어야한다. 1,6 저자들은간문맥내가스와장관포상기종이동시에발견된환자에서장괴사를의심하여, 적극적인수술적치료로회복한환자를경험하였기에문헌고찰과함께보고한다. REFERENCES 1. Peloponissios N, Halkic N, Pugnale M, et al. Hepatic portal gas in adults: review of the literature and presentation of a consecutive series of 11 cases. Arch Surg 2003;138:1367-1370. 2. Ho LM, Paulson EK, Thompson WM. Pneumatosis intestinalis in the adult: benign to life-threatening causes. Am J Roentgenol 2007;188:1604-1613. 3. Ito M, Horiguchi A, Miyakawa S. Pneumatosis intestinalis and hepatic portal venous gas. J Hepatobiliary Pancreat Surg 2008; 15:334-337. 4. Babaei M, Malek F, Mousavi S, Malek M. Pneumatosis cystoides intestinalis with hepatic portal venous gas: a rare finding with good outcome. Case Rep Clin Pract Rev 2004;5:369-372. 5. Wiesner W, Mortelé KJ, Glickman JN, Ji H, Ros PR. Pneumatosis intestinalis and portomesenteric venous gas in intestinal ischemia: correlation of CT findings with severity of ischemia and clinical outcome. Am J Roentgenol 2001;177:1319-1323. 6. Koami H, Isa T, Ishimine T, et al. Risk factors for bowel necrosis in patients with hepatic portal venous gas. Surg Today 2015; 45:156-161. 7. Wolfe JN, Evans WA. Gas in the portal veins of the liver in infants; a roentgenographic demonstration with postmortem anatomical correlation. Am J Roentgenol Radium Ther Nucl Med 1955;74:486-488. 8. Gan HN, Tan KY, Chong CK, Tay KH. Finding hepatic portal venous gas in an adult patient: its significance. Singapore Med J 2006;47:814-816. 9. McElvanna K, Campbell A, Diamond T. Hepatic portal venous gas--three non-fatal cases and review of the literature. Ulster Med J 2012;81:74-78. 10. Wu LL, Yang YS, Dou Y, Liu QS. A systematic analysis of pneumatosis cystoids intestinalis. World J Gastroenterol 2013;19:4973-4978. 11. Azzaroli F, Turco L, Ceroni L, et al. Pneumatosis cystoides intestinalis. World J Gastroenterol 2011;17:4932-4936. 12. Abboud B, El Hachem J, Yazbeck T, Doumit C. Hepatic portal venous gas: physiopathology, etiology, prognosis and treatment. World J Gastroenterol 2009;15:3585-3590. 13. Adar T, Paz K. Images in clinical medicine. Pneumatosis intestinalis. N Engl J Med 2013;368:e19. 14. Kim JO, Kim KH, Sohn DK, Kim AJ, Kim TK. Pneumatosis cystoides intestinalis with portal venous gas: two case reports. J Korean Soc Emerg Med 2004;15:47-50. 15. Jang HJ, Lee J. Pneumatosis intestinalis and hepatic portal venous gas caused by necrotizing colitis. Korean J Gastroenterol 2006;47:407-408. 16. Park HC, Lee WS, Joo SY, et al. Hepatic portal venous gas associated with acute pancreatitis: reports of two cases and review of literature. Korean J Gastroenterol 2007;50:131-135. 17. Yang SY, Lee YJ, Park SY, et al. Hepatic portal venous gas. J Korean Geriatr Soc 2010;14:48-52. 18. Lee HM, Cho SY, Lim YS, Kim YK, Song HC, Choi EJ. Pneumatosis intestinalis with hepatic portal venous gas in a hemodialysis patient. Korean J Nephrol 2011;30:546-550. 19. Yu SH, Eom YS, Lee DM, et al. A case of pneumatosis cystoides intestinalis and portal venous gas accompanied by emphysematous pyelonephritis in type 2 diabetes mellitus. J Korean Diabetes 2014;15:45-50. Vol. 65 No. 3, March 2015