선천성간내문맥정맥단락 1 이웅희 김영통 조성식 신형철 간문맥과간정맥및하대정맥과연결을보이는간내문맥정맥단락은선천성이거나간경변증과문맥고혈압에의한후천성일수있다. 선천성간내문맥정맥단락은주로소아에서생기며자연소실될수있다. 저자들은신생아와성인의선천성간내문맥정맥단락 5예를보고한다. 선천성간내문맥정맥단락은간문맥과간정맥혹은대정맥사이에이상연결을보이는질환으로난황정맥 (vitelline vein) 과제대정맥 (umbilical vein) 의비정상적인발생과정에의해생기며주로신생아에서보고되고있으며 (1-5) 드물게는성인에서도보고된다 (6-8). 선천성간내문맥정맥단락의일부는 2년이내자연소실되며, 단락의양이 60% 이상이면간성뇌병증을유발한가능성이높아수술이나코일색전술을시행한다 (9). 선천성간내문맥정맥단락은드물지만최근영상기법들이발달하고검사가늘어나면서그보고는늘어나는추세이다. 저자들은신생아와성인의선천성간내문맥정맥단락 5예를보고하고자한다. 의 4명은좌간엽의원위부에간내문맥정맥단락이있었고이중 3명은중정맥과좌문맥 (Fig. 1), 1명은좌정맥과좌문맥사이에단락이있었다. 이들중 1명은좌간엽에다발성의단락이보였다 (Fig. 2). 증례 5의성인환자는좌문맥과좌정맥이직접연결되는정맥관이남아있는형태의간내문맥정맥단락을보였다 (Fig. 3). 단락이있는간정맥은 5명중 3명은중정맥, 2명은좌정맥이었다. 간문맥은모두좌문맥의제대부분 (umbilical portion) 과연결이있었다. 신생아의 2명에서만추적검사를시행하였는데증례 1의환아는 3개월후초음파에서단락이소실되었고증례 2의환아는 6개월후에시행한 CT에서간내문맥정맥단락이자연소실되었다 (Fig. 1E) (Table 2). 이두환아는 초기에황달과패혈증으로입원하여치료받고치유가되어퇴 증례보고 원을했으며추적검사를시행할당시에는특별한문제는없었 다. 5명의환자중 4명은신생아시기에진단되었고 1명은 57세에진단되었다. 신생아 4명 ( 증례1-4) 모두생후 10일이내에시행한복부초음파로진단되었다. 신생아의 4명모두황달증 고 찰 상이있었고, 2명에서패혈증의증세가있었고, 혈액검사는황달과패혈증에해당하는검사만을시행하였고암모니아와갈락토즈에대한검사는시행하지않았다. 2예에서동맥관개존증이동반되었다 (Table 1). 57세여자환자 ( 증례 5) 는상복부동통을주소로내원하였고위내시경에서위폴립이있었다. 시행한 CT에서담석이있었고, 좌정맥과좌문맥사이의직접적인이상단락을보였는데 CT에서간경화의소견이없고혈액검사에서간이상의소견이없어간이상을동반하지않은정맥관잔존 (patent ductus venosus) 으로진단하였다. 신생아의 4명은초음파와 CT를시행하였으며성인환자는 CT만시행하였다. 5명모두좌간엽에단락을보였다. 신생아 문맥정맥단락은간내와간외로나뉠수있고간내문맥정맥단락은선천성혹은후천성으로생길수있으며후천성은간경화혹은문맥고혈압에의해생길수있다 (2). 선천성간내문맥정맥단락은발생과정에서난황-제대정맥총 (vitelloumbilical venous plexus) 의비정상적인합류에의해만들어진다 (2). 선천성간내문맥정맥단락의빈도가드물다고하지만최근영상기술이발달하면서그보고들이증가하고있다. Park 등 (1) 은만성간질환이있는 59세의남자에서생긴간내문맥정맥단락을보고하면서이전에발표된간내문맥정맥단락의증례들을재검토하여간내문맥정맥단락을 4가지형태로분류하였는데 1형은우문맥과하대정맥사이에하나의큰 단락, 2형은간분절에서문맥과간정맥의원위부분지사이에 1 순천향대학교천안병원영상의학과 이논문은 2008년 8월 16일접수하여 2008년 10 월 14일에채택되었음. 하나혹은다발성단락, 3형은원위부간문맥과간정맥사이에 405
이웅희외 : 선천성간내문맥정맥단락 누형성, 4형은간전체에원위부간문맥과간정맥간의다발성단락을보인다. 이중 1형이가장흔하다고한다. 저자들의증례 1, 2, 3은 2형에해당되며증례 4는 4형에해당되며증례 5 는정맥관잔존으로 Gallego 등 (2) 은정맥관잔존을제5형으 로분류하였다. 하지만, 선천성간내문맥정맥단락으로보고하고있는증례들을보면우리논문과같이 2형과정맥관잔존이많다 (4-6, 9). 이러한결과는위의분류가선천성뿐아니라간질환에의해발생할수있는후천성을모두포함하여분류한 C D Fig. 1. Intrahepatic portosystemic shunt between middle hepatic vein and left portal vein (Case 2).. Ultrasonography shows dilated peripheral branches of left portal vein (white arrow) and middle hepatic vein (black arrow).. Ultrasonography shows abnormally dilated anechoic vascular structures (arrowheads) in the periphery of the medial segment of left hepatic lobe. C. Oblique coronal image shows communication (arrowheads) between the peripheral branches of left portal vein (white arrow) and middle hepatic vein (black arrow). D, E. xial CT scan (D) shows dilated vessels in left hepatic lobe, which disappeared on follow up CT 6 months later (E). E 406
것이기때문에다소빈도에차이가있는것으로생각된다. 정맥관은태아순환의일종으로생후바로닫히기시작하여 15-20일경에완전히닫힌다. 좌문맥과좌정맥혹은하대정맥사이의태아순환으로간혹성인에서보고되고있다 (8). 대부분의선천성간내문맥정맥단락은신생아에서보고되고있지만 40세여자환자에서정맥관잔존을, 59세여자환자에서우정맥과우간정맥사이의루를보고하고있다 (6, 7). 선천성간내문맥정맥단락의진단은초음파로쉽게가능하다. 초음파에서문맥과정맥사이에비정상적인낭성혹은관상음영이보이고도플러초음파에서관상음영이혈관임을확인할수있다. 도플러초음파와 iodine-123 iodoamphetamine 을이용한동위원소검사로단락의양을측정할수있다고한다. CT에서문맥과정맥사이의기형적인혈관연결을볼수있다. 선천성간내문맥정맥단락을가진환자는대부분이증상 Table 1. Clinical Manifestations in 4 Cases of Neonate with Congenital Intrahepatic Portosystemic Shunts Case No 1 2 3 4 ge at diagnosis 10 days 7 days 8 days 10 days Sex M M F M Patient s information at birth Gestational age 36 weeks 37 weeks 39 weeks ody weight 2700 gm 3240 gm 2550 gm 2770 gm Delivery method Caesarean section vaginal delivery vaginal delivery Caesarean section Symptom at admission sepsis yellowish skin color poor feeding tachypnea bnormal lab findings Total/direct bilirubin(mg/dl) 15.6/1.5 22.3/1.5 17.5/1.2 23.6/4.5 ST/LT*(IU/L) 153/42 41/13 99/45 - C-reactive protein(mg/l) 96 0.01 22.3 - ssociated anomaly Patent ductus arteriosus - Patent ductus arteriosus, trial septa defect ST/LT*: spartate aminotransferase/lanine aminotransferase Table 2. CT Findings of 5 Cases of Congenital Intrahepatic Portosystemic Shunts Case No Imaging Findings Follow up 1 Communication between peripheral branches of middle hepatic vein and left portal vein disappeared 3 months later 2 Connection between peripheral branches of middle hepatic vein and left portal vein disappeared 6 months later 3 Communication between peripheral branches of left hepatic vein and left portal vein - 4 Multiple communications between peripheral branches of middle/left hepatic vein and - left portal vein 5 Connection between left hepatic vein and left portal vein - Fig. 2. Multiple intrahepatic portosystemic shunts between middle/left hepatic vein and left portal vein (Case 4).,. xial CT scans show multiple communications (arrowheads) between the peripheral branches of middle/left hepatic veins and left portal vein (P). M: middle hepatic vein, L; left hepatic vein 407
이웅희외 : 선천성간내문맥정맥단락 Fig. 3. Patent ductus venosus between left hepatic vein and left portal vein in a 51-year-old woman (Case 5).. xial CT scan shows prominent vasculature (curved arrow) from the umbilical portion of left portal vein (white arrow).. xial CT scan shows early filling of left hepatic vein (black arrow) through shunt from left portal vein. Right and middle hepatic veins are not filled with intravascular contrast media. C. Coronal image shows direct communication (curved black arrow) between left portal vein (white arrow) and left hepatic vein (black arrow). ecause hepatic contour and margin was intact with homogeneous hepatic attenuation, it was diagnosed as patent ductus venosus, not associated with chronic liver disease or portal hypertension. Patent ductus venosus is one of congenital intrahepatic portosystemic shunt. C 이없으나, 과암모네미아, 갈락토세미아, 그리고간성뇌병증을나타낼수있다 (2). 신생아에서황달과폐혈증은흔한임상증세이므로우리논문의환아들이간내문맥정맥단락이황달이나패혈증과의직접적인연관성을알수없다. 그리고과암모 결론적으로선천성간내문맥정맥단락은문맥과간정맥사이의비정상적인연결을보이는혈관기형으로신생아에서주로보이지만성인에서도보일수있다. 다발성으로보이기도하고추적검사에서자연소실되기도한다. 네미아와갈락토세미아에대한혈액검사를시행하지않았기때문에연관성을알수없다. 성인환자와자연소실된 2예의신생아는간성뇌병증을동반하지않았다. 참 고 문 헌 선천성간내문맥정맥단락이선천성심장결손과관련이있 1. Park JH, Cha SH, Han JK, Han MC. Intrahepatic portosystemic venous shunt. JR m J Roentgenol 1990;155:527-528 거나간의비정상적인분엽, 간모세포암, 그리고간외담도폐 2. Gallego C, Miralles M, Marin C, Muyor P, Gonza lez G, Garcia- 쇄와같은간담도이상과연관이있을수있다고한다. 그리고 Hidalgo E. Congenital hepatic shunts. Radiographics 2004;24:755-51명의환자중 12명에서진단당시간성뇌병증이있었다고하였다. 간성뇌병증은환자의나이와단락의양에따라좌우되는데단락의양이 60% 이상이거나환자의나이가많을수록간성뇌병증의가능성이증가한다고한다 (10). 선천성간내문맥정맥단락의장기간추적을보면 15% 에서 772 3. Stringer MD. The clinical anatomy of congenital portosystemic venous shunts. Clin nat 2008;21:147-157 4. Lewis M, quino NM. Congenital portohepatic vein fistula that resolved spontaneously in a neonate. JR m J Roentgenol 1992; 159:837-838 5. Saxena K, Sodhi KS, rora J, Thapa R, Suri S. Congenital intrahepatic portosystemic venous shunt in an infant with down syn- 생후 2년이내에소실되었다고한다 (10). 본연구의 2예에서만추적검사를시행하였지만 2예에서 6개월이내에단락이자 drome. JR m J Roentgenol 2004;183:1783-1784 연소실되었다. 6. Oguz, kata D, alkanci F, khan O. Intrahepatic portosystemic 408
venous shunt: diagnosis by colour/power Doppler imaging and three-dimensional ultrasound. r J Radiol 2003;76:487-490 7. Soon MS, Chen YY, Yen HH. Hepatobiliary and pancreatic: spontaneous intrahepatic portosystemic venous shunt. J Gastroenterol Hepatol 2006;21:918 8. Merkle EM, Gilkeson RC. Remnants of fetal circulation: appearance on MDCT in adults. JR m J Roentgenol 2005;185:541-549 9. Kim IO, Cheon JE, Kim WS, Chung JW, Yeon KM, Yoo SJ, et al. Congenital intrahepatic portohepatic venous shunt: treatment with coil embolisation. Pediatr Radiol 2000;30:336-338 10. Uchino T, Matsuda I, Endo F. The long-term prognosis of congenital portosystemic venous shunt. J Pediatr 1999;135:254-256 J Korean Radiol Soc 2008;59:405-409 Congenital Intrahepatic Portosystemic Shunts 1 Woong Hee Lee, M.D., Young Tong Kim, M.D., Sung Shick Jou, M.D., Hyeong Cheol Shin, M.D. 1 Department of Radiology, Cheonan Hospital, Soonchunhyang University Intrahepatic portosystemic shunts are an anomalous connection between the portal vein and hepatic vein/ivc, which may be either congenital or acquired secondary to liver cirrhosis or portal hypertension. Cases of congenital intrahepatic shunts are usually encountered in children and may spontaneously resolve. We report 5 cases of congenital intrahepatic portosystemic shunts in neonates and an adult. Index words : Portosystemic shunt, surgical ddress reprint requests to : Young Tong Kim, M.D., Department of Radiology, Cheonan Hospital, Soonchunhyang University 23-20 ongmyung-dong, Cheonan 330-721, Korea Tel. 82-41-570-3515 Fax. 82-41-579-9026 E-mail: ytokim@schch.co.kr 409