Budd-Chiari Syndrome Due to Antithrombin, Protein C and Protein S Dficiency and the Complete Obstruction of SVC

In this case, a 39 year-old man was admitted with Budd-Chiari syndrome associated with complete superior vena cava(SVC) obstruction causing general edema and hepatic failure. Conservative medical therapy was failed. And after the radiologist failed to invasive procedure of balloon dilatation, we attempted the inferior vena cava to right atrium bypass graft. Operation was done through median sternotomy and extended vertical oblique abdominal incision. A 24 mm Dacron tube was placed from the inferior vena cava just below the left renal vein to the right atrium without using the cardiopulmonary bypass pump. The patient's postoperative course was uneventful without signs of bleeding or any other complications. We used anticoagulants at the postoperative first day. At the postoperative 26th day, we performed abdominal Doppler sonography and we confirmed that the graft patency was good. The patient was discharged with SVC obstructive symptoms but we noticed relief of SVC obstructive symptoms in the course of follow-up.

항트롬빈, C 단백, S 단백 결핍에 의한 Budd-chiari syndrome과 상대정맥 폐색

본 증례의 39세 남자는 전신부종과 간부전증을 일으키는 Budd-Chiari syndrome과 상대정맥 폐색증을 가지고 입원하였다. 보존적 내과 치료 후 방사선과의 침습적 방법에 의한 확장술에 실패하여 하공정맥-우심방 단락술을 시행하였다. 수술은 정중흉골 절제술 및 직사행 복부 절제술을 통하여 직경 24 mm의 Dacron graft를 사용하여 좌신정맥 하에서 하공정맥-우심방 단락술을 체외순환을 하지 않고 시행하였다 수술후 출혈이나 합병증없이 양호한 결과를 보였으며 수술당일부터 항응고제 치료를 병행하였다. 술후 26일째 시행한 복부 도플러 초음파 상에서 graft의 유통성이 양호함을 확인하였다. 퇴원시 상대정맥 폐색 증상은 남아 있었으나 외래 추적 검사시 상대정맥 폐색 증상도 호전되었음을 확인할 수 있었다