Nguyen Thai Van, Nguyen Thinh Tien, Dong Hoang Duc, Duong Huy Quang
Department of Hepato-Gastroenterology, 108 Military Central Hospital, Hanoi, Vietnam.
Department of Internal Medicine, Thai Nguyen University of Medicine and Pharmacy, Thainguyen, Vietnam.
Case Rep Gastroenterol. 2021 Sep 30;15(3):877-884. doi: 10.1159/000518925. eCollection 2021 Sep-Dec.
Budd-Chiari syndrome (BCS) is a rare disorder caused by hepatic venous outflow obstruction that can lead to acute liver failure proposing liver transplantation or transjugular intrahepatic portosystemic shunt. However, the transjugular intrahepatic portosystemic shunt is not always successful due to the entire hepatic vein thrombosis while transplantation is not unfailingly feasible. In these situations, the direct intrahepatic portosystemic shunt (DIPS) is a viable alternative that may ameliorate portal hypertension in these patients. We described a case of a 21-year-old male with fulminant hepatic failure owning to BCS with a 4-day history of abdominal pain and nausea. Laboratory workup, including viral, autoimmune etiologies JAK2 mutation, Factor V Leiden, antiphospholipid antibody syndrome, was negative. The patient's clinical status worsened with hepatic encephalopathy stage II despite administering anticoagulation. Thus, the patient underwent urgently DIPS after unaccessible to the creation of a transjugular intrahepatic portosystemic shunt and impossible to transplantation. The patient's health was improved and discharged. Fulminant Budd-Chiari is a rare disease to be demanding prompt treatment. While transplantation or transjugular intrahepatic portosystemic shunt is failed, the DIPS is considered an alternative candidate associated with clinical improvement.
布加综合征(BCS)是一种由肝静脉流出道梗阻引起的罕见疾病,可导致急性肝衰竭,从而需要进行肝移植或经颈静脉肝内门体分流术。然而,由于整个肝静脉血栓形成,经颈静脉肝内门体分流术并不总是成功的,而移植也并非总是可行。在这些情况下,直接肝内门体分流术(DIPS)是一种可行的替代方法,可能会改善这些患者的门静脉高压。我们描述了一例21岁男性因布加综合征导致暴发性肝衰竭的病例,该患者有4天的腹痛和恶心病史。实验室检查,包括病毒、自身免疫病因、JAK2突变、凝血因子V Leiden、抗磷脂抗体综合征,均为阴性。尽管进行了抗凝治疗,患者的临床状况仍因II期肝性脑病而恶化。因此,在无法进行经颈静脉肝内门体分流术且无法进行移植的情况下,患者紧急接受了DIPS治疗。患者的健康状况得到改善并出院。暴发性布加综合征是一种罕见疾病,需要及时治疗。当移植或经颈静脉肝内门体分流术失败时,DIPS被认为是一种与临床改善相关的替代选择。