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揭示一例罕见的布加综合征病例。

Revealing an Exceptional Case of Budd-Chiari Syndrome.

作者信息

Narne Krishna Geetha, K I S N Vaishnavi, Kakumani Jagadeswar, T Vivekanandan, A Gowri Shankar

机构信息

Internal Medicine, Saveetha Medical College and Hospital, Saveetha Institute of Medical and Technical Sciences (SIMATS) Saveetha University, Chennai, IND.

出版信息

Cureus. 2024 Sep 22;16(9):e69949. doi: 10.7759/cureus.69949. eCollection 2024 Sep.

Abstract

Budd-Chiari syndrome (BCS) is a rare disorder characterized by hepatic venous outflow obstruction, leading to substantial effects, which include liver congestion, ascites, and liver failure. This is an unusual but very important form of secondary BCS caused by extraluminal compression from structures such as abscesses, tumors, or cysts. This case study exemplifies a 46-year-old female with no previous medical history who developed BCS due to the encasement of the IVC and hepatic veins secondary to a hydatid cyst, which is an uncommon presentation. Distension, ascites, and pain in the abdomen are the chief complaints. Investigations were positive for a hydatid cyst in the liver on imaging and serology, as well as features commensurate with BCS. The patient received conservative management with albendazole and anticoagulation. However, she refused cyst surgical resection and thus died three weeks after her discharge because of a cardiorespiratory arrest possibly associated with complications of her hydatid cyst like anaphylaxis secondary to rupture of the hydatid cyst. Therefore, this case brings out the significance of considering hydatid cysts when making a differential diagnosis on patients suffering from BCS, especially in endemic areas, and also underscores the importance of early recognition, treatment measures, and education to avoid fatal conditions.

摘要

布加综合征(BCS)是一种罕见的疾病,其特征为肝静脉流出道梗阻,可导致严重后果,包括肝脏充血、腹水和肝衰竭。这是由脓肿、肿瘤或囊肿等结构的管腔外压迫引起的一种不寻常但非常重要的继发性布加综合征形式。本病例研究例证了一名46岁既往无病史的女性,因肝包虫囊肿压迫下腔静脉和肝静脉而患上布加综合征,这是一种不常见的表现。腹胀、腹水和腹痛是主要症状。影像学和血清学检查显示肝脏存在包虫囊肿,且有与布加综合征相符的特征。患者接受了阿苯达唑和抗凝的保守治疗。然而,她拒绝了囊肿手术切除,出院三周后因心肺骤停死亡,可能与包虫囊肿并发症如包虫囊肿破裂继发过敏反应有关。因此,该病例凸显了在对布加综合征患者进行鉴别诊断时,尤其是在流行地区,考虑包虫囊肿的重要性,同时也强调了早期识别、治疗措施和教育以避免致命情况的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/45d1/11496205/d612703e3b05/cureus-0016-00000069949-i01.jpg

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