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一例罕见的儿科病例:一名5岁男孩患布加综合征并伴有上消化道出血。

A rare pediatric case: Budd-Chiari Syndrome and upper gastrointestinal bleeding in a 5-year-old boy.

作者信息

Ibrahim Mohamed Ismail, Egge Ahmed Abdi Aw, Elmi Omar Ali, Abdi Mohamoud Hashi, Abdilahi Mohamed Ali, Ali Abdirahman Omer

机构信息

College of Health Sciences, School of Medicine and Surgery, Amoud University, Borama, Somalia; Mass CTH, Pediatric department, Somalia.

College of Health Sciences, School of Medicine and Surgery, Amoud University, Borama, Somalia; School of Postgraduate Studies and Research, Amoud University, Amoud Valley, Borama 25263, Somalia.

出版信息

Int J Surg Case Rep. 2025 Feb;127:110992. doi: 10.1016/j.ijscr.2025.110992. Epub 2025 Jan 29.

Abstract

INTRODUCTION AND IMPORTANCE

Budd-Chiari Syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction, often presenting with ascites, hepatomegaly, and abdominal pain. The diagnosis and management of BCS, especially in resource-limited settings, pose significant challenges that can lead to poor patient outcomes.

CASE PRESENTATION

This case report describes a previously healthy individual presenting with progressive abdominal distention, hematemesis, and right upper quadrant pain. The patient had a history of trauma and underwent subsequent percutaneous drainage for ascites. Imaging studies revealed non-opacified hepatic veins, splenomegaly, ascites, and a "nutmeg liver" pattern, confirming the diagnosis of BCS. The patient received blood transfusions, diuretics, and nutritional support. A Transjugular Intrahepatic Portosystemic Shunt (TIPS) procedure was planned but ultimately hindered by financial constraints.

CLINICAL DISCUSSION

This case highlights the challenges of managing BCS in resource-limited settings, particularly regarding access to specialized treatments like TIPS. It emphasizes the need for early diagnosis, multidisciplinary care, and the development of cost-effective treatment strategies to improve patient outcomes.

CONCLUSION

The management of Budd-Chiari Syndrome is complex, particularly in settings with limited resources. This case underscores the importance of timely intervention and the need for innovative approaches to healthcare delivery that can accommodate financial barriers while ensuring patient safety and care quality.

摘要

引言与重要性

布加综合征(BCS)是一种罕见疾病,其特征为肝静脉流出道梗阻,常表现为腹水、肝肿大和腹痛。布加综合征的诊断与治疗,尤其是在资源有限的环境中,面临重大挑战,可能导致患者预后不良。

病例介绍

本病例报告描述了一名既往健康的个体,出现进行性腹胀、呕血和右上腹疼痛。该患者有外伤史,随后接受了腹水的经皮引流。影像学检查显示肝静脉不显影、脾肿大、腹水以及“槟榔肝”模式,确诊为布加综合征。患者接受了输血、利尿剂和营养支持。计划进行经颈静脉肝内门体分流术(TIPS),但最终因经济限制而受阻。

临床讨论

本病例突出了在资源有限的环境中管理布加综合征的挑战,特别是在获得像TIPS这样的专科治疗方面。它强调了早期诊断、多学科护理以及制定具有成本效益的治疗策略以改善患者预后的必要性。

结论

布加综合征的管理很复杂,尤其是在资源有限的环境中。本病例强调了及时干预的重要性以及需要创新的医疗服务提供方式,既能适应经济障碍,又能确保患者安全和护理质量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/92fe/11834072/7eacec8501b4/gr1.jpg

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