Dobek Adam, Stefańczyk-Jakubowicz Katarzyna, Maciąg Rafał, Przewratil Przemysław, Szubert Wojciech, Stefańczyk Ludomir
I Department of Radiology and Diagnostic Imaging, Norbert Barlicki Memorial Teaching Hospital No. 1, Medical University of Łódź, Łódź, Poland.
II Department of Radiology and Diagnostic Imaging, Central Teaching Hospital of the Medical University of Łódź, Łódź, Poland.
Am J Case Rep. 2024 Dec 17;25:e946013. doi: 10.12659/AJCR.946013.
BACKGROUND Arterioportal fistulas (APFs) are abnormal connections between the arterial and portal venous systems, leading to portal hypertension (PH) and symptoms such as gastrointestinal bleeding, splenomegaly, and hepatic pain. Symptoms typically appear by the age of 2 years in about 75% of cases. CASE REPORT A 7-year-old boy with an asymptomatic APF developed life-threatening complications following a Clostridium difficile infection. He initially had chronic diarrhea, abdominal pain, weight loss, and anorexia for 3 weeks, despite normal liver enzymes. After antibiotic and antifungal treatment, his condition worsened, resulting in severe anemia and hemorrhagic shock due to variceal bleeding. Further evaluations revealed significant PH secondary to the APF. Intensive care involved blood transfusions, fluid resuscitation, and high-frequency ventilation. Emergency embolization successfully reduced PH and controlled bleeding. After stabilization, the patient was transferred for further care. A week later, a color Doppler ultrasound (CD-US) detected a thrombus in the left portal vein, which decreased by the 19-day follow-up. Spleen size reduction indicated decreased portal pressure. The presence and reduction of the thrombus and alleviation of PH symptoms indicate therapeutic success. Ten months after embolization, the patient remained asymptomatic, with normal liver function and no thrombus on follow-up imaging. CONCLUSIONS Early diagnosis and intervention are crucial in managing congenital APF in children. Severe variceal bleeding triggered by exacerbated PH due to a Clostridium difficile infection demonstrates the complications of APF. Endovascular treatment was highly effective, resulting in significant improvement. The recommended diagnostic approach includes initial computed tomography angiogram and CD-US, followed by digital subtraction angiography with possible intervention, and monitoring with CD-US.
动门脉瘘（APF）是动脉和门静脉系统之间的异常连接，可导致门静脉高压（PH）以及诸如胃肠道出血、脾肿大和肝区疼痛等症状。约75%的病例中，症状通常在2岁前出现。病例报告：一名7岁无症状APF男孩在艰难梭菌感染后出现危及生命的并发症。尽管肝酶正常，但他最初有3周的慢性腹泻、腹痛、体重减轻和食欲不振。抗生素和抗真菌治疗后，他的病情恶化，因静脉曲张破裂出血导致严重贫血和失血性休克。进一步评估发现APF继发严重PH。重症监护包括输血、液体复苏和高频通气。紧急栓塞成功降低了PH并控制了出血。病情稳定后，患者转至其他科室进一步治疗。一周后，彩色多普勒超声（CD-US）检测到左门静脉血栓形成，在19天的随访中血栓减少。脾脏大小缩小表明门静脉压力降低。血栓的出现与减少以及PH症状的缓解表明治疗成功。栓塞术后10个月，患者仍无症状，肝功能正常，随访影像检查未发现血栓。结论：早期诊断和干预对儿童先天性APF的管理至关重要。艰难梭菌感染导致PH加重引发的严重静脉曲张破裂出血证明了APF的并发症。血管内治疗非常有效，带来显著改善。推荐的诊断方法包括最初的计算机断层血管造影和CD-US，随后进行可能需要干预的数字减影血管造影，并通过CD-US进行监测。
