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肝外门静脉高压症患儿门静脉系统的血管造影模式及其病因学和临床相关性。

Angiographic patterns of portal venous system in children with extrahepatic portal hypertension and its etiological and clinical relevance.

作者信息

Yuldashev R Z, Aliev M M, Maksudov M F, Khaydarov U O, Shokhaydarov Sh

机构信息

Department of Pediatric Surgery, Tashkent Pediatric Medical Institute, Tashkent, Uzbekistan.

Republican Specialized Scientific-Practical Medical Center of Pediatrics, Tashkent, Uzbekistan.

出版信息

Pediatr Surg Int. 2023 Feb 1;39(1):97. doi: 10.1007/s00383-023-05384-3.

DOI:10.1007/s00383-023-05384-3
PMID:36723662
Abstract

PURPOSE

To study the relationship between the angiographic pattern of extrahepatic portal vein obstruction (EHPVO) and its etiology and clinical manifestations.

METHODS

Clinical, etiological, and angiographic findings in 155 children with EHPVO were reviewed. Anatomy of extrahepatic portal venous system (EPVS) was categorized into five imaging patterns. Assessment of the severity of esophageal and gastric varices (EV and GV) was performed by upper gastrointestinal endoscopy.

RESULTS

Based on multislice CT angiography, most commonly observed pattern of EHPVO was type I (48.4%) and type II (29%). According to anamnesis, 68 (43.8%) children had pathological conditions in neonatal period. Of these, 35 (22.6%) had an umbilical vein catheterization, 11 (7.1%) had a history of omphalitis, and 9 (5.8%) had prolonged jaundice. Thirteen (8.4%) patients had various septic conditions in neonatal period and it was more common associated with widespread thrombosis throughout the EPVS (type 5)-28% of observations. Significantly lower risk of bleeding from EV (p = 0.01) was noted in children with type IV pattern, whereas children with type III and V patterns had higher grades of EV.

CONCLUSION

Angiographic pattern of portomesenteric occlusion may provide a clue to its etiology, and clinical manifestation, especially in children with widespread thrombosis throughout the EPVS.

摘要

目的

研究肝外门静脉阻塞(EHPVO)的血管造影表现与其病因及临床表现之间的关系。

方法

回顾了155例EHPVO患儿的临床、病因及血管造影结果。肝外门静脉系统(EPVS)的解剖结构分为五种影像模式。通过上消化道内镜检查评估食管和胃静脉曲张(EV和GV)的严重程度。

结果

基于多层CT血管造影,EHPVO最常见的模式为I型(48.4%)和II型(29%)。根据病史,68例(43.8%)患儿在新生儿期有病理情况。其中,35例(22.6%)曾行脐静脉置管,11例(7.1%)有脐炎病史,9例(5.8%)有黄疸持续时间延长。13例(8.4%)患者在新生儿期有各种败血症情况,且更常见于整个EPVS广泛血栓形成(5型)——占观察病例的28%。IV型患儿发生EV出血的风险显著较低(p = 0.01),而III型和V型患儿的EV分级较高。

结论

门静脉肠系膜阻塞的血管造影表现可能为其病因及临床表现提供线索,尤其是在整个EPVS广泛血栓形成的患儿中。

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Extrahepatic Portal Vein Thrombosis, an Important Cause of Portal Hypertension in Children.
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