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多脾并伴有背侧胰腺和十二指肠前门静脉缺如,约1例。

Polysplenia with agenesis of the dorsal pancreas and preduodenal portal vein, about a case.

作者信息

Elhattabi Khalid, Elyamine Othmane, Bouali Mounir, El Bakouri Abdelilah, Bensardi Fatimazahra, Fadil Abdelaziz

机构信息

Department of Visceral Surgical Emergencies, University Hospital Center Ibn Rochd, Faculty of Medicine and Pharmacy, Hassan II University, Casablanca, Morocco.

Department of General Surgery, University Hospital Center Ibn Rochd, Faculty of Medicine and Pharmacy, Hassan II University, Casablanca, Morocco.

出版信息

Int J Surg Case Rep. 2020;76:357-360. doi: 10.1016/j.ijscr.2020.10.005. Epub 2020 Oct 7.

Abstract

PURPOSE OF THE STUDY

Polysplenia is a complex polymalformative syndrome that is frequently accompanied by an inconsistent spectrum of visceral and vascular anatomical abnormalities and is extremely undiscovered in adulthood. The objective of this article is to limit the intraoperative risks generated by the lack of knowledge of these anatomical variations by insisting on the inconstancy of all these variations and the perfect knowledge of the reference anatomy and the exploration of the anatomical variations in imaging before the surgery.

PATIENT AND METHODS

The patient was 50-year-old who was hospitalized in our department for gastric adenocarcinoma. During her extension assessment, a polysplenia syndrome was accidentally discovered on the scan images, which showed an exceptional association between preduodenal portal vein, agenesis of the dorsal pancreas, and polysplenia.

CONCLUSION

There are neither specific clinical symptoms of the polysplenia syndrome, nor any biological sign; hence, the interest of recognizing this pathology is to avoid diagnostic errors, but also to guide the surgeon during the surgical act performed.

摘要

研究目的

多脾综合征是一种复杂的多畸形综合征,常伴有一系列不一致的内脏和血管解剖异常,在成年期极为罕见。本文的目的是通过强调所有这些变异的不稳定性、对参考解剖结构的完美了解以及术前对成像中解剖变异的探索,来降低因对这些解剖变异缺乏了解而产生的术中风险。

患者与方法

患者为一名50岁女性,因胃腺癌入住我科。在其扩展评估期间,扫描图像意外发现多脾综合征,显示十二指肠前门静脉、背侧胰腺缺如和多脾之间存在特殊关联。

结论

多脾综合征既没有特定的临床症状,也没有任何生物学体征;因此,认识这种病理情况的意义在于避免诊断错误,同时在手术过程中为外科医生提供指导。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9cf2/7569255/ff94066e6642/gr1.jpg

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