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多脾综合征合并左位下腔静脉的内脏反位的解剖学、胚胎学及影像学表现

Anatomy, embryology, and imaging of situs ambiguous with polysplenia and left IVC.

作者信息

Montero-Macías Rosa, Krief David, Villefranque Vincent, Kilani Mohamed, Castillo-Tuñon Juan Manuel

机构信息

Obstetrics and Gynecology Department, Centre Hospitalier Simone-Veil, 14 Rue de Saint-Prix, 95600 Eaubonne, France.

Radiology Department, Centre Hospitalier Simone-Veil, Eaubonne, France.

出版信息

Radiol Case Rep. 2023 Feb 27;18(5):1727-1732. doi: 10.1016/j.radcr.2023.02.005. eCollection 2023 May.

DOI:10.1016/j.radcr.2023.02.005
PMID:36895889
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9989316/
Abstract

The heterotaxy syndrome is a type of syndrome that involves multiple visceral abnormalities, vascular ones and associated with left isomerism. Malformation of gastroenterologic system includes polysplenia (segmented spleen or multiple splenules), agenesis (partial or complete) of the dorsal pancreas and anomalous of the inferior vena cava implantation. Here, we describe and show the anatomy of a patient with left side inferior vena cava, situs ambiguous (complete common mesentery), polysplenia, and short pancreas. We also discuss about the embryologic process and the implications of these anomalies during gynecologic, digestive, and liver surgeries.

摘要

内脏反位综合征是一种涉及多种内脏异常（包括血管异常）且与左位异构相关的综合征。胃肠系统畸形包括多脾症（分叶脾或多个脾小结）、背侧胰腺发育不全（部分或完全）以及下腔静脉植入异常。在此，我们描述并展示了一名左侧下腔静脉、内脏位置不明确（完全共同肠系膜）、多脾症和短胰腺患者的解剖结构。我们还讨论了胚胎学过程以及这些异常在妇科、消化和肝脏手术中的影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7a5/9989316/ad351ff90368/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7a5/9989316/3e32eec351a7/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7a5/9989316/5e0933112971/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7a5/9989316/3e726ae3e2ba/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7a5/9989316/ad351ff90368/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7a5/9989316/3e32eec351a7/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7a5/9989316/5e0933112971/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7a5/9989316/3e726ae3e2ba/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7a5/9989316/ad351ff90368/gr4.jpg

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Niger J Surg. 2020 Jan-Jun;26(1):72-77. doi: 10.4103/njs.NJS_47_183. Epub 2020 Feb 10.
3
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成人偶然发现的伴有内脏位置不明确、共同肠系膜和下腔静脉中断的多脾综合征。
Radiol Case Rep. 2019 Jun 29;14(9):1072-1075. doi: 10.1016/j.radcr.2019.05.032. eCollection 2019 Sep.
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Abnormalities of Fetal Situs: An Overview and Literature Review.胎儿位置异常：概述及文献回顾。
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Ann Pediatr Cardiol. 2014 Sep;7(3):187-92. doi: 10.4103/0974-2069.140834.
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