Eitler Katalin, Bibok András, Telkes Gábor
Department of Surgery, Transplantation and Gastroenterology, Semmelweis University, Budapest, Hungary.
Medical Imaging Centre, Semmelweis University, Budapest, Hungary.
Int J Gen Med. 2022 Mar 3;15:2437-2449. doi: 10.2147/IJGM.S295444. eCollection 2022.
Situs inversus totalis is a rare congenital abnormality characterized by a mirror-image transposition of both the abdominal and the thoracic organs. While this anomaly is known since the ancient times, practicing doctors do not have much experience with it. Laterality is established early in development, and any failure in that process might lead to a wide variety of disorders which may be partial or complete. describes the normal anatomy, is the complete reversal, and is used for any other abnormality of left-right development. Sidedness is regulated by genes: over 100 genes have been linked to laterality defects. Frequency of situs inversus is 1:10,000 and is more frequent in males: 1.5:1. Advanced imaging modalities can be used to assess fine anatomical details, which play a crucial role in these cases to plan radiologic or surgical interventions. Percutaneous biliary procedures, portal vein embolization are really challenging procedures in SIT patients due to the mirror effect. As most surgeons are right-handed, SIT operations can cause difficulties: handling the instruments with their left hand or the pedals with their left foot can be uncomfortable Organ, especially liver transplantation represents an extraordinary surgical challenge. Solutions to overcome the anatomic differences include the use of segment or reduced size graft with rotation, modified piggy-back technique, side to-side caval anastomosis, and vascular conduit. Because of its rarity and special nature, surgical patients with situs inversus may require more flexibility and creativity from the surgical team.
完全性内脏反位是一种罕见的先天性异常,其特征是腹部和胸部器官呈镜像转位。虽然这种异常自古以来就为人所知,但执业医生对其经验不多。身体左右侧性在发育早期就已确立,该过程中的任何失败都可能导致各种部分或完全性的疾病。[此处原文缺失相关内容]描述正常解剖结构,[此处原文缺失相关内容]是完全反转,[此处原文缺失相关内容]用于描述左右发育的任何其他异常。身体左右侧性由基因调控:超过100种基因与左右侧性缺陷有关。内脏反位的发生率为1:10000,男性更为常见,男女比例为1.5:1。先进的成像方式可用于评估精细的解剖细节,这在这些病例中对于规划放射学或手术干预起着关键作用。由于镜像效应,经皮胆道手术、门静脉栓塞术在完全性内脏反位患者中是极具挑战性的手术。由于大多数外科医生是右利手,完全性内脏反位手术可能会造成困难:用左手操作器械或用左脚操作踏板可能会不舒服。器官移植,尤其是肝移植是一项特殊的手术挑战。克服解剖差异的解决方案包括使用带旋转的节段性或缩小尺寸的移植物、改良背驮式技术、腔静脉侧侧吻合术和血管导管。由于其罕见性和特殊性,完全性内脏反位的外科手术患者可能需要手术团队具备更大的灵活性和创造力。