Kwon Se Hwan, Shin So Youn
Department of Radiology, Kyung Hee University Hospital, College of Medicine, Kyung Hee University, Seoul, Republic of Korea.
J Thorac Dis. 2018 Feb;10(2):E138-E141. doi: 10.21037/jtd.2018.01.128.
Polysplenia syndrome is a rare condition (about 2.5:100,000 live births) wherein a person has two or more spleens with accompanying various thoracic and abdominal organ abnormalities. Polysplenia is usually associated with situs ambiguous, while polysplenia with situs inversus is very rare. Herein, we report a rare case of incidentally found polysplenia syndrome combined with situs inversus in a 59-year-old female who also had an interrupted inferior vena cava (IVC) with azygos continuation, patent ductus arteriosus (PDA), and variations in the aortic arch branches. An aberrant left subclavian artery with Kommerell's diverticulum and a large aneurysm in the non-aberrant, proximal-right subclavian artery were also noted.
多脾综合征是一种罕见病症（约每10万例活产中有2.5例），即一个人有两个或更多脾脏，并伴有各种胸腹部器官异常。多脾通常与内脏转位不明确相关，而伴有内脏反位的多脾非常罕见。在此，我们报告一例罕见病例，一名59岁女性偶然发现多脾综合征合并内脏反位，同时还存在下腔静脉中断并奇静脉延续、动脉导管未闭以及主动脉弓分支变异。还发现一条异常的左锁骨下动脉伴Kommerell憩室，以及在正常位置的右锁骨下动脉近端有一个大动脉瘤。
