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vol. 16
Letter to the Editor

Polysplenia syndrome with a rare variation between the common hepatic artery and the superior mesenteric artery in adults

Jingjing Liu
Kai Yang
Jianbo Wang

Department of Interventional Radiology, The Sixth People’s Hospital Affiliated to Shanghai Jiaotong University, Xuhui District, Shanghai, China
Arch Med Sci 2020; 16 (5): 1263–1266
Online publish date: 2020/08/10
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Polysplenia syndrome (PSS), a heterotaxy syndrome, is a rare congenital anomaly with a reported incidence of 1 in 250,000 live births and was first described by Helwig [1] in 1929. Most patients with PSS die in the early neonatal period because the disease is often accompanied by serve cardiac and biliary abnormalities [2]. However, some patients have only mild cardiovascular malformations or anomalies in the abdominal organs, which are typically diagnosed incidentally in adulthood [3]. Anomalies in the cardiovascular tract including bilateral superior vena cava, interruption of the inferior vena cava with azygos continuation, ventricular septal defect, ostium primum defect, and morphologic left ventricular outflow obstruction were present in at least 50% of 146 autopsied cases of polysplenia reviewed [4]. Preduodenal portal vein is another anomaly frequently associated with polysplenia syndrome and Rameshbabu et al. described this anomaly in 7 out of 19 cases of his own autopsied series [5]. PSS is a rare disease, and there is gradually increasing literature reporting the accompanying various abnormalities in order to make people realize what these accompanying abnormalities are. However, PSS with a variation between the common hepatic artery (CHA) and the superior mesenteric artery (SMA) in adults is rarely reported in the literature. The CHA is a short blood vessel arising from the celiac trunk (CT) that supplies the liver, pylorus, pancreas, and duodenum [6]. The SMA arises from the anterior surface of the abdominal aorta, approximately 2 cm below the CT, and supplies the pancreas, duodenum, jejunum, ileum, cecum, appendix, and ascending colon, as well as part of the transverse colon [7].
We herein report on a patient who was diagnosed with PSS and was later found to have a vascular anatomical variation between the CHA and the SMA. It makes people realize that PSS may be accompanied by a new vascular variability and highlights the importance for anatomists, surgeons, and radiologists of the awareness of this easily missed and rare vascular anomaly in PSS due to its prominent significance in many clinical procedures, such as radiological abdominal intervention and abdominal surgery.
A 39-year-old Chinese woman with no prior medical history presented with intermittent abdominal pain for 6–7 days. She had no previous history of splenectomy, and physical examination revealed no abnormality. She underwent plain and contrast-enhanced upper...

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