成人多脾综合征的腹部CT表现

Abdominal CT imaging findings of polysplenia syndrome in adulthood:report of four cases and review of the literature

目的分析成人多脾综合征腹部CT影像特点,提高对多脾综合征的认识。方法回顾性分析4例多脾综合征的腹部CT表现。重点观察脾脏、胃、肝、胆囊、胰腺、肠管以及血管的位置和发育异常。同时回顾文献报道的15例成人多脾综合征的CT表现。结果本组4例患者均为CT检查偶然发现。男1例,女3例,脾脏数目2～12个,均沿胃大弯侧排列,其中1例位于右上腹;4例均存在短胰及肠旋转不良;3例肝段下腔静脉缺如,奇静脉异常连接并扩张;1例肝脏呈中间位;1例胆囊呈中间位并结石,1例胆囊左位。结论熟悉多脾综合征各种异常的CT表现,有助于准确诊断,减少漏误诊。

Objective To analyze the computed tomographic（CT） features of the abdominal anomalies consistent with polysplenia syndrome in adults, and increase awareness for Polysplenia Syndrome. Methods The abdominal CT imaging materials of four cases with Polysplenia Sydrome were analysed retrospectively. Attention was directed to the location and dysplasia of the multiple spleens, stomach, liver, pancreas, intestine and venous. We also reviewed the CT findings of 15 adult patients described in the literature. Results 4 patients comprised 3 females and 1 male were incidentally discovered on CT examination. The number of spleens was from 2 to 12 ,all were along the greater curvature of the stomach,which was located in the right upper quadrant in one patient. A short pancreas was seen in four, intestinal malrotation in four and absent hepatic inferior vena cava with dilated and abnormal connection azygos vein in three. The liver was in the midline in one, the gallbladder was in the midline in one, in the left in one. Conclusion Understanding of CT imaging findings of various abdominal abnormalities in polysplenia syndrome can contribute to accurate diagnosis and reduce misdiagnosis.