Unusual association of Axenfeld-Rieger syndrome and wandering spleen:A case report  

作　　者：Yi-Lin Chang Joseph Lin Yu-Hsien Li Lien-Cheng Tsao 

机构地区：[1]Department of General Surgery,Changhua Christian Hospital,Changhua 50006,Taiwan

出　　处：《World Journal of Clinical Cases》2020年第8期1502-1506,共5页世界临床病例杂志

摘　　要：BACKGROUND Axenfeld-Rieger syndrome(ARS)is an autosomal dominant genetic disease characterized by ocular developmental disorders and its association with torsion of wandering spleen(WS)has not been reported to date to the best of our knowledge.This study aimed to describe a rare case of ARS observed at our emergency department.CASE SUMMARY A 25-year-old female presented with a constant lower abdominal pain of increasing severity.Diagnostic computed tomography with intravenous contrast material showed a non-homogenously enhanced splenic parenchyma with a twisted vascular pedicle.Further,an emergent laparoscopic exploration was performed,and an ischemic spleen without its normal ligamentous attachments was noted.Notably,the spleen did not regain its normal vascularity after detorsion;thus,we performed the laparoscopic total splenectomy.The postoperative course was uneventful,and the patient was discharged on the 5th postoperative day.This case demonstrates a rare association of WS and ARS.CONCLUSION Early diagnosis of WS in the emergency department is important to prevent pedicle torsion or splenic necrosis and to avoid splenectomy.

关 键 词：WANDERING SPLEEN Axenfeld-Rieger SYNDROME Laparoscopic SPLENECTOMY Autosomal dominant ABDOMINAL pain Emergent surgery Case report 

分 类 号：R596.1[医药卫生—内科学] R551.1[医药卫生—临床医学]