PDF(Pubmed)
Abstract：
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.
METHODS: 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.
CONCLUSIONS: Early diagnosis of WS in the emergency department is important to prevent pedicle torsion or splenic necrosis and to avoid splenectomy.
METHODS: 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.
CONCLUSIONS: Early diagnosis of WS in the emergency department is important to prevent pedicle torsion or splenic necrosis and to avoid splenectomy.
摘要：
背景：Axenfeld-Rieger综合征（ARS）是一种常染色体显性遗传性疾病，以眼部发育障碍为特征，据我们所知，迄今为止尚未报道其与游荡脾扭转（WS）的相关性。这项研究旨在描述在我们急诊科观察到的罕见ARS病例。
方法：一名25岁女性表现为持续的下腹痛，其严重程度加重。静脉造影剂的诊断性计算机断层扫描显示脾实质非均匀增强，血管蒂扭曲。Further,进行了紧急腹腔镜探查，并注意到缺血的脾脏没有其正常的韧带附件。值得注意的是,扭曲后脾脏没有恢复正常的血管；因此，我们进行了腹腔镜全脾切除术。术后进展顺利，患者在术后第5天出院。这种情况表明WS和ARS的罕见关联。
结论：在急诊科早期诊断WS对预防蒂扭转或脾坏死和避免脾切除是重要的。
方法：一名25岁女性表现为持续的下腹痛，其严重程度加重。静脉造影剂的诊断性计算机断层扫描显示脾实质非均匀增强，血管蒂扭曲。Further,进行了紧急腹腔镜探查，并注意到缺血的脾脏没有其正常的韧带附件。值得注意的是,扭曲后脾脏没有恢复正常的血管；因此，我们进行了腹腔镜全脾切除术。术后进展顺利，患者在术后第5天出院。这种情况表明WS和ARS的罕见关联。
结论：在急诊科早期诊断WS对预防蒂扭转或脾坏死和避免脾切除是重要的。
