Zhang Yu-Yan, Ruan Jiang, Fu Yan
Department of Gastroenterology, The Second Affiliated Hospital of Kunming Medical University, Kunming 650101, Yunnan Province, China.
World J Gastrointest Surg. 2024 Nov 27;16(11):3578-3583. doi: 10.4240/wjgs.v16.i11.3578.
Asplenia-type heterotaxy syndrome (HS) is rare and refers to visceral malposition and dysmorphism. It is associated with a high infant mortality rate due to cardiac anomalies, and related digestive endoscopic interventions are poorly understood. With the improved long-term prognosis of these individuals after modern cardiac surgery, intra-abdominal anomalies have become increasingly significant.
Herein, we report successful endoscopic retrograde cholangiopancreatography (ERCP) in a 14-year-old male with asplenia-type HS that involved unique imaging findings and technical difficulties. His anatomic anomalies included complex congenital heart disease, midline liver placement, an absent spleen, a left-sided inferior vena cava, and dextroposition of the stomach and pancreas. He suffered from choledocholithiasis with obstructive jaundice, and the stone was successfully extracted with a basket following endoscopic papillary balloon dilation.
Although anatomic anomalies in HS increase the degree of technical difficulty when performing ERCP, they can be safely managed by experienced endoscopists, as illustrated by the present case. Identifying these variations with imaging modalities and being aware of them before initiating an invasive intervention are crucial to preventing potential complications.
无脾型内脏反位综合征（HS）较为罕见，是指内脏位置异常和形态异常。由于心脏异常，其婴儿死亡率较高，且相关的消化内镜干预措施尚不清楚。随着现代心脏手术后这些个体长期预后的改善，腹内异常变得越来越重要。
在此，我们报告了一例14岁无脾型HS男性患者成功进行内镜逆行胰胆管造影（ERCP）的病例，该病例涉及独特的影像学表现和技术难题。他的解剖学异常包括复杂先天性心脏病、肝脏位于中线、无脾、左侧下腔静脉以及胃和胰腺右旋。他患有胆总管结石伴梗阻性黄疸，在内镜乳头球囊扩张后用网篮成功取出结石。
尽管HS中的解剖学异常增加了ERCP操作的技术难度，但如本病例所示，经验丰富的内镜医师可以安全地处理这些情况。在进行侵入性干预之前，通过影像学检查识别这些变异并了解它们对于预防潜在并发症至关重要。
