Obinata Hirofumi, Nishibe Shinichi, Ishihara Yoko
Department of Anesthesiology, Saitama International Medical Center, 1397-1 Yamane, Hidaka, Saitama, Japan.
Department of Anesthesiology, Self-Defense Forces Central Hospital, 1-2-24 Ikejiri, Setagaya, Tokyo, Japan.
JA Clin Rep. 2018;4(1):16. doi: 10.1186/s40981-018-0154-5. Epub 2018 Feb 12.
Heterotaxy syndrome (HS) is characterized by a wide variety of cardiac and extra-cardiac malformations, including pulmonary valve stenosis, interruption of the inferior vena cava, total anomalous pulmonary venous connection (TAPVC), asplenia, polysplenia, intestinal malrotation, and preduodenal portal vein (PDPV). We report the case of a heterotaxic infant with an infracardiac TAPVC and preduodenal portal vein who experienced repetitive hemodynamic instability during urgent laparotomy for duodenal obstruction.
A 3-day-old boy with HS was planned to undergo urgent laparotomy for duodenal atresia. Echocardiogram showed an interrupted inferior vena cava, single right ventricle, pulmonary valve stenosis, and infracardiac TAPVC. On exploratory laparotomy, intestinal malrotation characterized by Ladd's band was found. During further exploration, repetitive severe hypotension and hypoxia occurred. Thorough examination revealed a greatly dilated PDPV crossing over and compressing the proximal duodenum externally. Finally, we considered the possibility that surgical manipulation directly compressed the dilated PDPV into which the TAPVC had pulmonary venous drainage, leading to repetitive pulmonary venous obstruction (PVO). Computed tomography, which was examined after laparotomy, indicated that the vertical vein from pulmonary venous confluence drained into the portal vein.
PDPV is a rare anomaly associated with HS. In case of intestinal malrotation and duodenal obstruction in HS with infracardiac TAPVC, both the presence of PDPV and the possibility of pulmonary venous drainage into the PDPV should be considered by pediatric surgeons and anesthesiologists performing laparotomy to avoid catastrophic PVO.
异构综合征（HS）的特征是存在多种心脏和心脏外畸形，包括肺动脉瓣狭窄、下腔静脉中断、完全性肺静脉异位连接（TAPVC）、无脾、多脾、肠旋转不良和十二指肠前门静脉（PDPV）。我们报告了一例患有心内型TAPVC和十二指肠前门静脉的异构婴儿病例，该婴儿在因十二指肠梗阻进行紧急剖腹手术期间反复出现血流动力学不稳定。
一名患有HS的3日龄男婴计划因十二指肠闭锁接受紧急剖腹手术。超声心动图显示下腔静脉中断、单心室、肺动脉瓣狭窄和心内型TAPVC。在剖腹探查术中，发现以Ladd束为特征的肠旋转不良。在进一步探查过程中，反复出现严重低血压和缺氧。全面检查发现一条极度扩张的PDPV从上方穿过并在外部压迫十二指肠近端。最后，我们考虑手术操作直接将扩张的PDPV压迫到TAPVC有肺静脉引流的部位，导致反复性肺静脉梗阻（PVO）。剖腹手术后进行的计算机断层扫描显示，肺静脉汇合处的垂直静脉引流至门静脉。
PDPV是一种与HS相关的罕见异常。对于患有心内型TAPVC的HS患者出现肠旋转不良和十二指肠梗阻的情况，进行剖腹手术的小儿外科医生和麻醉医生应考虑PDPV的存在以及肺静脉引流至PDPV的可能性，以避免灾难性的PVO。
