Fideler Frank, Mustafi Migdad, Kirschner Hans-Joachim, Gerbig Ines, Fuchs Jörg, Hofbeck Michael, Kumpf Matthias, Kagan Oliver, Michel Jörg, Jost Walter, Neunhoeffer Felix
Department of Anesthesiology and Intensive Care Medicine, University Hospital, Tübingen, Germany.
Department of Thoracic, Heart and Vascular Surgery, University Hospital, Tübingen, Germany.
European J Pediatr Surg Rep. 2023 Apr 10;11(1):e15-e19. doi: 10.1055/s-0043-1767734. eCollection 2023 Jan.
Both congenital diaphragmatic hernias (CDHs) and omphaloceles show relevant overall mortality rates as individual findings. The combination of the two has been described only sparsely in the literature and almost always with a fatal course. Here, we describe a term neonate with a rare high-risk constellation of left-sided CDH and a large omphalocele who was successfully treated on extracorporeal life support (ECLS). Prenatally, the patient was diagnosed with a large omphalocele and a left CDH with a lung volume of ∼27% and an observed to expected lung-to-head ratio of 30%. Due to respiratory insufficiency, an ECLS device was implanted. As weaning from ECLS was not foreseeable, the female infant underwent successful surgery on ECLS on the ninth day of life. Perioperative high-frequency oscillatory ventilation and circulatory and coagulation management under point-of-care monitoring were the main anesthesiological challenges. Over the following 3 days, ECLS weaning was successful, and the patient was extubated after another 43 days. Surgical treatment on ECLS can expand the spectrum of therapy in high-risk constellations if potential risks are minimized and there is close interdisciplinary cooperation.
先天性膈疝（CDH）和脐膨出作为个体病例均显示出较高的总体死亡率。两者合并出现的情况在文献中仅有少量描述，且几乎总是致命的病程。在此，我们描述一名足月儿，患有罕见的高危组合，即左侧CDH和巨大脐膨出，经体外生命支持（ECLS）成功治疗。产前，该患者被诊断为巨大脐膨出和左侧CDH，肺容积约为27%，观察到的肺头比与预期肺头比为30%。由于呼吸功能不全，植入了ECLS设备。由于无法预见能否脱离ECLS，该女婴在出生后第9天在ECLS支持下成功接受了手术。围手术期高频振荡通气以及在床旁监测下的循环和凝血管理是主要的麻醉挑战。在接下来的3天里，成功撤离ECLS，再过43天后患者拔管。如果将潜在风险降至最低并进行密切的多学科合作，在ECLS支持下进行手术治疗可以扩大高危组合病例的治疗范围。
