Kirby Eimear, Keijzer Richard
Trinity College Dublin School of Medicine, Trinity Biomedical Sciences Institute, Dublin, Ireland.
Thorlakson Chair in Surgical Research, Division of Pediatric Surgery, Department of Surgery and Children's Hospital Research Institute of Manitoba, University of Manitoba, AE402-820 Sherbrook Street, Winnipeg, MB, R3A 1S1, Canada.
Pediatr Surg Int. 2020 Apr;36(4):415-429. doi: 10.1007/s00383-020-04625-z. Epub 2020 Feb 18.
Congenital diaphragmatic hernia (CDH) is a developmental birth defect consisting of a diaphragmatic defect and abnormal lung development. CDH complicates 2.3-2.8 per 10,000 live births. Despite efforts to standardize clinical practice, management of CDH remains challenging. Frequent re-evaluation of clinical practices in CDH reveals that management of CDH is evolving from one of postnatal stabilization to prenatal optimization. Translational research reveals promising avenues for in utero therapeutic intervention, including fetoscopic endoluminal tracheal occlusion. These remain highly experimental and demand improved antenatal diagnostics. Timely diagnosis of CDH and identification of severely affected fetuses allow time for delivery planning or in utero therapeutics. Optimal perinatal care and surgical treatment strategies are highly debated. Improved CDH mortality rates have placed increased emphasis on identifying and monitoring the long-term sequelae of disease throughout childhood and into adulthood. We review the current management strategies for CDH, highlighting where progress has been made, and where future developments have the potential to revolutionize care in this vulnerable patient population.
先天性膈疝(CDH)是一种发育性出生缺陷,包括膈肌缺损和肺部发育异常。CDH在每10000例活产中的发生率为2.3 - 2.8例。尽管人们努力规范临床实践,但CDH的管理仍然具有挑战性。对CDH临床实践的频繁重新评估表明,CDH的管理正从产后稳定治疗向产前优化治疗转变。转化研究揭示了子宫内治疗干预的有前景的途径,包括胎儿镜下腔内气管阻塞。这些仍处于高度实验阶段,需要改进产前诊断。CDH的及时诊断和严重受影响胎儿的识别为分娩计划或子宫内治疗留出了时间。最佳围产期护理和手术治疗策略备受争议。CDH死亡率的改善使得人们更加重视识别和监测整个儿童期及成年期疾病的长期后遗症。我们回顾了CDH目前的管理策略,突出了已取得进展的方面,以及未来发展有可能彻底改变这一脆弱患者群体护理方式的方面。