Sankaran Deepika, Hirose Shinjiro, Null Donald Morley, Ravula Niroop R, Lakshminrusimha Satyan
Division of Neonatology, Department of Pediatrics, University of California at Davis, Davis, CA 95817, USA.
Division of Pediatric Surgery, Department of Pediatrics, University of California at Davis, Davis, CA 95817, USA.
Children (Basel). 2021 Dec 9;8(12):1163. doi: 10.3390/children8121163.
The diagnosis of congenital diaphragmatic hernia (CDH) is associated with significant morbidity and mortality. Survival of neonates with CDH has improved recently, although the clinical course is complicated by sequelae of hypoplastic pulmonary parenchyma and vasculature, pulmonary hypertension, ventilation/perfusion (V/Q) mismatch, reduced pulmonary function and poor somatic growth. In this case report, we describe an infant with an antenatal diagnosis of CDH with a poor prognosis who underwent initial surgery followed by a tracheostomy but had a worsening clinical course due to a large area of ventilated but poorly perfused lung based on a V/Q nuclear scintigraphy scan. The emphysematous left lung was causing mediastinal shift and compression of the right lung, further compromising gas exchange. The infant had clinical improvement following bronchial blockade of the under-perfused left lung. This paved the way for further management with resection of the under-perfused lung lobe and continued clinical improvement. We present the novel use of selective bronchial blockade in a challenging case of CDH to determine if surgical lung resection may benefit the infant. We also review the physiology of gas exchange during the use of a bronchial occluder and the relevant literature.
先天性膈疝(CDH)的诊断与显著的发病率和死亡率相关。尽管临床过程因肺实质和血管发育不全、肺动脉高压、通气/灌注(V/Q)不匹配、肺功能下降和躯体生长不良等后遗症而变得复杂,但近年来患有CDH的新生儿的存活率有所提高。在本病例报告中,我们描述了一名产前诊断为CDH且预后不良的婴儿,该婴儿接受了初次手术并随后进行了气管切开术,但基于V/Q核素闪烁扫描,由于大面积通气但灌注不良的肺,其临床过程恶化。气肿的左肺导致纵隔移位并压迫右肺,进一步损害气体交换。在对灌注不足的左肺进行支气管封堵后,婴儿的临床症状有所改善。这为进一步切除灌注不足的肺叶并持续改善临床症状的治疗铺平了道路。我们展示了在一例具有挑战性的CDH病例中选择性支气管封堵的新应用,以确定手术性肺叶切除是否可能使婴儿受益。我们还回顾了使用支气管封堵器期间气体交换的生理学及相关文献。