Larsen Ulla Lei, Agertoft Lone, Herskind Anne Maria, Strøm Thomas, Toft Palle, Halken Susanne
Research Unit for Department of Anaesthesiology & Intensive Care, Odense University Hospital, Odense, Denmark.
Department of Clinical Research, Faculty of Health sciences, University of Southern Denmark, Odense, Denmark.
Pediatr Pulmonol. 2025 Jan;60(1):e27468. doi: 10.1002/ppul.27468.
A main feature of CDH is lung hypoplasia and the related presentation of pulmonary hypertension and cardiac dysfunction. Multiple factors influence pulmonary status after CDH: degree of hypoplasia, ventilator-induced injury, altered growth and development of pulmonary structures, reduced diaphragm function and chest wall abnormalities. The evolution of pulmonary sequela in this population is still unclear. We aimed to describe the pulmonary status of our population of CDH-survivors and evaluated on risk factors.
CDH-survivors (1998-2015) were included and performed lung function tests and chest X-rays.
Fifty-one (51/71, 71.8%) participated. Median age was 12.2 (5.5-21.4) years, 28 (54.9%) male, 42 (82.4%) had left-sided hernias, 10 (19.6%) needed patch-repair and median length of stay in hospital was 28.0 (IQR 18.5-61.6) days in Table 1. Spirometry including bronchodilator response (BDR)-test, body plethysmography, and diffusion capacity, were available for 48, 42, and 40 participants. The mean (SD) z-score for FEV1 and FVC was -0.26 (1.70) and -0.28 (1.70). Twenty-one (43.8%) had obstructive patterns and six had positive BDR. TLC mean (SD) z-score was -0.18 (1.10). Four showed restricted/mixed patterns and 13 showed signs of hyperinflation. Increased RV/TLC-ratio and reduced FEV1 was associated with longer time on mechanical ventilation. Diffusion capacity was decreased in three cases. Chest X-ray revealed hernia recurrence (13.9%) and scoliosis (38.9%).
Mild obstructive impairment and hyperinflation was frequent in our CDH cohort and only a small subset of restrictive disorders were identified. We advocate follow-up by a specialized multidisciplinary team through childhood and into adulthood.
先天性膈疝（CDH）的一个主要特征是肺发育不全以及相关的肺动脉高压和心脏功能障碍表现。多种因素会影响CDH后的肺部状况：发育不全程度、呼吸机诱导性损伤、肺结构生长发育改变、膈肌功能降低以及胸壁异常。该人群肺部后遗症的演变仍不清楚。我们旨在描述我们的CDH存活者群体的肺部状况，并评估危险因素。
纳入1998 - 2015年的CDH存活者并进行肺功能测试和胸部X光检查。
51人（51/71，71.8%）参与。中位年龄为12.2（5.5 - 21.4）岁，男性28人（54.9%），42人（82.4%）为左侧疝，10人（19.6%）需要补片修复，住院中位时长为28.0（四分位间距18.5 - 61.6）天，见表1。48名、42名和40名参与者分别进行了包括支气管扩张剂反应（BDR）测试的肺量计检查、体容积描记法和弥散功能检查。第一秒用力呼气容积（FEV1）和用力肺活量（FVC）的平均（标准差）z评分分别为 - 0.26（1.70）和 - 0.28（1.70）。21人（43.8%）有阻塞性模式，6人支气管扩张剂反应阳性。肺总量（TLC）平均（标准差）z评分为 - 0.18（1.10）。4人表现为限制性/混合性模式，13人有肺过度充气迹象。右心室/肺总量（RV/TLC）比值增加和FEV1降低与机械通气时间延长有关。3例弥散功能降低。胸部X光检查显示疝复发（13.9%）和脊柱侧弯（38.9%）。
在我们的CDH队列中，轻度阻塞性损害和肺过度充气很常见，仅发现一小部分限制性疾病。我们主张由专业的多学科团队在儿童期直至成年期进行随访。
