Neonatal Intensive Care Unit, Perth Children's Hospital, Perth, Western Australia, Australia.
Children's Lung Health, Telethon Kids Institute, Perth, Western Australia, Australia.
Arch Dis Child. 2019 Aug;104(8):761-767. doi: 10.1136/archdischild-2018-316091. Epub 2019 Mar 15.
Survival rates for congenital diaphragmatic hernia (CDH) are increasing. The long-term outcomes of CDH survivors were compared with a healthy control group to assess the morbidity for guidance of antenatal counselling and long-term follow-up programmes.
Participants born with CDH in Western Australia 1993-2008 were eligible with matched controls from the general population. Participants had comprehensive lung function tests, echocardiogram, low-dose chest CT scan and completed a Strengths and Difficulties Questionnaire (SDQ) and quality of life (QOL) questionnaire.
34 matched case-control pairs were recruited. Demographic data between groups were similar. Cases were smaller at follow-up (weight Z-score of -0.2vs0.3; p=0.03; height Z-score of -0.3vs0.6; p=0.01). Cases had lower mean Z-scores for forced expiratory volume in 1 s (FEV) (-1.49 vs -0.01; p=0.004), FEV/forced vital capacity (-1.92 vs -1.2; p=0.009) and forced expiratory flow at 25-75% (FEF25-75) (-1.18vs0.23; p=0.007). Cases had significantly worse respiratory mechanics using forced oscillation technique. Subpleural triangles architectural distortion, linear opacities and scoliosis on chest CT were significantly higher in cases. Prosthetic patch requirement was associated with worse lung mechanics and peak cough flow. Cases had significantly higher rates of gastro-oesophageal reflux disease (GORD) and GORD medication usage. Developmental delay was significantly higher in cases. More cases had a total difficulties score in the high to very high range (25% vs 0%, p=0.03) on the SDQ and reported lower objective QOL scores (70.2 vs 79.8, p=0.02).
Survivors of CDH may have significant adverse long-term medical and psychosocial issues that would be better recognised and managed in a multidisciplinary clinic.
先天性膈疝（CDH）的存活率正在提高。将 CDH 幸存者的长期结果与健康对照组进行比较，以评估发病率，为产前咨询和长期随访计划提供指导。
1993 年至 2008 年在西澳大利亚出生的患有 CDH 的参与者符合条件，并与普通人群中的匹配对照组进行匹配。参与者接受了全面的肺功能测试、超声心动图、低剂量胸部 CT 扫描，并完成了《强弱问卷》（SDQ）和生活质量（QOL）问卷。
共招募了 34 对匹配的病例对照。两组间的人口统计学数据相似。随访时，病例组的体重 Z 评分较小（-0.2 对 0.3；p=0.03），身高 Z 评分较小（-0.3 对 0.6；p=0.01）。病例组的 1 秒用力呼气量（FEV）的平均 Z 评分较低（-1.49 对-0.01；p=0.004），FEV/用力肺活量（-1.92 对-1.2；p=0.009）和 25-75%用力呼出量（FEF25-75）（-1.18 对 0.23；p=0.007）。病例组使用强迫振荡技术的呼吸力学明显较差。胸部 CT 上的胸膜下三角形结构扭曲、线性混浊和脊柱侧凸在病例组中明显更高。假体补丁的需求与更差的肺力学和峰值咳嗽流量有关。病例组的胃食管反流病（GORD）和 GORD 药物使用的发生率明显更高。病例组的发育迟缓发生率明显更高。SDQ 中，更多的病例总困难评分处于高至极高范围（25%对 0%，p=0.03），并报告了较低的客观 QOL 评分（70.2 对 79.8，p=0.02）。
CDH 幸存者可能存在严重的长期医疗和心理社会问题，如果在多学科诊所中得到更好的识别和管理，这些问题可能会得到更好的识别和管理。
