Long-term medical and psychosocial outcomes in congenital diaphragmatic hernia survivors

Abstract

ObjectiveSurvival 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 and designParticipants 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.Results34 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 (FEV1) (−1.49 vs −0.01; p=0.004), FEV1/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).ConclusionSurvivors of CDH may have significant adverse long-term medical and psychosocial issues that would be better recognised and managed in a multidisciplinary clinic.

Chest CT scoring for evaluation of lung sequelae in congenital diaphragmatic hernia survivors

“…A detailed comparison of CT abnormalities between studies is difficult, owing to different scoring methods, age at evaluation, CT modalities, and the severity of disease. Studies on CT findings in CLD of prematurity report subpleural linear and triangular opacities in 65%‐95% of the patients 10,14 . These opacities represent neofissures and strands of atelectasis formed by hyperaerated and atelectatic zones.…”

“…Some long‐term follow‐up programs report abnormalities on chest radiographs 7,16,17 in more than 50%. A very recent publication reports on spirometry‐guided chest CT in 26 patients with CDH compared with controls at a median age of 10 years using a modified Brody score 10 . The main findings in this older age group were subpleural linear and triangular markings as well as architectural distortion.…”

“…Studies on CT findings in CLD of prematurity report subpleural linear and triangular opacities in 65%-95% of the patients. 10,14 These opacities represent neofissures and strands of atelectasis formed by hyperaerated and atelectatic zones. While bronchiectasis is rarely reported in individuals with CLD of prematurity, almost half of the CT scans in our CDH patients showed some degree of bronchiectasis.…”

“…Apart from a structured clinical evaluation, a respiratory questionnaire, and a cardiac ultrasound, 8 this follow‐up program includes an evaluation of structural lung sequelae with chest computed tomography (CT). Although other follow‐up programs included chest radiographs, scintigraphy or magnetic resonance imaging (MRI), limited data are available in literature on structural lung abnormalities in CDH survivors after the neonatal age 1,5‐7,9 or on the correlation with later clinical and lung function parameters 10 …”

“…Although other follow-up programs included chest radiographs, scintigraphy or magnetic resonance imaging (MRI), limited data are available in literature on structural lung abnormalities in CDH survivors after the neonatal age 1,[5][6][7]9 or on the correlation with later clinical and lung function parameters. 10 The purpose of performing chest CT scans is to document the structural lung sequelae at age 1 and to correlate them with prenatal and neonatal parameters as well as clinical outcome. Our hypothesis is that structural lung sequelae will cover a broad spectrum from very subtle to very severe lung damage and that certain prenatal and neonatal parameters will correlate with the extent of the CT abnormalities.…”