INTRODUCTION:Congenital diaphragmatic hernia (CDH) is a critical congenital defect with significant potential morbidity. There are limited studies describing long-term functional outcomes in CDH survivors. We aimed to examine clinical characteristics and serial change in functional status scale (FSS) after hospital discharge in CDH survivors. METHODS:Single-center retrospective cohort study of infants with CDH admitted to the PICU. Baseline demographics and disease-specific variables were collected. We extracted medical record data to calculate FSS scores at 4 timepoints: hospital discharge, 0-6-months, 6-12-months, and >12-months after discharge. We excluded patients that died before discharge. We assessed inter-rater reliability of FSS calculations. We recorded the change in median composite and domain-related FSS scores up to 1 year after discharge.RESULTS: FSS data was analyzed for a random sample of 135 newborns (60% male, average birth weight 3.03 kg) in the PICU from Jan 2009 to Dec 2019. CDH was left-sided in 74%. Defect sizes were A (20%), B (34%), C (34%), and D (3%). Median CDH risk score was 2.00 [1.75, 3.00]. 23 (17%) patients died before discharge. Median [IQR] FSS score at hospital discharge was 8.0 [7.0, 9.0] with 32 (30.5%) patients having at least moderate impairment (FSS >9). Median [IQR] FSS at 0-6-months (n=104), 6-12-months (n=104), and at >12-months (n=103) followup visits were 7.
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