Alireza Akhondi-Asl, PhD
Instructor in Anaesthesia
Boston Children's Hospital, United States
Disclosure information not submitted.
Terry Buchmiller, MD
Associate in Surgery, Department of Surgery
Boston Children's Hospital, United States
Disclosure information not submitted.
Sally Vitali, MD
Senior Associate in Critical Care Medicine
Boston Children's Hospital, United States
Disclosure information not submitted.
Jill Zalieckas, MD, MPH
Associate in Critical Care Medicine; Assistant in Surgery
Boston Children's Hospital, United States
Disclosure information not submitted.
Belinda Dickie, MD, PhD
Assistant in Surgery, Department of Surgery
Boston Children's Hospital, United States
Disclosure information not submitted.
Nilesh Mehta, MD
Senior Associate in Critical Care Medicine
Boston Children's Hospital, United States
Disclosure information not submitted.
Ben Albert, MD
Critical Care Medicine Fellowship Program Director; Assistant in Critical Care Medicine
Boston Children's Hospital, United States
Disclosure information not submitted.
Title: Long-term Functional Outcomes in Critically Ill Neonates with Congenital Diaphragmatic Hernia
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) follow-up visits were 7.0 [7.0, 8.0], 7.0 [ 6.0, 8.0], and 6.0 [6.0, 8.0], respectively. 17 patients (16.5%) had at least moderate impairment at >12-months. Median composite FSS scores decreased by 2.0 points from hospital discharge to the >12-months follow-up. Median feeding domain scores improved by 1.0 [1.0, 3.0] whereas the other median individual domain scores remained unchanged with no impairment.
Conclusions: We observed unchanged or improving functional status serially through one year after hospital discharge in this cohort of CDH survivors. FSS scores were characterized as mild (FSS < 9) at discharge and one-year follow-up. The feeding domain had the highest level of functional impairment. Optimal feeding practices may impact long-term growth and should be further investigated in this population.