Pediatr Crit Care Med

. 2023 Apr 26.

 doi: 10.1097/PCC.0000000000003249. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/37098788/

Long-Term Functional Outcomes at 1-Year After Hospital Discharge in Critically Ill Neonates With Congenital Diaphragmatic Hernia

Jill E O’Hara ^{1 2 3 4 5 6 7 8}, Terry L Buchmiller ^1 4, Lori J Bechard ^1 2, Alireza Akhondi-Asl ^1 2, Gary Visner ^1 5, Catherine Sheils ^1 5, Ronald Becker ^1 6, Mollie Studley ⁷, Lindsay Lemire ⁴, Mary P Mullen ^1 8, Sally Vitali ^{1 2 3 4 5 6 7 8}, Nilesh M Mehta ^{1 2 3 4 5 6 7 8}, Belinda Dickie ^1 2 4, Jill M Zalieckas ^1 2 3 4, Ben D Albert ^{1 2 3 4 5 6 7 8}

Affiliations expand

• PMID: 37098788
• DOI: 10.1097/PCC.0000000000003249

Abstract

Objectives: Congenital diaphragmatic hernia (CDH) is a birth defect associated with long-term morbidity. Our objective was to examine longitudinal change in Functional Status Scale (FSS) after hospital discharge in CDH survivors.

Design: Single-center retrospective cohort study.

Setting: Center for comprehensive CDH management at a quaternary, free-standing children’s hospital.

Patients: Infants with Bochdalek CDH were admitted to the ICU between January 2009 and December 2019 and survived until hospital discharge.

Interventions: None.

Measurements and main results: One hundred forty-two infants (58% male, mean birth weight 3.08 kg, 80% left-sided defects) met inclusion criteria. Relevant clinical data were extracted from the medical record to calculate FSS (primary outcome) at hospital discharge and three subsequent outpatient follow-up time points. The median (interquartile range [IQR]) FSS score at hospital discharge was 8.0 (7.0-9.0); 39 patients (27.5%) had at least moderate impairment (FSS ≥ 9). Median (IQR) FSS at 0- to 6-month (n = 141), 6- to 12-month (n = 141), and over 12-month (n = 140) follow-up visits were 7.0 (7.0-8.0), 7.0 (6.0-8.0), and 6.0 (6.0-7.0), respectively. Twenty-one patients (15%) had at least moderate impairment at over 12-month follow-up; median composite FSS scores in the over 12-month time point decreased by 2.0 points from hospital discharge. Median feeding domain scores improved by 1.0 (1.0-2.0), whereas other domain scores remained without impairment. Multivariable analysis demonstrated right-sided, C- or D-size defects, extracorporeal membrane oxygenation use, cardiopulmonary resuscitation, and chromosomal anomalies were associated with impairment.

Conclusions: The majority of CDH survivors at our center had mild functional status impairment (FSS ≤ 8) at discharge and 1-year follow-up; however, nearly 15% of patients had moderate impairment during this time period. The feeding domain had the highest level of functional impairment. We observed unchanged or improving functional status longitudinally over 1-year follow-up after hospital discharge. Longitudinal outcomes will guide interdisciplinary management strategies in CDH survivors.

Copyright © 2023 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.