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Long-Term Functional Outcomes at 1-Year After Hospital Discharge in Critically Ill Neonates With Congenital
Jill E O'Hara1,2,3,4,5,6,7,8, Terry L Buchmiller1,4, Lori J Bechard1,2
1Harvard Medical School, Boston, MA.
Insights
Congenital diaphragmatic hernia (CDH) survivors show improving functional status after hospital discharge. While most had mild impairment, feeding difficulties persisted in some CDH patients.
Area of Science:
- Pediatric Surgery
- Neonatology
- Developmental Pediatrics
Background:
- Congenital diaphragmatic hernia (CDH) is a complex birth defect with significant long-term health implications for survivors.
- Assessing functional status post-discharge is crucial for understanding the long-term morbidity associated with CDH.
- The Functional Status Scale (FSS) is a validated tool for evaluating functional outcomes in pediatric populations.
Purpose of the Study:
- To longitudinally evaluate changes in functional status using the FSS in infants surviving congenital diaphragmatic hernia (CDH) after hospital discharge.
- To identify specific domains of functional impairment that may persist or improve over time in CDH survivors.
- To determine factors associated with functional impairment in CDH survivors.
Main Methods:
- A single-center retrospective cohort study was conducted involving infants with Bochdalek CDH admitted between January 2009 and December 2019.
- Functional Status Scale (FSS) scores were calculated at hospital discharge and at three subsequent outpatient follow-up intervals (0-6 months, 6-12 months, and >12 months).
- Clinical data were extracted from medical records to identify potential factors associated with functional impairment.
Main Results:
- One hundred forty-two CDH survivors were analyzed. At discharge, the median FSS was 8.0, with 27.5% experiencing at least moderate impairment (FSS ≥ 9).
- Longitudinal follow-up showed improving median FSS scores, reaching 6.0 (>12 months), with 15% still having moderate impairment.
- The feeding domain exhibited the highest level of functional impairment, while other domains showed improvement or remained unimpaired.
Conclusions:
- The majority of CDH survivors experience mild functional impairment at discharge and 1-year follow-up, with a trend toward improvement.
- Feeding difficulties represent a significant area of concern requiring targeted interventions in CDH survivors.
- Factors such as defect laterality, size, ECMO use, CPR, and chromosomal anomalies are associated with functional impairment, guiding future management strategies.
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.
