Quality of Life Outcomes in Congenital Diaphragm Hernia: Can Prenatal Imaging Aid in this Discussion?
Fariha Sheikh MD 1, 2, Adesola Akinkuotu MD1, Sarah Jane Clark BS1, Irving J. Zamora MD1, 2, Darrell
Cass MD1, Oluyinka Olutoye MD PhD1, and Timothy C. Lee MD1
Department of Surgery, University of Florida College of Medicine, Jacksonville, FL
Texas Children’s Fetal Center, Michael E. DeBakey Department of Surgery, Baylor College of Medicine,
Houston, TX
Purpose
Quality of life (QOL) is a common point of discussion during prenatal counseling for congenital
diaphragm hernia (CDH) patients. However to date, there has been no study examining the association
of prenatal imaging characteristics and post-natal QOL outcomes for this patient population. The
objective of this study was to describe the association between prenatal CDH imaging and post-natal
QOL using a validated pediatric QOL survey.
Methods
The study was a retrospective review of CDH patients counseled at a fetal center between March 2002
and March 2014. Prenatal imaging characteristics analyzed were observed to expected total fetal lung
volumes (O/E TFLV) and percent liver herniation (%LH) measured on MRI; and ultrasound derived lung
to head (LHR) ratio. Parents were contacted by telephone to participate in the validated Pediatric
Quality of Life Inventory (PedsQL™, Version 4.0). Patients had to be at least 2 years of age for this
questionnaire. QOL was assessed on a 5-point scale per question converted to percentages with 100%
=none, 75%=minimal, 50%=some, 25%=many and 0%=significant perceived deficiencies in physical and
psychosocial (emotional, social, and school) functioning.
Results
Of the 95 CDH survivors within the study period, 68 were eligible for the QOL survey and 28 (42%) were
successfully contacted. The mean age of patients included in the survey was 5.5 years (2.3-11.7 years).
As viewed in Table 1, patients with severe CDH were not perceived as having significant functional
deficits compared to CDH patients with milder disease. Furthermore, across all categories, irrespective
of disease severity, patients had minimal QOL limitations. Patients without any limitations had a lower
rate of oxygen dependence at 30 days of life (29% vs. 71%, OR 0.16, CI 0.031-0.82, p=0.02). ECMO was
not associated with significantly worse QOL in physical functioning or psychosocial functioning.
Calculation of Cronbach’s alpha reliability coefficient yielded a correlation of 0.951 for the overall
survey, 0.911 for physical functioning questions, and 0.901 for psychosocial functioning.
Conclusions
Patients who presented with a prenatal diagnosis of severe CDH had similar postnatal QOL outcomes
when compared with CDH patients with less severe disease. Furthermore, even patients who required
ECMO had minimal limitations throughout all QOL categories. This survey information is useful in
counseling parents on QOL expectations for their child with a prenatal diagnosis of CDH.
Table 1
n
All patients
LHR >1
LHR 1-1.4
LHR <1
O/E TFLV >35%
O/E TFLV <35%
No ECMO
ECMO
LH <20%
LH >20%
28
20
6
2
16
10
18
10
5
12
Physical
Functioning Score
91%
94%
80%
86%
95%
84%
90%
91%
94%
83%
p-value
0.128
0.698
0.588
0.383
Psychosocial
Functioning Score
81%
84%
73%
77%
84%
78%
80%
82%
83%
75%
p-value
0.306
0.421
0.494
0.328