Introduction
Congenital diaphragmatic hernia (CDH) is a severe birth defect, occuring
in approximately 1 in 3,000 live born neonates. 1Despite optimal neonatal treatment, the disease is associated with high
mortality and survivors often suffer short and long term
morbidities.1 These include respiratory,
gastro-intestinal and neurologic impairments.2Neurodevelopmental delays, as well as behavioral difficulties have been
linked to CDH in the past and certain risk factors have been suggested,
including gestational age at birth, disease severity, associated
anomalies, the requirement for extracorporeal membrane oxygenation and
long stay in the neonatal intensive care unit. 2-5
In infants with CDH, imaging studies have demonstrated several
abnormalities including increased extra-axial space, delayed sulcation
and white matter injury but the exact mechanisms remain unclear. As for
other congenital malformations with an increased risk for
neurodevelopmental abnormalities, parents may ask whether these are
already present at the time of prenatal diagnosis, or they are more
likely to be postnatally acquired. This will become even more important
with the advent of effective fetal therapy 6, 7, which
aims at fetuses with the more severe forms of CDH. For those parents and
physicians likewise will want to understand whether brain development in
CDH is already altered prenatally, and when present, if this would be
severity dependent.8 Currently, there is limited data
on in utero brain development in CDH fetuses. 8-10.
Radhakrishnan et al described enlargement of the extra-axial space and
congestion of the venous sinuses in the third trimester. A further study
reported a correlation between cerebellar and vermian dimensions and the
severity of lung hypoplasia (evidenced by a lower fetal lung volume).8 Recently, we described in a longitudinal ; study
changes in cerebellar growth, and correlated this to disease severity;
this study however was done by ultrasound.5
The objective was to describe brain development in CDH fetuses however
using the gold standard imaging modality (MRI) to document cortical
folding as well as the dimensions and volume of different brain
structures in comparison to that in normal fetuses longitudinally during
the second half of pregnancy.