Abstract

Congenital diaphragmatic hernia (CDH) is a severe pediatric disorder with herniation of abdominal viscera into the thoracic cavity. Since neurodevelopmental impairment constitutes a common outcome, we performed morphometric magnetic resonance imaging (MRI) analysis on CDH infants to investigate cortical parameters such as cortical thickness (CT) and local gyrification index (LGI). By assessing CT and LGI distributions and their correlations with variables which might have an impact on oxygen delivery (total lung volume, TLV), we aimed to detect how altered perfusion affects cortical development in CDH. A group of CDH patients received both prenatal (i.e., fetal stage) and postnatal MRI. From postnatal high-resolution T2-weighted images, mean CT and LGI distributions of 16 CDH were computed and statistically compared to those of 13 controls. Moreover, TLV measures obtained from fetal MRI were further correlated to LGI. Compared to controls, CDH infants exhibited areas of hypogiria within bilateral fronto-temporo-parietal labels, while no differences were found for CT. LGI significantly correlated with TLV within bilateral temporal lobes and left frontal lobe, involving language- and auditory-related brain areas. Although the causes of neurodevelopmental impairment in CDH are still unclear, our results may suggest their link with altered cortical maturation and possible impaired oxygen perfusion.

Highlights

  • Congenital diaphragmatic hernia (CDH) is a severe pediatric disorder characterized by herniation of abdominal viscera into the thoracic cavity through a diaphragmatic defect, mostly on the left side [1], associated with a variable degree of pulmonary hypoplasia, pulmonary hypertension, and cardiac function abnormalities

  • We proposed a morphometric assessment to investigate subtle cortical alterations of cortical thickness (CT) and local gyrification index (LGI) on CDH infant brains, since their role in neurological development has been fully addressed [17,18]

  • Among 22 CDH fulfilling the inclusion criteria, 6 subjects were excluded from the statistical analysis due to processing issues related to image artefacts

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Summary

Introduction

Congenital diaphragmatic hernia (CDH) is a severe pediatric disorder characterized by herniation of abdominal viscera into the thoracic cavity through a diaphragmatic defect, mostly on the left side [1], associated with a variable degree of pulmonary hypoplasia, pulmonary hypertension, and cardiac function abnormalities. Earlier studies report long-term outcomes, with evidence of impaired language and visual motor skills, behavioral, cognitive and developmental delay and hearing loss [5,6,7,8,9,10] This evidence was further confirmed for short-term evaluation, where neurodevelopmental assessment at one year of age revealed mild (44%) and severe (13%) delay in at least one domain among cognitive, language and motor functions, depending on several determinants such as intubation, oxygen requirement and intensive care unit stay [4,11]. A morphometric approach was recently proposed to measure brain structures of CDH fetuses in terms of fronto-occipital, brain biparietal and bone biparietal diameters, revealing enlarged extra-axial spaces and cerebellar dimensions associated with CDH severity [16] In this context, we proposed a morphometric assessment to investigate subtle cortical alterations of cortical thickness (CT) and local gyrification index (LGI) on CDH infant brains, since their role in neurological development has been fully addressed [17,18]. CT varies between 1 and 4.5 mm with an average value of approximately

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