Abnormal brain development and maturation on magnetic resonance imaging in survivors of severe congenital diaphragmatic hernia

• Published in: Journal of pediatric surgery 2012-03, Vol.47 (3), p.453-461
• Main Authors: Danzer, Enrico, Zarnow, Deborah, Gerdes, Marsha, D'Agostino, Jo Ann, Siegle, Jennifer, Bebbington, Michael W, Flake, Alan W, Adzick, N. Scott, Hedrick, Holly L
• Format: Article
• Language: English
• Subjects: Brain - growth & development; Brain - pathology; Brain Diseases - diagnosis; Brain Diseases - etiology; Brain maturation; Congenital diaphragmatic hernia; ECMO; Female; Follow-Up Studies; Hernia, Diaphragmatic - complications; Hernia, Diaphragmatic - diagnosis; Hernia, Diaphragmatic - therapy; Hernias, Diaphragmatic, Congenital; Humans; Infant; Infant, Newborn; Intracranial Hemorrhages - diagnosis; Intracranial Hemorrhages - etiology; Leukomalacia, Periventricular - diagnosis; Leukomalacia, Periventricular - etiology; Magnetic Resonance Imaging; Male; MR imaging; Pediatrics; Periventricular leukomalacia; Retrospective Studies; Severity of Illness Index; Surgery; Survivors; Total maturation score
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2011.10.002

Abstract Purpose The aim of the study was to evaluate the incidence of abnormal brain maturation in survivors of severe congenital diaphragmatic hernia (CDH). Material and Methods Between July 2004 and December 2009, 50 CDH survivors underwent detailed brain magnetic resonance (MR) imaging before discharge. Magnetic resonance images were analyzed to evaluate the presence of structural brain abnormalities and to calculate overall brain maturation using the total maturation score (TMS). Results Thirty-two children (64%) underwent MR imaging between 39 and 43 weeks of gestation, allowing for evaluation of the TMS. Eighteen (36%) underwent MR imaging between 44 and 69 weeks of gestation, allowing for structural analysis of brain maturity only. The mean TMS was 14.1 ± 1.2 and significantly lower than reported age-matched normative data in infants without CDH (15.3 ± 1.0, P = .02). The TMS in 4 patients (12.5%) corresponded to a delay of 1 month in structural brain development. Eight infants (25%) demonstrated a 2-week delay. Periventricular leukomalacia was detected in 9 (18%), incomplete development of the opercula in 7 (14%), various degrees of intracranial hemorrhage in 24 (48%), and prominent extraaxial fluid spaces in 20 (40%) cases. Conclusions Brain maturation in infants with severe CDH appears to be delayed. Long-term neurodevelopmental follow-up is needed to determine the significance of a lower-than-expected TMS and the presence of structural brain abnormalities on functional outcomes in this population.