TY - JOUR
T1 - Abnormal brain development and maturation on magnetic resonance imaging in survivors of severe congenital diaphragmatic hernia
AU - Danzer, Enrico
AU - Zarnow, Deborah
AU - Gerdes, Marsha
AU - D'Agostino, Jo Ann
AU - Siegle, Jennifer
AU - Bebbington, Michael W.
AU - Flake, Alan W.
AU - Adzick, N. Scott
AU - Hedrick, Holly L.
PY - 2012/3/1
Y1 - 2012/3/1
N2 - 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.
AB - 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.
KW - Brain maturation
KW - Congenital diaphragmatic hernia
KW - ECMO
KW - MR imaging
KW - Periventricular leukomalacia
KW - Total maturation score
UR - http://www.scopus.com/inward/record.url?scp=84858187406&partnerID=8YFLogxK
U2 - 10.1016/j.jpedsurg.2011.10.002
DO - 10.1016/j.jpedsurg.2011.10.002
M3 - Article
C2 - 22424337
AN - SCOPUS:84858187406
SN - 0022-3468
VL - 47
SP - 453
EP - 461
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 3
ER -