RT Book, Section
A1 Bianchi, Diana W.
A1 Crombleholme, Timothy M.
A1 D'Alton, Mary E.
A1 Malone, Fergal D.
SR Print(0)
ID 1106397564
T1 Congenital Diaphragmatic Hernia
T2 Fetology: Diagnosis and Management of the Fetal Patient, 2e
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 978-0-07-144201-5
LK obgyn.mhmedical.com/content.aspx?aid=1106397564
RD 2024/04/19
AB Key  PointsDefect  in  formation  of  diaphragm  that  is  associated  with  pulmonary  hypoplasia.Incidence  is  around  1  in  2200  with  85%  to  90%  being  left  sided,  10%  to  15%  right  sided,  and  2%  bilateral.Approximately  60%  of  cases  are  isolated  and  40%  are  complex  or  syndromic.Multiple  sonographic  and  MRI  techniques  have  been  described  to  predict  lung  volume,  which  is  an  important  predictor  of  survival.  Liver  position  is  also  very  important  with  regard  to  outcome.In  the  most  severe  cases  of  congenital  diaphragmatic  hernia  (CDH),  the  fetal  tracheal  occlusion  (FETO)  with  a  balloon  is  removed  prior  to  delivery  “or  EXIT-to-ECMO  offers  the  best  hope  for  survival.”All  fetuses  with  CDH  should  be  delivered  in  a  tertiary  center  with  specific  neonatal  and  surgical  expertise  in  this  condition  and  ECMO  capability.Cesarean  section  delivery  is  indicated  only  for  standard  obstetrical  reasons.Approximately  a  dozen  single-gene  disorders  have  CDH  as  a  major  feature.  With  increased  cytogenetic  resolution  through  array  cGH,  more  syndromic  cases  are  being  recognized  as  being  associated  with  a  chromosome  abnormality.  Fetal  karyotype  is  indicated.