RT Book, Section
A1 Bianchi, Diana W.
A1 Crombleholme, Timothy M.
A1 D'Alton, Mary E.
A1 Malone, Fergal D.
SR Print(0)
ID 1106399253
T1 Hirschsprung's Disease
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=1106399253
RD 2023/03/20
AB Key  PointsOne  of  the  most  common  causes  of  intestinal  obstruction  in  the  newborn.  Characterized  by  severe  constipation  due  to  functional  colonic  obstruction  with  megacolon.Hirschsprung's  disease  rarely  presents  prenatally,  but  when  it  does  it  is  usually  due  to  total  colonic  aganglionosis.Sonographic  features  may  include  dilated  loops  of  small  intestine  occasionally  with  enterolithiasis.About  a  quarter  of  affected  patients  have  associated  anomalies.  There  is  a  strong  association  with  Down  syndrome.Hirschsprung's  disease  diagnosed  prenatally  is  at  increased  risk  for  syndromic  associations  by  being  long  segment  aganglionosis.Level  II  sonogram  and  antenatal  karyotype  are  recommended.Diagnosis  requires  postnatal  rectal  biopsy  to  confirm  aganglionosis.Treatment  is  a  pull-through  procedure  to  bring  normal  ganglionated  bowel  to  the  dentate  line.