TY  - CHAP
M1  - Book, Section
TI  - Meconium Ileus
A1  - Carlyle, Brent E.
A1  - Borowitz, Drucy S.
A1  - Glick, Philip L.
A2  - Ziegler, Moritz M.
A2  - Azizkhan, Richard G.
A2  - Allmen, Daniel von
A2  - Weber, Thomas R.
Y1  - 2014
N1  - 
T2  - Operative Pediatric Surgery, 2e
AB  - Cystic  fibrosis  (CF)  is  an  autosomal  recessive  disease  characterized  by  respiratory,  digestive,  reproductive,  and  sweat  gland  abnormalities.Meconium  ileus  (MI)  accounts  for  up  to  33%  of  neonatal  intestinal  obstructions  and  occurs  in  approximately  20%  of  newborns  diagnosed  with  CF.More  than  1870  distinct  mutations  of  the  cystic  fibrosis  transmembrane  regulator  (CFTR)  protein  have  been  identified,  some  of  which  cause  CF.  The  most  common  mutation  is  F508del,  which  accounts  for  approximately  two  third  of  all  mutations  in  patients  with  CF.Prenatal  diagnosis  of  MI  may  be  made  by  sonogram,  most  commonly  showing  dilated  bowel  or  increased  echogenicity  within  the  bowel  lumen.  The  positive  predictive  value  of  hyperechoic  masses  in  a  high-risk  fetus  is  estimated  to  be  52%,  whereas  in  the  low-risk  fetus  the  estimate  is  only  6.4%.MI  can  be  simple  or  complicated.  In  the  simple  form,  thickened  meconium  begins  to  form  in  utero  and,  as  it  obstructs  the  mid  ileum,  proximal  dilatation,  bowel  wall  thickening,  and  congestion  occur.  In  complicated  MI  volvulus,  atresia,  necrosis,  perforation,  meconium  peritonitis,  and  pseudocyst  formation  may  occur.Nonoperative  management  with  Gastrografin®  enema  is  the  preferred  initial  treatment  option  for  simple  MI.  Special  care  should  be  observed  to  avoid  hypovolemic  shock  with  adequate  prior  intravenous  (IV)  hydration.  Bowel  perforation  may  be  avoided  by  forgoing  balloon  catheter  inflation  and  maintaining  a  low  hydrostatic  pressure  during  enema  instillation.Complicated  MI  and  simple  MI  that  fails  enema  treatment  will  require  bowel  resection  with  a  temporary  enterostomy  or  resection  with  primary  anastomosis.  The  authors  advocate  a  double  barrel  enterostomy;  followed  by  serial  irrigations  of  the  open  stomas  and  enteral  feedings  in  the  distal  stoma  to  stimulate  bowel  growth.  Bowel  continuity  is  usually  restored  6  to  12  weeks  later.Infants  with  simple  MI  and  CF  may  be  given  breast  milk  or  routine  infant  formula,  enzymes,  and  vitamins.  Those  who  have  a  complicated  surgical  course  will  require  either  continuous  enteral  feedings  or  total  parenteral  nutrition  (TPN).  Pancreatic  enzymes  must  be  given  orally  starting  at  2000  to  4000  lipase  units  per  120  mL  of  full-strength  formula.The  prognosis  for  patients  with  CF  and  MI  is  very  promising;  recently  published  studies  show  1-year  survival  rates  as  high  as  100%.  Long-term  follow-up  at  13  years  shows  similar  pulmonary  function  and  nutritional  status  in  CF  patients  with  or  without  MI.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/19
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1100434546
ER  -